Respiratory disease

What we need to knowWhat we need to do

Mark W Yates FRACP

What we need to knowWhat we need to do

J Paul Seale PhD, FRACP

Patients with chronic obstructive pulmonary disease and a stable daytime Pao2 of ≤ 55 mmHg (7.3kPa) live longer and have a better quality of life if provided with long-term continuous oxygen therapy. It is reasonable to offer continuous oxygen therapy also to patients with other lung diseases that cause chronic hypoxaemia. Indications for supplemental oxygen therapy during exercise (ambulatory oxygen therapy) and sleep (nocturnal oxygen therapy) are less clear.

Christine F McDonald PhD, FRACP · Alan J Crockett PSM, MPH · Iven H Young BSc, MB BS, PhD, FRACP

Peter A Frith Head of Southern Respiratory Services, Respiratory Unit, Flinders Medical Centre, Bedford Drive, Bedford Park, SA 5042. Peter.frithATrgh.sa.gov.au To the Editor: The informative study by Simons and colleagues1 has highlighted a major concern. Chronic obstructive pulmonary disease (COPD) is one of Australia’s top four causes of death and burden of illness,2 yet the authors have made no mention of COPD. Failure to recognise the importance of this disease is an endemic attitude in Australia and globally3-5 that results in under-representation of COPD in epidemiological surveys and in inadequate funding for effective treatments and research. The study found that peak expiratory flow (PEF) provides the highest hazard ratios for predicting time to death in women (and the second highest in men). There is even a “dose–response” effect. Using the term “impaired PEF” is a bit like saying “impaired ECG” without attributing a diagnosis. PEF is a measure of airway calibre, and impairment of PEF indicates airway disease — largely COPD in this population. Smoking accounts for about 85% of the risk of COPD, and about 50% of smokers develop airflow limitation,4-6 so it is not surprising that the interaction between PEF and smoking was the most important predictor of reduced survival in this large cohort. It’s time to stop hiding our heads in the ashtray! Smoking combined with low PEF is COPD. We must demand that our medical and epidemiological professions uncover people with undiagnosed COPD. Early diagnosis is simple.5 It’s not normal to be unable to keep up with friends at work or during recreation because of breathlessness, and a daily cough is really an airway disease. If symptoms are acknowledged, spirometry will confirm the diagnosis. We should help our patients to enunciate these hidden symptoms so their condition can be diagnosed accurately, and effective management begun, as highlighted in the “COPDX management guidelines”.5 Primary and secondary prevention must focus on reducing smoking among young people. Smoking cessation, the use of effective drugs, and pulmonary rehabilitation are the cornerstones of COPD therapy that lead to better quality survival. COPD is common and under-diagnosed. Simons et al have partly exposed this deadly condition. Their data, added to other Australian data,7 should trigger actions that facilitate earlier diagnosis throughout Australia and support delivery of effective treatment to the thousands “dying a slow death” from COPD. Australia’s illness burden from COPD is high. Its prevalence and burden in Australia are rising, especially in women. Globally, the World Health Organization expects COPD to rise from 12th to 5th as a cause of illness burden by 2020. We must acknowledge that PEF impairment is not simply a mysterious risk factor for early “all-cause mortality”, but is indicative of COPD being a major contributor to death in this population.

Peter A Frith

Leon A Simons,* Judith Simons† * Director, Dubbo Study of the elderly, † Data Manager, Lipid Department, St Vincent’s Hospital, Darlinghurst, NSW 2010. L. SimonsATnotes.med.unsw.edu.au In reply: The prospective Dubbo Study of the elderly has produced a series of publications in which reduced peak expiratory flow (PEF) has been shown to be associated with increased risk of death,1,2 as well as increased risk of heart attack,3 ischaemic stroke4 and admission to a nursing home. 5 We have employed a purely statistical definition of impaired PEF, namely the lowest third of our sex-specific population distribution. We agree that many subjects so defined with impaired PEF, and who are smokers, will have underlying and potentially undiagnosed chronic obstructive pulmonary disease (COPD). Epidemiological studies have highlighted the importance of impaired PEF. It is now time for health professionals to implement the COPDX management guidelines referred to by Frith6 in a still more effective manner and to devise better prevention programs.

Leon A Simons · Judith Simons

To the Editor: The disastrous events of Boxing Day, 2004 left hundreds of thousands dead, injured or homeless across large parts of Asia. Many aid teams dispatched to affected areas are grappling with the aftermath of this catastrophe. Here, I present one of many clinical observations of what we encountered in the field. It is a clinical anecdote, but one worth sharing, as it may guide future teams in similar situations. A 62-year-old woman was admitted to hospital with a history of vague ill-health for 12 months, and a subacute illness over the 4 weeks since immersion in the tsunami, with persistant cough, dyspnoea and weakness to the point of being largely bed-bound. She was cachectic, had a fever of 37.5°C and scattered crackles in both lower lung fields. Radiology facilities were not available and she was not producing sputum. She was treated empirically with antituberculous chemotherapy, as well as broad-spectrum antibiotics in the form of amoxycillin and ciprofloxacin orally, but her condition did not improve. X-ray facilities became available soon thereafter, and a chest x-ray showed changes more in keeping with a necrotising pneumonia than tuberculosis ( [a]). Her treatment was changed to intravenous meropenem (1 g every 8 hours) and her condition was slowly improving when we left. During our 2-week posting in Banda Aceh, we saw about 6–10 patients at three hospitals presenting about a month after their immersion, with fluctuating fever; chronic, non-productive cough; and radiological evidence of bilateral, asymmetric, necrotising pneumonia with cavitation. Some patients developed empyemas and pneumothoraces ( [b]). They failed to respond to broad-spectrum antibiotics including ampicillin/gentamicin/metronidazole and ticarcillin–clavulanate/cotrimoxazole. Burkholderia pseudomallei was cultured from the pleural fluid of two of these patients, and Nocardia sp. from the sputum of another. A notable feature of these patients was their subacute presentation weeks after immersion in the tsunami, the persistence of symptoms despite other broad spectrum antibiotic therapy, and the development of radiological and clinical manifestations of necrosis with pleural involvement. Many of our patients described the wave as being “black”. In view of the immersion in muddy water in a tropical environment, B. pseudomallei is likely to have been one of the causative organisms in many of these cases. However, it has not been possible to culture B. pseudomallei from all patients, and it is likely that their infections were polymicrobial given the circumstances of their injuries. A variety of bacterial organisms, as well as fungi, have been recognised in other such situations.1-3 When we were there, Fakinah hospital provided one of the few laboratory services in Banda Aceh, and the availability of these facilities were limited as they were focused on public health surveillance. Thus, collection of specimens for culture was not routine. Furthermore, when we arrived there were no nurses, no medical records and no medication or observation charts. While the situation improved rapidly during our stay, these limitations meant that recognition of emerging clinical patterns was important. Many of the antibiotics initially used for patients with immersion injuries were ineffective in this setting, and the use of carbapenems became our first-line, or early second-line, antibiotic in post-immersion respiratory infections in Banda Aceh. Chest x-rays of patients with subacute necrotising pneumonia (a) Bilateral consolidation with scarring and early cavitation in the lower lung fields (b) Bilateral necrotising pneumonia complicated by right pneumothorax

Anthony M Allworth

Vanessa M McDonald,* Peter G Gibson† * Clinical Nurse Consultant, † Professor, Department of Respiratory and Sleep Medicine, John Hunter Hospital, Locked Bag 1, Hunter Region Mail Centre, Newcastle, NSW 2310. Peter. GibsonAThunter.health.nsw.gov.au To the Editor: The delivery of asthma drugs via inhalation offers the best balance between efficacy and safety. However, poor inhalation technique limits the efficacy of this approach. In recent years, there has been a progressive increase in the types of inhalation devices used in asthma management. We questioned whether this would lead to “inhaler-device polypharmacy”, a situation in which an individual used multiple types of inhalation device to deliver his or her asthma medications. We conducted a novel investigation of this issue in 2004. We examined the computerised records of adults with asthma who had been enrolled in a standardised, evidence-based asthma management and education program1 between 2000 and 2004. We noted the number and type of inhaler devices used, as well as competence with each device (a trained asthma educator had observed and scored inhalation technique). We defined “inhaler-device polypharmacy” as the use of two or more different types of inhalation device. The devices assessed in the education program included a pressurised metered-dose inhaler (with and without a spacer), turbuhaler, accuhaler, aeroliser, autohaler, and handihaler. Nebuliser use was not included in the evaluation. We assessed a total of 511 patients: 278 (107 male; mean age, 37 years) between 1 January 2000 and 1 January 2002 (Period 1), and 233 patients (55 male; mean age, 40 years) between 2 January 2002 and 1 January 2004 (Period 2). Period 1 patients were distinct from Period 2 patients in that the latter began their treatment after the release of combination asthma therapy in a single inhaler, when polypharmacy may have been expected to diminish. Inhaler-device polypharmacy was present in 203 (73%; 95% CI, 68%–78%) patients during Period 1 and 164 (70%; 64%–75%) in Period 2 (P = 0.3) (Box). In Period 1, inhalation technique was inadequate with at least one device in 58 (29%) patients using inhaler polypharmacy and in 19 (25%) using only one device (Box). In Period 2, inhalation technique was inadequate with at least one device in 85 (52%) patients using inhaler polypharmacy and in 25 (36%) using only one device. In both Period 1 and Period 2 patients, inadequate inhaler technique with at least one device increased with the number of devices used (P values 0.02 and 0.05, respectively) (Box). We conclude that inhaler-device polypharmacy is a common problem among adults with asthma. Inadequate inhalation-device technique is also common, especially among patients using three or more delivery devices. Inhaler-device polypharmacy could lead to poor asthma control through inadequate delivery of medication. Patients with poor asthma control should be evaluated for their asthma management skills, including competency in using inhaler devices. These skills should be optimised before a new drug and/or device is added to their treatment regimen. We see no justification for the use of more than two inhalation delivery devices in asthma management. Number of asthma patients using single or multiple inhalation devices and proportion of those patients with inadequate technique, over two time periods One device Two devices Three or more devices Period 1* (n = 278) 75 (27%) 150 (54%) 53 (19%) Period 2† (n = 233) 69 (30%) 129 (55%) 35 (15%) Patients with inadequate inhalation-device technique Period 1* 19/75 (25%) 35/150 (23%) 23/53 (43%) Period 2† 25/69 (36%) 64/129 (50%) 21/35 (60%) * 1 Jan 2000–1 Jan 2002. † 2 Jan 2002–1 Jan 2004.

Vanessa M McDonald · Peter G Gibson

Objective: To describe the spectrum of oxygen-delivery methods.Design: Clinical audit.Setting: Medical wards of a tertiary referral teaching hospital in August 2004.Participants: 98 medical patients receiving supplemental oxygen.Results: Of the 98 patients, 40 were not receiving oxygen by customary methods. In classifying the patterns of oxygen delivery, we describe the transcephalic, submental, and (inadvertent) rectal approaches, as well as lachrymal insufflation and the “Venturi cravat”. We also describe novel oxygen-weaning methods, including the half-wean, reverse wean, and placebo wean.Conclusions: Many patients receive oxygen by unconventional methods. We postulate that this is evidence of a renewed interest in the historical routes of oxygen delivery.

John R Attia MD, PhD, FRCPC · Balakrishnan R Nair FRACP, FRCP · Stephen R Mears DipIM · Karen I Hitchcock BA, MB BS

To the Editor: Poor adherence to therapy with asthma preventer medications is common among teenagers with asthma. This is often simple forgetfulness rather than active non-compliance. One of our adherent patients shared his secret with us — the alarm mode on his mobile phone. He set this to be activated twice daily to remind him to take his medicine. A recent survey by Newspoll showed that about 90% of teenagers aged between 13 and 19 years in Melbourne and Sydney have a mobile phone, most of them on a prepaid plan.1 We have subsequently recommended this reminder system to other patients, who have expressed interest in adopting the practice. This could be useful for all patients required to take regular medications. In the absence of a mobile phone, many household appliances (such as microwave ovens) have electronic timers that could be programmed to sound an alarm on a regular basis.

Gaudenz M Hafen · John Massie

Marcus (“Marc”) Faunce was born in Sydney on 5 December 1922, and died in the early hours of 14 June 2004 in Canberra, after a protracted struggle with renal cancer. After qualifying in medicine from the University of Adelaide in 1946, Marc served in the Australian Army Medical Corps in post-war Japan and trained at the British Postgraduate Medical School at Hammersmith. He became Senior Registrar at Sydney Hospital in 1949 and married Marjorie Morison in 1951, obtaining his membership of the Royal Australasian College of Physicians (RACP) in the same year. From 1954 to 1956 he did research at the Brompton Chest Hospital in London, becoming a Member of the Royal College of Physicians (RCP) (London) in 1955. Two years later, Marc and Marjorie moved back to Australia and settled in Canberra. Marc established himself as a consultant physician and salaried specialist with the Commonwealth antituberculosis campaign. He was elected a Fellow of the RACP in 1965 and of the RCP (London) in 1968. During his 35 years of full-time practice, Marc was much loved by his patients and admired by colleagues and students as a great general physician with a special interest in respiratory medicine. He co-founded the Canberra Medical Society in 1959. He served on the ACT Medical Board (1963–1974) and was Senior Physician Consultant to the Royal Australian Air Force Medical Directorate (1976–1980), with the rank of Group Captain. For these services he was made an Officer of the Order of the British Empire in 1969 and a Member of the Order of Australia (Military Division) in 1981. Marc was a staunch advocate for a medical school in Canberra. He served on the Board of Management of the Royal Canberra Hospital (RCH) from 1967 to 1974. He fought against the demolition of the RCH, situated adjacent to Lake Burley Griffin. The battle to save the hospital ultimately failed, and its units were progressively transferred to the Woden Valley Hospital (now the Canberra Hospital) from 1991 until its eventual closure in 1993, when Marc was given the honorary accolade of Emeritus Consultant to the RCH. It was a source of great satisfaction to Marc that his son, Thomas, was appointed Senior Lecturer and Chair of Personal and Professional Development at the new Medical School established at the Australian National University in 2004. Marc was renowned for his stringent adherence to medical confidentiality, being individually selected to be honorary personal physician to five prime ministers and six governors-general, for which services he was made a Commander of the Royal Victorian Order in 1995. He was a voracious and eclectic reader. Though distrusting spiritual conceptions, he heavily annotated many copies of the New Testament. His favoured social introduction was “Just sit down and let me tell you the story of my life”. Marc is survived by his sons Thomas and Marcus and daughter Charlotte.

Bryan S Furnass

Objectives: To investigate complementary and alternative medicine (CAM) use by patients with chronic obstructive pulmonary disease (COPD) and to explore their beliefs about CAM.Design and participants: Cross-sectional study of 173 patients with moderate to severe COPD, and indepth interviews with a purposive sample of 28 patients.Setting: Ambulatory care.Main outcome measures: Use of CAM; beliefs about the value of CAM.Results: 71 patients (41%) claimed to be using some form of CAM. Most commonly used were multivitamins and minerals, and garlic was the most commonly used herbal preparation. Patients reported that advertisements and people with prior experience of using CAM were their major sources of information. Extent of knowledge about CAM, degree of faith in CAM and personal attitudes influenced decisions to try CAM. Patients used CAM to promote general wellbeing, to counteract drug side effects, to compensate for dietary deficiencies and to ameliorate their disease. Efficacy appeared less important to users than safety. CAM practitioners were regarded as more convincing, informative, considerate and available compared with mainstream health professionals.Conclusions: Communication between patients and mainstream health professionals about CAM use could be improved by health professionals being more accepting of CAM use and having some basic knowledge about commonly used CAM preparations.

Johnson George MPharm · David C M Kong MPharm PhD · Kay Stewart BPharm(Hons), PhD · Lisa L Ioannides-Demos BPharm, PhD · Nick M Santamaria RN, PhD

Anton Y Peleg,* Wendy J Munckhof† * Infectious Diseases Registrar, Alfred Hospital, Prahran, VIC 3181; † Specialist in Infectious Diseases and Microbiology, Infection Management Service, Princess Alexandra Hospital, Brisbane, QLD. A. PelegATalfred.org.au To the Editor: Infection with community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) is emerging in many countries, including Australia.1 We report the first case of fatal necrotising pneumonia caused by CA-MRSA in Australia. A previously well 21-year-old Aboriginal man presented to the emergency department with fever and a productive cough. He had no known risk factors for sepsis (such as immunosuppression, diabetes, HIV infection, alcoholism, asplenia or recent influenza) and no history of hospitalisation in the previous 12 months. Chest x-ray revealed left mid-zone consolidation. He was prescribed amoxycillin–clavulanate and discharged. Two days later, the patient re-presented, with rigors, haemoptysis and agitation. Examination revealed a respiratory rate of 38 breaths per minute, oxygen saturation of 79% breathing room air, temperature of 38.7°C, sinus tachycardia (135 beats per minute), and a systolic blood pressure of 80 mmHg. Respiratory examination revealed diffuse coarse crepitations. No other source of infection was identified. The patient required intubation, mechanical ventilation and inotropic support. Sputum and blood samples were taken for culture, and empirical treatment was begun with intravenous ceftriaxone, erythromycin and a single dose of gentamicin and rifampicin. Initial investigations showed leukopenia (2.3 × 109/L; reference range [RR], 3.9–12.7 × 109/L), acute renal failure with a serum creatinine level of 0.18 mmol/L (RR, 0.06–0.11 mmol/L), and severe metabolic and respiratory acidosis (pH, 7.19; RR, 7.38–7.43). The following day, blood and sputum cultures showed gram-positive cocci resembling staphylococci, and intravenous flucloxacillin was added to the antibiotic regimen. Repeat chest x-ray revealed bilateral necrotising pneumonia. Despite resuscitation efforts, the patient died 48 hours after admission. Susceptibility testing of blood and sputum isolates subsequently confirmed MRSA. The isolate was sensitive to erythromycin, clindamycin, gentamicin, ciprofloxacin, tetracycline, vancomycin, rifampicin and fusidic acid. Methicillin resistance was confirmed by detection of the mecA gene by polymerase chain reaction (PCR). Further PCR testing of the isolate revealed the Panton–Valentine leukocidin (pvl) gene, an important virulence factor that has been associated with necrotising pneumonia2 and death,3 and is rarely found in methicillin-susceptible S. aureus or hospital-acquired MRSA isolates.1,2,5 Typing of the isolate by pulsed-field gel electrophoresis showed that it was the recently described “R” pulsotype of CA-MRSA, or “Queensland clone”.4 This clone was first noted in the white population in south-east Queensland in 2000, and is uncommon in Aboriginal people.4 Most CA-MRSA infection in Aboriginal people is caused by WA-MRSA, which may be less virulent than the Queensland clone of CAMRSA as it lacks the Panton–Valentine leukocidin.5 This is the first reported case of fatal necrotising pneumonia caused by CA-MRSA in Australia and illustrates the invasive nature of this infection. Thus far, CA-MRSA has predominantly caused skin and soft tissue infections, but the incidence of life-threatening sepsis is increasing. The first case of severe pneumonia caused by CA-MRSA in Australia was reported in early 2003.6 Fatal cases of necrotising pneumonia caused by CA-MRSA have also been described in the United States3 and France,7 and this presentation is becoming a particular feature of this organism. Clinicians should consider the possibility of CA-MRSA in any patient presenting to hospital with severe staphylococcal sepsis or pneumonia and should consider including parenteral vancomycin in the initial empirical therapy, particularly in geographic locations where CA-MRSA has been reported and in ethnic groups at increased risk.

Anton Y Peleg · Wendy J Munckhof

Marianne E Jauncey,* Paul K Armstrong,† Emily L Morgan,‡ Jeremy M McAnulty§ NSW Public Health Officer, Public Health Training and Development Branch; † Medical Epidemiologist, § Director, Communicable Diseases Branch; NSW Health, North Sydney, NSW. ‡ General Practitioner, Ballina West Medical Centre, Ballina, NSW. Marianne.jaunceyATyahoo.com.au To the Editor: In 2003, severe acute respiratory syndrome (SARS) became the first pandemic of the 21st century. Despite spreading to 29 countries, a rapid and coordinated international effort led to its containment. Here, we examine Australia’s only laboratory-confirmed case, and the investigation of possible subsequent transmission. In June 2003, the World Health Organization (WHO) notified Australian health authorities of a 26-year-old tourist in whom SARS-coronavirus-specific antibodies had recently been detected. She was part of a retrospective serological survey of people who stayed at the Hotel Metropole, Hong Kong, on 21 February,1 the same time a SARS source case infected at least 14 other hotel guests.2 On 22 February, the 26-year-old tourist travelled to Australia and 4 days later developed myalgia, lethargy and cough. On 6 March, 6 days before the first WHO global alert on SARS, she saw a general practitioner (GP) in northern New South Wales, to whom she also reported nausea, vomiting, nocturnal fever and pronounced lethargy. On examination she was afebrile, pale, unwell, with a cough and clear chest on auscultation. She declined further investigations and hospital admission; her condition gradually improved, and she left Australia 6 days later. She reported close contact with only three people during her Australian visit — her partner, the GP, and the GP’s surgery nurse. None reported subsequent illness and all tested negative for SARS-coronavirus antibody by direct immunofluorescence, a highly sensitive and specific method.3 Australia was fortunate that the tourist was not particularly infectious. The Hotel Metropole case was identified as the source case for four national and international clusters of SARS.2 The resulting human and economic cost was substantial.4 Without specific treatments, basic public health measures proved the only effective means to contain SARS. These included rapid case detection and isolation, contact tracing, handwashing and the correct use of personal protective equipment.5 Many GP practices and some hospitals in Australia do not have isolation facilities or infection control resources to effectively contain diseases like SARS. In the event of local transmission of SARS, infection may well have occurred in Australian healthcare workers. In the wake of SARS and, more recently, avian influenza, GPs must develop infection control plans to protect their own health as well as that of their patients. These should include obtaining a history of travel to outbreak-affected areas, reserving an area for patient isolation, and using appropriate infection control precautions during such outbreaks. Clinicians in other healthcare settings also need to review current infection control practices. If Australia is to remain the “lucky country” with regard to communicable diseases, basic public health measures aimed at preventing transmission of infection in healthcare settings is essential.

Marianne E Jauncey · Paul K Armstrong · Emily L Morgan · Jeremy M McAnulty

Clinical record A 33-year-old woman presented to the emergency department with a 10-day history of vesicular rash, 2 days of cough and fever, and 12 hours of dyspnoea, malaise and facial swelling. Her three children were recovering uneventfully from chickenpox. She had no past history of varicella infection or vaccination. She was a non-smoker, had no pre-existing medical conditions and was not known to be pregnant at the time of presentation. On presentation, the patient was in respiratory distress, with a respiratory rate of 36 per minute, pulse of 140 bpm, and oxygen saturation of 72% in room air. Subcutaneous emphysema was noted over the upper chest wall. Multiple healing and healed vesicles were present. The provisional clinical diagnosis was pneumonia, in the presence of resolving varicella. Results of blood tests on admission are shown in Box 1. Blood film demonstrated neutrophilia with toxic changes. Chest x-ray revealed pneumomediastinum and subcutaneous emphysema, while computed tomography showed pneumomediastinum and consolidation in the left upper lobe with cavitation (Box 2A). Electrocardiography revealed sinus tachycardia with paroxysmal atrial fibrillation. The patient was admitted to the intensive care unit with acute respiratory failure. Antimicrobial therapy was begun with ceftriaxone, flucloxacillin and aciclovir based on the clinical diagnosis of pneumonia with cavitation in a patient with active varicella. Blood cultures revealed gram-positive cocci in chains after 24 hours of incubation; intravenous clindamycin was added to the antibiotic regimen. The next morning cultures of blood and bronchoalveolar fluid confirmed the presence of Streptococcus pyogenes (group A streptococcus). Uncontrolled sepsis progressed within hours to multiorgan failure, precipitated by an overwhelming systemic inflammatory response syndrome. Clinical features were consistent with streptococcal toxic shock syndrome, with the primary streptococcal infection being pneumonia. Intensive care management included haemofiltration for acute renal failure; inotropic support for septic shock; invasive lung ventilation for respiratory failure (acute respiratory distress syndrome); activated protein C for the systemic inflammatory response syndrome; and infusion of intravenous gamma globulin for toxic shock syndrome, and fresh frozen plasma and platelets for coagulation dysfunction and thrombocytopenia. Despite maximal support, the patient died 44 hours after presentation. An autopsy confined to the chest and abdomen showed interstitial emphysema involving the mediastinum, pericardium and left upper lobe of the lung, and extensive necrotising left bronchopneumonia with abscess formation, but no signs of pneumothorax (Box 2B). Numerous gram-positive cocci consistent with streptococci were evident on microscopy of lung sections. Varicella zoster virus DNA was detected by polymerase chain reaction in one of three tissue samples tested, but no viral cytopathic changes were seen, and no virus was detected by culture. Varicella (chickenpox) is a highly infectious disease caused by varicella zoster virus. Monthly notifications in Australia fluctuate from 40 to 180, with three to four deaths yearly.1 Varicella zoster pneumonia with interstitial pneumonitis and respiratory distress syndrome is a well recognised complication which occurs more frequently in adults than children (particularly smokers, pregnant women and those who are immunocompromised). Despite the presence of varicella zoster virus DNA, there was no histological evidence that varicella pneumonia predisposed to the development of secondary group A streptococcal pneumonia in this case. In children, varicella is a well recognised predisposing condition for invasive group A streptococcal infection, preceding 15% of recorded cases of this infection in a recent Canadian study.2 The most common manifestation of invasive group A streptococcal infection is necrotising fasciitis, a serious but rare infection of the deeper layers of skin and fatty subcutaneous tissues. This is one of the fastest-spreading infections known, consuming tissue at a rate of up to 3 cm per hour.3 Pneumonia is a less common manifestation but has a higher fatality rate (38% versus 12% for necrotising fasciitis), with a reported median survival of 2 days.4 In adults, varicella is less common as a predisposing condition for invasive group A streptococcal infection, but cases have been reported of varicella gangrenosum with limb-necrotising fasciitis and toxic shock syndrome5 and of septic arthritis6 secondary to group A streptococcal infection after chickenpox. The speed of the patient’s demise and the presence of streptococcal bacteraemia and streptococci in postmortem lung tissue supports S. pyogenes as the pathogen causing death in this patient. Pneumomediastinum has many causes, particularly barotrauma and other forms of trauma, but is rare secondary to pneumonia and pulmonary abscess. We believe that this is the first case of group A streptococcal pneumonia with mediastinal gas leakage recorded in Australia. Pneumomediastinum occurring ultimately as a complication of varicella has similarly not been reported previously in this country. Primary vaccination of children is now recommended,7 but is not federally funded. Vaccination of adolescents and adults should also be strongly considered, as these age groups are more at risk of severe or lethal complications, including overwhelming secondary infection. Once invasive group A streptococcal infection is clinically suspected, it can be confirmed bacteriologically by culture of blood or affected tissue. Early medical treatment with intravenous penicillin and clindamycin is indicated. When infection is associated with streptococcal toxic shock syndrome, normal human immunoglobulin reduces organ failure and possibly mortality.8 Aggressive surgical debridement is critical for invasive fasciitis or myositis. However, in our patient, surgical debridement of affected pulmonary and mediastinal tissue was not an option. The prognosis was clearly extremely poor at presentation. The illness could have been prevented by varicella vaccination. Lessons from practice Varicella in adults is potentially serious, with a significant risk of very severe secondary bacterial infection. Varicella zoster pneumonia with interstitial pneumonitis and respiratory distress syndrome is a well recognised complication of varicella, and is more common in adults than children, particularly smokers, pregnant women and those who are immunocompromised. Varicella vaccination, although not federally funded, is recommended for children and should be strongly considered in adolescents and adults because of their higher risk of severe and lethal complications. Invasive group A streptococcal infection spreads rapidly and is often fatal; if suspected clinically, treatment should be begun urgently with intravenous penicillin and clindamycin; the addition of intravenous immunoglobulin for toxic shock should be considered, and surgical debridement for invasive fasciitis or myositis. 1 Results of blood tests in a patient with complications from varicella Test Result Normal range On admission Serum sodium (mmol/L) 121 135–145 Serum potassium (mmol/L) 3.4 3.2–4.5 Serum chloride (mmol/L) 79 100–110 Urea (mmol/L) 47.7 3.0–8.0 Creatinine (mmol/L) 0.48 0.05–0.10 Albumin (g/L) 21 33–47 Globulins (g/L) 50 25–45 Bilirubin (μmol/L) 31 < 20 Alkaline phosphatase (U/L) 144 30–120 γ-Glutamyl transferase (U/L) 308 < 50 Aspartate aminotransferase (U/L) 89 < 40 Lactate dehydrogenase (U/L) 425 110–250 Prothrombin time (s) 19 8–14 Fibrinogen (g/L) 9.9 1.5–4.0 Haemoglobin (g/L) 127 110–165 White blood cell count (cells/L) 17.3 x 109 3.5–11.0 x 109 Neutrophils (cells/L) 16.2 x 109 2.0–8.0 x 109 Platelets (cells/L) 113 x 109 140–400 x 109 6 h after admission* pH 7.01 7.35–7.45 pco2 (mmHg) 54 35–45 po2 (mmHg) 87 75–100 * After intubation and ventilation. 2 Appearance of the lungs in a patient with group A streptococcal pneumonia A: Computed tomography of the chest on admission showed subcutaneous emphysema, pneumomediastinum and consolidation in the left upper lobe with cavitation. B: Postmortem examination showed extensive necrotising left bronchopneumonia with abscess formation.

Peter JO Stride FRACP, FRCP · Matthys JJ Campher FANZCA · Janice M Geary RN, GradDipInfectionControl, GradCertHealthManagement · Christopher Coulter FRACP, FRCPA · Edwina E Duhig BMedSci, FRCPA

The IPCRG is fostering international links between primary care clinicians and researchers Over 450 primary care clinicians and researchers from around the world gathered for the second conference of the International Primary Care Respiratory Group (IPCRG) in Melbourne in February 2004. The IPCRG is an international umbrella organisation for national primary care respiratory interest groups.1 It was established as a charitable company in June 2000 by general practitioners (GPs) and other primary care health professionals from several countries, including Australia, at a meeting of the United Kingdom GP asthma group. Australian GPs have been actively involved in all aspects, including the executive, since its inception. The organisation currently has 21 member countries, represented by national organisations. Australia is represented by the National Asthma Council. The aims of the IPCRG are to provide an international network for research in community settings, to guide and disseminate evidence-based guidelines appropriate for primary care professionals (eg, GPs, nurses, pharmacists and healthcare workers), and to provide practical resources for “respiratory professionals” in community settings around the world. Those involved in IPCRG include such diverse groups as GPs, respiratory scientists, asthma educators, pharmacists and physiotherapists. The theme of the 2004 conference was “the way forward” in managing respiratory disease in primary care. Major topics were asthma, allergy and chronic obstructive pulmonary disease (COPD), while tuberculosis, community-acquired pneumonia and quality-of-life measurement also received substantial attention. Topics were covered from the perspectives of clinical care, people and public policy, and practical training, with plenary sessions, workshops and submitted papers and posters. Core issues arisingProactive models of care should be developed and tested. Most primary care respiratory management is reactive, and not organised or systematic. Different models of evidence-based proactive care need to be developed and tested. New models presented at the conference included telephone consultations for asthma review, integrated decision support, nurse-led asthma clinics, practice-based professional development programs linked to patient audits, and community-based pharmacy outreach programs. Guidelines need to be translated into daily practice. Several internationally developed guidelines for asthma, COPD and rhinitis cannot be implemented in primary care (Professor Onno van Schayck, Faculty of Medicine, University of Maastricht and University of Nijmegen, The Netherlands). Several plenary sessions discussed how best to link guidelines to clinical practice in general practice and other forms of primary care, including nurse-run asthma clinics and Aboriginal community-controlled health services. The “consensus” was that a model centred on the respiratory complaints described by the patient at presentation is more sustainable and may be more effective (eg, a model centred on “cough” may be superior to one centred on “COPD”). The link between allergy and asthma must be applied in clinical practice. Evidence is emerging that effective management of allergic rhinitis may ameliorate and prevent asthma (Associate Professor Mini Tang, Head, Department of Immunology, Murdoch Children’s Research Institute, Melbourne, and Dr Jacques Bouchard, St Joseph’s Hospital, La Malbaie, Quebec, Canada). As almost 45% of the Australian population is atopic, and about a third suffer allergic disease (Professor Robyn O’Hehir, Head of Allergy and Respiratory Medicine, Alfred Hospital, and Monash University, Melbourne, and the Cooperative Research Centre for Asthma, Sydney), a number of speakers stressed the need to consider allergy assessment in all patients with asthma. Undiagnosed asthma and chronic obstructive pulmonary disease must be identified more efficiently. Effective management is now available for people with early signs of asthma and COPD and will substantially improve their quality of life (Dr Christine Jenkins, Director, Clinical Trials Unit, Woolcock Institute of Medical Research, Royal Prince Alfred Hospital, Sydney). More precise assessment of disease severity is needed. The severity of asthma and COPD needs to be specified more precisely to maximise the benefits and minimise the risks of interventions. Smoking-cessation programs, appropriate use of inhaled corticosteroids and long-acting bronchodilators, pulmonary rehabilitation, and self-management strategies all have a role, depending on severity of the condition (Dr Christine Jenkins). Major topics discussedAsthma. With the recent release of the report on the global burden of asthma,2 Professor Richard Beasley (Director, Medical Research Institute of New Zealand, Wellington, New Zealand) highlighted the increasing prevalence of asthma, particularly in Asia. He maintained that the explanation is still uncertain, but multifactorial, with environmental factors, increasing urbanisation and the increasing prevalence of allergic disorders all implicated. This suggests that addressing the increase in prevalence will require multifaceted responses by clinicians, public health physicians, consumers, industry and governments (eg, by providing all essential drugs to treat people with asthma in all Asia-Pacific countries). He argued that primary care is a substantial part of the solution and has specific challenges, including development of simple algorithms for patients who present with vague symptoms of cough, shortness of breath and wheezing. Emerging evidence pointed to a need to ensure that the lowest dose of inhaled corticosteroid is used to control symptoms. Some newly recognised side effects include dental and vision problems. Up to 45% of people taking moderate amounts of inhaled corticosteroids report some side effects (Professor Thys Van der Molen, Department of General Practice, University of Groningen, The Netherlands). Van der Molen commented that primary-care clinicians often do not have the time or tools to identify these less well known side effects. Severe acute respiratory syndrome. Associate Professor Cheong Pak Yean (Family Physician, Faculty of Medicine, National University of Singapore) described Singapore’s response to the epidemic of severe acute respiratory syndrome (SARS). The strategy involved detecting, isolating and “ring-fencing” the virus at four levels — the border, hospital, community, and primary care. Border defence involved screening travellers for possible SARS as they entered Singapore. The hospital defence involved managing all people with SARS (or possible SARS) in hospital, with use of personal protective equipment by staff (masks, gloves, gowns, and goggles) plus barrier nursing in single-patient isolation rooms. Community defences included mass education, twice-daily temperature measurement for any suspected cases, and closure of “at-risk” gatherings, such as markets. In primary care, strategies used included GP education campaigns, telephone information hotlines, and “fever and evacuation” rooms in GP surgeries (separate rooms for patients with suspected SARS awaiting evacuation to hospital). Chronic obstructive pulmonary disease. Identifying people with early smoking-related lung damage would seem a major task. The number of people with COPD in Australia will increase significantly over the next 10–15 years; the estimated prevalence may well exceed 300 000 cases (Professor Justin Beilby, Department of General Practice, University of Adelaide, SA), and the total number of COPD sufferers (both diagnosed and undiagnosed) could range from 620 000 to 2.6 million cases.3 A GP-friendly algorithm based on the recently completed COPDX guidelines4 was presented. Fitting on two A4 pages, this has since been refined and released (Box). Symptom-based questionnaires. There was much discussion about GPs identifying specific conditions, such as undiagnosed COPD. Professor David Price (General Practice Airways Group, Professor of Primary Care Respiratory Medicine, University of Aberdeen, UK) reported on a study of the link between symptoms and results of spirometry testing in 417 current or former smokers recruited from primary-care practices in the United Kingdom and the United States. Predictors were identified that may be useful in identifying early COPD, as measured by spirometry, including: Age group (in years). Pack-years smoked (How many cigarettes do you currently smoke each day [if you are an ex-smoker, how many did you smoke each day]? What is the total number of years you have smoked cigarettes?). Recent cough (Have you coughed more in the past few years?). Breathing-related work loss (During the past 3 years, have you had any breathing problems that have kept you off work, indoors, at home, or in bed?). Hospitalisation for breathing problems (Have you ever been admitted to hospital with breathing problems?). Recent breathlessness (Have you been short of breath more often in the past few years?). Cold usually goes to the chest (If you get a cold, does it usually go to your chest?). It was agreed that these questions require validation among other communities before they can be used as predictors in everyday clinical practice. ConclusionThis was the second IPCRG conference, the first being held in Amsterdam in 2002. The developing international IPCRG networks have now begun developing innovative research programs, such as further validation of the symptom-based questionnaires discussed above. These programs will become the platform for the next conference, to be held in Oslo in 2006. The IPCRG research subcommittee has begun developing a strategic plan for the group — a challenge, because of the diversity of the member countries and the variable access to resources and research expertise across the group. However, meeting the challenge through sharing expertise and skills may also be a great opportunity for IPCRG. Main steps of COPDX checklist for diagnosis and management of chronic obstructive pulmonary disease5 C – Confirm diagnosis Presence and history of symptoms Smoking – history and willingness to quit Spirometry – measure FEV1 and FEV1/FVC and assess reversibility of airflow limitation O – Optimise function Including check of smoking status, optimal therapy and exercise status P – Prevent deterioration Essential steps (including pneumococcal and annual influenza vaccination) Risk-factor reduction (including help with smoking cessation) D – Develop self-management plan Including referral for pulmonary rehabilitation or to respiratory physician or hospital, if appropriate X – Manage eXacerbations Including ensuring understanding of importance of early treatment for exacerbations; regular review

Justin J Beilby MD, FRACGP · Nicholas J Glasgow MD, FRACGP, FAChPM · H John Fardy DRCOG, FRACGP

Only when spirometry is performed in a uniform way can we expect its widespread use in primary care Spirometry appears to be an undervalued investigation in general practice, despite its capacity to inform clinicians about diagnosis, severity assessment, and optimal treatment for airways disease. The omission of spirometry from a thorough assessment of patients with breathlessness seems just as inappropriate as failing to measure the blood sugar level in a patient with thirst, polyuria and blurred vision. There are substantial individual and community risks in not performing a simple diagnostic test such as spirometry. In Australia, underdiagnosis of chronic obstructive pulmonary disease (COPD) and asthma is a documented consequence of this.1,2 Yet, anecdotal reports from general practitioners suggest that it is difficult to incorporate spirometry into the consultation, and there have been variable outcomes after systematic efforts to teach optimal performance of the test.3,4 There are many reasons for this, including the complexity of properly performing the test, the cost of equipment, the time taken to perform bronchodilator reversibility testing, and controversy regarding interpretation of results. Although Australian guidelines for the diagnosis and management of asthma and COPD5,6 clearly define the central role of spirometry in making a diagnosis and assessing severity, the practical implementation of the test remains a challenge. Bronchodilator reversibility testing should inform the clinician about the presence and severity of airway obstruction and its reversibility in response to a standard dose of bronchodilator. Once this information is reported, the clinician can determine — in combination with the other information available — whether asthma or COPD is likely. There is considerable overlap in the bronchodilator responsiveness of these two diseases, so that spirometry may not be diagnostic. However, the consistent performance and interpretation of any test is essential to maximise its value, allow comparison of results and to ensure its sensitivity and specificity are maintained. In this issue of the Journal (page 610), Borg et al report the results of a survey of 60 lung-function laboratories in Australia and New Zealand, and highlight marked differences between laboratories in performance and interpretation of bronchodilator reversibility testing.7 These variations, in a setting where rigorous quality assurance and standardisation would be expected, indicate that substantial work is needed to bring uniformity to spirometry and establish commonly agreed criteria for assessing reversibility of airway obstruction. Do such criteria exist? There are international guidelines for the performance and interpretation of lung-function tests,8,9 and respiratory laboratories would generally aim to achieve these standards, although they may be more difficult to attain in primary care. Recommendations for assessing reversibility are given in a Thoracic Society of Australia and New Zealand (TSANZ) position paper.10 These are similar to the American Thoracic Society (ATS) standards, which indicate that a 12% increase in forced expiratory volume in 1 second (FEV1) over baseline and a minimum 200 mL improvement in FEV1 or forced vital capacity (FVC) constitute a positive response to bronchodilator. However, the TSANZ guidelines lack detail, particularly with regard to the type, dose and timing of bronchodilator administration, the factors that varied most between laboratories. By contrast, the ATS guidelines indicate that bronchodilator reversibility should be assessed by use of a short-acting β2-agonist, equivalent to 200 μg salbutamol or 500 μg terbutaline by a metered-dose inhaler. Although laboratories may choose to enhance the sensitivity of the test and optimise delivery of β-agonist by using spacers, it does not appear appropriate to administer high doses of combination bronchodilators by nebuliser for conventional reversibility testing.11 It is also outside current ATS guidelines for standardisation of reversibility testing. Is this variability between respiratory function laboratories of concern, and what are the implications? Firstly, comparisons between results from different laboratories should not be made with the assumption that the test has been performed under identical conditions. Secondly, it is essential for clinicians referring patients to respiratory laboratories to know the local features of spirometry testing to be able to interpret the results appropriately. Thirdly, it would be most desirable, and many would argue essential, for Australian laboratories to agree to a set of standards and apply them universally for spirometry and reversibility testing. Fourthly, implementing spirometry testing in primary care and educating and up-skilling GPs is unlikely to succeed without an agreed position on acceptable standards for performance and interpretation. Among respiratory scientists and thoracic physicians, there is a range of views on the feasibility of implementing more widespread use of spirometry in primary care. Although it is a highly desirable goal, expressly supported by the peak bodies in asthma and COPD care, there are major challenges. Many argue that accurate performance and interpretation of bronchodilator reversibility testing is difficult and that GPs should be offered a range of options, which should include greater access to laboratories and pathology services for spirometry. There is a plethora of articles which provide background information to assist in implementing quality control procedures to standardise equipment and test performance, and provide reference values and guidelines for interpretation of results. Despite these specifics, the ATS guidelines frankly acknowledge “There is no clear consensus on what constitutes reversibility in subjects with airflow obstruction”. Nevertheless, agreement should be reached regarding the way in which a standard test is performed, even if reaching agreement on its interpretation is difficult. The article by Borg et al highlights the urgent need for agreed standards in Australia for spirometry. The TSANZ and the Australian and New Zealand Society of Respiratory Scientists are in the best position to take up this urgent task. Transferring this expertise into community practice, either in specialist or in primary care, remains a challenge that must be met if we are to maximise the possibilities for diagnosing and managing airways disease.12 One of the perceived hurdles to this process is the Medicare Benefits Schedule descriptor for office spirometry (Item 11506), which specifies that the test should be done before and after administration of bronchodilator to attract payment. The TSANZ and the Royal Australian College of General Practitioners have prepared a submission to have this descriptor changed to allow payment for testing before or after administration of bronchodilator. The availability of a wide range of affordable, electronic spirometers with built-in software for determining reference values, along with a “Buyers guide to spirometry”, currently being written, will add to the educational resources needed to help GPs in their use of spirometry for assessing patients with breathlessness. Standardised guidelines should greatly assist the implementation of spirometry in primary care and result in more appropriate treatment and better outcomes for patients.

Christine Jenkins MB BS, MD, FRACP · Iven Young MB BS, PhD, FRACP

Objectives: To determine the variation in the methods used to assess and interpret the reversibility of airflow limitation in lung-function laboratories throughout Australia and New Zealand.Design: A postal survey performed in 2000, requesting details of methods used to assess and interpret bronchodilator reversibility.Setting and participants: 60 lung-function laboratories identified from the Australian and New Zealand Society of Respiratory Science mailing list.Main outcome measures: Bronchodilator agent, dose, mode of administration, time to repeat spirometry and definition of a significant response.Results: 37 laboratories responded (response rate, 64%). Thirty-three laboratories used salbutamol as their routine bronchodilator agent. Twenty-four laboratories used a metered-dose inhaler (MDI) with (21) or without (3) a spacer device as the preferred mode of bronchodilator administration. There was wide variation in the bronchodilator dose administered (median, 400 μg; range, 200–800 μg salbutamol for MDIs) and the time to repeat spirometry following bronchodilator administration (median, 10 min; range, 4–20 min). Ten laboratories used criteria consistent with either the National Asthma Council or Thoracic Society of Australia and New Zealand COPDX guidelines to define a significant bronchodilator response, and two used American Thoracic Society criteria. The remaining 25 respondents listed a variety of other criteria.Conclusion: The methods used to assess and interpret acute bronchodilator reversibility in lung-function laboratories in Australia and New Zealand vary considerably. This may have a significant effect on the diagnosis and management of patients. Laboratories should report the method used to assess bronchodilator response.

Brigitte M Borg BApplSc · David W Reid MB BS, FRACP · E Haydn Walters DM, FRACP · David P Johns PhD, FANZSRS

Elizabeth J Comino Senior Research Fellow, School of Public Health and Community Medicine, University of New South Wales, Liverpool Hospital, Liverpool, NSW. E. CominoATunsw.edu.au To the Editor: The Australian Bureau of Statistics recently released details of asthma mortality for 2002. These figures indicate that asthma mortality has continued to decline in 2002 and that deaths in young people aged 5–34 years are at their lowest level since the early 1950s (Box). In this age group, the number of deaths fell from 43 in 2001 to 33 in 2002 (a 23.3% drop), and for all ages the number of deaths fell from 422 in 2001 to 397 in 2002 (a 5.9% drop). This suggests that the various asthma awareness activities, spearheaded by the National Asthma Council and other interest groups, have been successful in raising awareness of asthma and its management. Or does it? Emerging evidence suggests other changes in the epidemiology of asthma in Australia. Robertson recently reported a 26% decrease in the prevalence of asthma in Melbourne school children between 1993 and 2002, but increased reporting of rhinitis and eczema over the same period.1 The significance of these findings is difficult to interpret without measures of airway function. A second study supported these results and also observed a small decline in the prevalence of parent-reported asthma, but found little change in atopy or airway hyperresponsiveness.2 Age-adjusted hospital separation rates for asthma decreased by 31.1% in young people and 31.4% in all ages between 1989–90 and 1999–00.3 There is little evidence of improved management of asthma in the general practice setting. Data published from the BEACH (Bettering the Evaluation and Care of Health) survey of general practice activity indicates a significant reduction in rates of presentation for asthma among children but not adults, with no changes in indicators of severity over time.4 Our recent research in south-western Sydney, examining uptake of the “asthma 3+ visit plan”5,6 by general practitioners and their patients, is disappointing. It suggests reluctance on the part of both GPs and patients to participate in the plan. Clearly, there remains much that we do not understand about the natural history of asthma.7 We need to continue to monitor asthma through regular surveys and routine data collection in order to understand more about fluctuations in asthma prevalence, the relationship to changing child-rearing practices (such as use of childcare facilities) and the impact of management practices. Asthma mortality in Australians aged 5–34 years, 1920–2002* * Points on the graph represent 3-year “moving” averages — for example, the 2001 value is the average of 2000, 2001 and 2002 data; the 2000 value is the average of 1999, 2000 and 2001 data, etc. This technique is used to smooth annual fluctuations that occur in data of this kind.

Elizabeth J Comino

Nicola Petrosillo,* Vincenzo Puro,† Giuseppe Ippolito‡ * Clinical Director, † Epidemiologist, ‡ Scientific Director, National Institute for Infectious Diseases “L. Spallanzani”, Rome, 00149, Italy. petrosilloATinmi.it To the Editor: In their article describing the Australian experience of border screening for severe acute respiratory syndrome (SARS), Samaan and coworkers add new insights about the low efficacy of this measure in identifying SARS cases at entry into a country.1 To our knowledge, this is the first report on this issue from a low-risk area for SARS.2 Indeed, as summarised by Samaan et al, other available data derive from countries where people with SARS, entering at the early stage of the epidemic, generated a sustained local transmission of SARS-associated coronavirus (SARS-CoV) disease. Among the reasons for a low sensitivity of entry screening, Samaan suggests that subjects may evade screening by making false declarations or by taking anti-pyretic drugs, or by simply being in the incubation period with no symptoms or only mild symptoms. To contribute to this debate, we report the experience of our Institute, which was designated as a referring centre for SARS by the Italian Ministry of Health. In Italy, where only four imported probable cases of SARS were identified and no local transmission occurred,3 entry screening was implemented at the two international airports of Milan and Rome. In particular, travellers and crews arriving from World Health Organization SARS-designated areas, directly or after transiting in other EU countries, were provided with health alert cards and screened for body temperature.4 Suspected SARS cases identified at Rome airport were to be referred to our institute. However, of the 72 subjects attending our admission unit for clinical evaluation for possible SARS, none was referred by the airport authorities. Among these patients was one of the four people with SARS arriving in Italy: an airline flight crew member coming from a SARS-designated area who passed both exit and entry screening, despite complaining of mild fever before his departure. He was admitted 6 days after arrival, at which time the clinical picture had evolved into full-blown SARS. He was discharged after 2 weeks. Among the measures recommended by WHO to reduce SARS-CoV spread, the identification of symptomatic subjects at border departure screening was the only measure with some evidence of efficacy, although this only reduced on-flight transmission.5 Conversely, evidence from several sources, including Samaan et al, showed that screening travellers (visual inspection and screening for fever) as they disembark identifies very few SARS cases and is of questionable value.1 We agree with the conclusions of Samaan et al that, in the light of a possible resurgence of SARS or similar diseases (avian flu), entry screening should, at least, be more focused, and needs further evaluation, including cost-effectiveness analysis.

Nicola Petrosillo · Vincenzo Puro · Giuseppe Ippolito

Stephanie L Jones,* Eugene Athan,† Daniel O’Brien,† Stephen R Graves,‡ Chelsea Nguyen,§ John Stenos¶ * Infectious Diseases Registrar, † Infectious Diseases Physician, Geelong Hospital, Ryrie St, Geelong, VIC 3220; ‡ Medical Microbiologist, § Scientist, ¶ Senior Scientist, Australian Rickettsial Reference Laboratory, Barwon Health, Geelong, VIC. StephljonesATyahoo.com To the Editor: Murine typhus (caused by Rickettsia typhi) has not been previously described in the state of Victoria, although it is well known in Western Australia, Queensland and South Australia. In 2002, a 49-year-old man presented to Geelong Hospital, Victoria, with a 10-day history of fever, myalgia, rigors, headache, rash, sore throat, dry cough and pleuritic chest pain. On examination, he had a fever (temperature, 39.2°C), hypoxia (oxygen saturation, 91% in room air), tachycardia, a central maculopapular rash and conjunctivitis. Blood tests revealed hyponatraemia, thrombocytopenia, white cell count in the reference range, with left-shifted neutrophil change (toxic granulation and increased immature forms) and a C-reactive protein level of 377 mg/L (reference range, < 10 mg/L). The patient lived on a hobby farm close to Geelong. Two weeks before becoming unwell, he had cleaned out the contents of a shearing shed, including two rotten sheepskins in which rats had been nesting. He reported generating a lot of dust and debris in the air. He had not noticed any tick, flea or other insect bites. Serological testing was performed for rickettsia. Baseline serum, taken 10 days after symptom onset, showed antibodies to the typhus group of rickettsiae, R. typhi (murine typhus) and R. prowazekii (epidemic typhus), with a titre of 2000. The titre rose over the following 4 days to 64 000, a fivefold increase, diagnostic of typhus group infection. Antibody titre to the spotted fever group of rickettsiae was significantly lower (peak titre, 8000). The patient was treated with oral doxycycline and recovered completely. Murine typhus was first described in Adelaide in 19221 and is now considered endemic in parts of Western Australia and Queensland.2,3 A possible case reported from Melbourne4 was, in retrospect, probably Brill–Zinsser disease (relapsed epidemic typhus). Murine typhus has an incubation period of 8–16 days and is generally self-limiting, although fatalities have occurred.1 The disease typically presents with fevers, prominent myalgia, a central rash, nausea, conjunctivitis, and often significant pulmonary involvement. Unlike the tick-borne spotted fever group of rickettsiae, R. typhi is transmitted by rodent fleas. Transmission occurs either by aerosolisation and inhalation of infected flea faeces, often during demolition or cleaning of rat-infested environments, or, less commonly, by inoculation of faeces into a fleabite. Murine typhus is usually diagnosed retrospectively by serological testing using microimmunofluorescence. Antibodies are usually detectable 7 to 9 days after disease onset, and IgG may persist for years. Cross-reactivity is seen between R. typhi and R. prowazekii; it is not possible to identify the pathogen by serological testing alone.5 Specific diagnosis is based on known local epidemiology and, as epidemic typhus does not occur in Australia, we believe this was a case of murine typhus, the first described in Victoria.

Stephanie L Jones · Eugene Athan · Daniel O’Brien · Stephen R Graves · Chelsea Nguyen · John Stenos

Kirsty L Buising,* Karin A Thursky,† James F Black,‡ Graham V Brown§ * Clinical Research Fellow, † Infectious Diseases Physician, ‡ Head of Epidemiology, § Head, Victorian Infectious Diseases Service, Royal Melbourne Hospital, Grattan Street, Parkville, VIC 3050. Kirsty.buisingATmh.org.au To the Editor: The 2003 Australian guidelines on antibiotic therapy suggest that the Pneumonia Severity Index (PSI) may be used to triage site of care and antibiotic selection for patients with community-acquired pneumonia.1,2 The PSI was developed as a mortality prediction tool, using data from over 14 000 patients with community-acquired pneumonia.3 The antibiotic guidelines suggest specifically that PSI classes I and II represent patients suitable for outpatient therapy, and that class V can identify patients likely to require intensive care and broad-spectrum antibiotic therapy. We believe this is beyond the previously recommended applications of the PSI and advise caution about its use to identify patients with severe pneumonia. In the cohort used to validate the PSI, only 32% of patients with severe pneumonia (requiring intensive care) were in class V, indicating that the PSI has poor sensitivity for severe pneumonia.3 This finding has been reflected in other studies.4 The strength of the PSI lies in its ability to identify low-risk patients, as the title of the validating article suggests.3 The PSI is so heavily weighted by age and comorbidities that younger patients needing intensive care are unlikely to accumulate enough points to reach class V. This is important, as early identification of patients with severe pneumonia and initiation of broad-spectrum antibiotic therapy and intensive-care support improves outcomes. We are concerned that the PSI may be widely accepted for a purpose for which it was not intended and has not been validated. In underestimating the severity of illness in two-thirds of patients with “severe pneumonia”, the guidelines may provide false reassurance, while clinicians may lose confidence in the PSI if they find it “misses” most patients requiring intensive care. Current evidence does support use of the PSI to guide decisions about inpatient or outpatient therapy. However, the modified British Thoracic Society (BTS) Severity Score is a simpler, better-validated tool to identify patients with “severe pneumonia” who are likely to need intensive care assessment5 (Box). This tool is useful for junior staff to “flag” patients with potentially severe pneumonia and ensure that they are discussed with a senior clinician. As always, the final management and antibiotic selection should be guided by clinical judgement. We believe that the antibiotic guidelines are valuable to encourage appropriate antibiotic use; our aim is to promote discussion of their content relating to this particular condition. Suggested alternative approach to assessing patients with community-acquired pneumonia Step 1: Does the patient need admission to hospital? Assess with the Pneumonia Severity Index (PSI). Class I or II: consider outpatient management (but also need to consider comorbidities, social supports, likelihood of compliance). Class III-V: likely to need inpatient management. Step 2: Does the patient need admission to the intensive care unit? Assess with the modified British Thoracic Society (BTS) Severity Score. Class as severe if two or more of the following features are present on initial assessment or within 24 hours of presentation (and are not attributable to another cause): Confusion (acute onset) Serum urea level > 7 mmol/L Respiratory rate ≥ 30 breaths/minute Systolic blood pressure < 90 mmHg or diastolic blood pressure ≤ 60 mmHg If severe, discuss the case with a senior clinician and consider intensive-care review and aggressive broad-spectrum antibiotics.

Kirsty L Buising · Karin A Thursky · James F Black · Graham V Brown

A 28-year-old man presented with a cough of one month’s duration and intermittent fever. Physical examination showed reduced breath sounds and fine inspiratory crackles at both lung bases. A chest x-ray showed diffuse micronodules in both lung fields, and a computed tomography scan revealed tiny calculi (calco-spherites) in the alveolar air spaces (Box). Pulmonary alveolar microlithiasis is a rare disease of unknown aetiology; about 400 cases have been reported. Patients can present with any of a variety of chest symptoms (eg, a non-productive cough and dyspnoea on exertion). Most reported cases involve people in the 20 to 50 years age group. About 50% of cases are familial. There is no definitive treatment, but disodium etidronate has been tried. The prognosis is variable. It has been suggested that, in many patients, the microliths continue to form and increase in size as the disease progresses. When the disease does progress, it may do so very slowly — patients have been reported in whom respiratory failure and death ensued after a period as long as 40 years.

Bobby Bhalotra MB BS, MD · Atul Gogia MB BS · Pratibha Gupta MB BS · Neeraj Jain MB BS, MD

Viral bronchiolitis is the commonest cause of hospital admission in young infants. Respiratory syncytial virus is responsible for most cases of bronchiolitis. Secondary bacterial infection is rare and antibiotics are seldom necessary. Most children with bronchiolitis develop only mild illness and can be managed at home. Infants born prematurely, those with pre-existing cardiac or respiratory disease, and infants in the first three months of life are more likely to need hospital admission. On current evidence, nebulised adrenaline, inhaled and systemic corticosteroids, and inhaled bronchodilators do not have a role in the routine management of infants with bronchiolitis.

Dominic A Fitzgerald MB BS, PhD, FRACP · Henry A Kilham MB BS, FRACP

Clinical records Patient 1 A 5-month-old girl presented with biphasic stridor (ie, stridor present during inspiration and expiration) and feeding difficulties since birth. The stridor was not related to her position or to her level of wakefulness. Asthma treatment with bronchodilators, inhaled corticosteroids (up to 500 μg/day fluticasone propionate) and courses of oral steroids were prescribed by several doctors from 6 weeks of age for her “noisy breathing”. These treatments did not alter her symptoms. No investigations were undertaken. There was no significant perinatal or other medical history. The child appeared well; she was thriving and her development was appropriate for her age. The biphasic stridor was not associated with wheeze or tachypnoea. She had mild tracheal tug and chest wall recession. Her chest was clear, and the findings from the remainder of the examination were normal. A chest x-ray (CXR) showed a right aortic arch and reduced air–tissue interface at the carina, consistent with tracheomalacia. A barium swallow showed a posterior indentation of the mid-oesophagus, suggestive of a vascular ring (extrinsic compression of the oesophagus and trachea by aberrantly sited blood vessels [Box 1]). Bronchoscopy confirmed these findings and showed significant reflux oesophagitis. A magnetic resonance angiogram confirmed the presence of a vascular ring formed by a double aortic arch, with each arch giving rise to its own common carotid and subclavian arteries. Surgical correction of the compressive vascular band on the trachea 3 days later was uneventful. The proton pump inhibitor omeprazole was prescribed for the reflux oesophagitis, but this proved insufficient. After further hospitalisations for pulmonary aspiration, a fundoplication was performed and a gastrostomy tube inserted. Twelve months later, she remains asymptomatic with normal growth. Patient 2 A 9-week-old girl presented with biphasic stridor and a 3-week history of intermittent central cyanosis associated with feeding. She was noted to have had “noisy breathing” from birth. At age 6 weeks, she had been admitted to another hospital with cough and more prominent biphasic stridor. Her respiratory difficulties were attributed to bronchiolitis caused by respiratory syncytial virus. She required supportive treatment including oxygen for 5 days in hospital. On discharge, she improved, but continued to have very noisy breathing, which was worse when she was active or feeding. She was bottle fed with infant formula and noted to have occasional episodes of transient cyanosis during feeding. On examination, she appeared well and was thriving. She had a moist cough, moderate subcostal recession and audible biphasic stridor. A CXR showed a left, normally sited aortic arch and poor delineation of the distal tracheal air column suggestive of tracheomalacia. A barium swallow (Box 2A) showed significant oesophageal compression anteriorly and posteriorly, consistent with a double aortic arch. A magnetic resonance angiogram (Box 2B) confirmed the presence of the double aortic arch, as well as extrinsic, anterior compression of the distal trachea. Bronchoscopy, performed before surgery to divide the vascular ring, showed mild tracheomalacia. The infant was discharged a week later with reduced stridor and recession. No further episodes of central cyanosis with feeding occurred during the 6-month follow-up period after surgery. Biphasic stridor from birth or early infancy suggests fixed proximal airway obstruction, which may be intra- or extrathoracic. Conversely, variable inspiratory stridor suggests a less severe, extrathoracic, dynamic obstruction. Patients with biphasic stridor (such as that caused by a vascular ring) are often initially misdiagnosed as having asthma because of noisy respirations, although stridor is never a sign of asthma, but rather of proximal airway compromise. Stridor differs from wheeze in that it has a different pitch and harsher sound than the more musical pitch of a wheeze. Stridor is heard predominantly during inspiration. Children with a vascular ring usually present in infancy with non-specific symptoms of dyspnoea, cough, inspiratory or biphasic stridor, and sometimes an expiratory “wheeze” (presumably related to downstream obstruction of the intrathoracic trachea) as well as feeding problems.3-5 A vascular ring occurs when one or more aortic arch abnormalities, with or without a patent ductus arteriosus or ligamentum, produce a ring that completely encircles the trachea and oesophagus, leading to symptoms of tracheal or oesophageal compression6 (Box 1). Differential diagnosesThe differential diagnosis of persistent biphasic stridor in an infant includes severe laryngomalacia, tracheomalacia and, less commonly, vocal cord paresis (causing a hoarse cry), subglottic haemangioma (causing rapidly progressing stridor, sometimes associated with a facial haemangioma) and vascular ring. Laryngomalacia is the most common cause of neonatal inspiratory stridor, but is an unlikely cause of biphasic stridor from birth, unless it is very severe. Stridor in laryngomalacia more commonly occurs after several weeks of age, is usually limited to inspiration and varies with posture and airflow (eg, it is louder with crying). Signs usually gradually resolve without treatment by 12 to 18 months of age.7 Tracheomalacia, a condition characterised by weakness of the tracheal walls and supporting cartilage (localised to the region of external compression by blood vessels), commonly occurs in association with lesions such as a vascular ring and persists for several years until the tracheal cartilage firms. The formation of the ring depends on the preservation or deletion of specific segments of the rudimentary aortic arch complex, or the presence of major arteries with anomalous origins or remnants (eg, ligamentum arteriosum) compressing the trachea and oesophagus. The double aortic arch is the most common form of vascular ring,4 and is characterised by persistence of both embryonic aortic arches, with separate carotid and subclavian arteries originating from each arch. The ascending aorta bifurcates anterior to the trachea to form the aortic arches, and each courses either right or left of the trachea and the oesophagus. The larger of the two arches usually crosses posterior to the oesophagus and unites with the other arch in the posterior mediastinum to form the single descending aorta. This can be seen as a posterior indentation on the mid-oesophagus on a barium swallow (Box 2A). When biphasic stridor is detected in an infant, a chest x-ray (CXR) and a barium swallow are simple initial investigations that together will usually confirm or exclude a vascular ring as the underlying cause.8-10 The CXR may show a right-sided aortic arch, a poorly visualised distal trachea, or another cause for tracheal compression or deviation (eg, a mediastinal mass). A barium swallow may show abnormal indentations on the posterior oesophageal wall. If these abnormalities are detected, referral for more detailed investigations (including bronchoscopy and magnetic resonance angiography) and treatment is appropriate.11,12 Tracheomalacia may occur in isolation, and the diagnosis often relies on the bronchoscopist’s interpretation of the airway calibre and shape at bronchoscopy. ManagementAfter surgical treatment for a vascular ring, it is essential that the child’s parents receive ongoing management advice, as tracheomalacia will persist, and there is therefore an increased risk of severe croup. The child may continue to display noisy breathing for a period of a few months to several years13 and should be followed up until school age. In school-age children, any residual inspiratory flow limitation can be quantified using inspiratory flow volume loops with spirometry. Residual tracheomalacia commonly results in difficulty clearing airway secretions (impaired mucociliary clearance) through the functionally narrowed section of trachea at the site of the previous extrinsic wall compression. This may manifest as a brassy, rattly cough that persists longer than expected after a viral infection. It would be appropriate to consider influenza and pneumococcal vaccination in these children. Lessons from practice Stridor is never a feature of asthma and can be differentiated from wheezing by its predominance during inspiration, its harsher sound and different pitch. Biphasic stridor suggests fixed intra- or extrathoracic proximal airway obstruction. Any infant with biphasic stridor should have a chest x-ray and a barium swallow to detect the presence of a rare congenital anomaly in which aortic arch or large blood vessel abnormalities produce a ring encircling and compressing the trachea and oesophagus. Management of patients with vascular ring must include postoperative advice concerning the ongoing tracheomalacia and the associated risk of croup, as well as possible continuation of noisy breathing and impaired mucociliary clearance, which may prolong a viral-induced, rattly cough. 1: A double aortic arch (as in Patient 1) The simplified anatomy of a double aortic arch, which produces a vascular ring encircling the trachea and oesophagus, causing symptoms of tracheal or oesophageal compression. 2: Investigations for biphasic stridor (Patient 2) 2A: Barium swallow, showing anterior (arrow A) and posterior (arrow B) compression of the oesophagus, together with a posterior bulge (arrow C) caused by a double aortic arch. 2B: Magnetic resonance image, showing the double aortic arch — superior vena cava (A), brachiocephalic vein (B), pulmonary arteries (C, D), prominent left aortic arch (E), and smaller right aortic arch (F).

Sami Spencer · Belinda H Yeoh MB BS · Peter P Van Asperen MD, FRACP · Dominic A Fitzgerald MB BS, PhD, FRACP

Can we solve a riddle by burying an illusion? Over the past 40 years, the prevalence of asthma appeared to rise inexorably in both the developed and the developing world. So the report by Robertson and colleagues in this issue of the Journal (page 273), showing a decline in reported symptoms in children since 1993,1 is to be warmly welcomed. It comes with supporting evidence, in the form of reduced hospital admissions for asthma in Victoria, but also with a catch. The prevalence of reported hay fever and eczema has increased over the same period. Why the prevalence of asthma increased and why it may now be stabilising or declining is baffling. Robertson et al suggest one possibility may be the increased use of daycare facilities. The “hygiene hypothesis” — the inverse relationship between microbial or infection exposure and allergic disease — was first suggested by Gerrard et al while studying allergic disease in the Metis Indian community in Canada.2 Strachan refined this observation from large UK cohorts, in which he found an inverse relation between the number of older siblings and the prevalence of hay fever, but not asthma.3 He emphasised that the link was through atopy to allergic disease. Several studies have subsequently shown reduced asthma prevalence in school-aged children with early daycare attendance. These children tend to show more early wheezing, but less asthma later on. The proposed explanation is that they contract more upper respiratory tract infections from close contact with children, and this leads them to have more early wheezing, but, in turn, protects them from later developing atopy and atopic disease. This explanation seems less likely in the study by Robertson et al, given that the prevalence of hay fever and eczema increased while asthma prevalence decreased. However, one would need to know the atopic status of the children to clarify the issue. The 26% reduction in current wheeze prevalence in Melbourne, while significant, is within the range found in Australia in phase 1 of the International Study of Asthma and Allergies in Childhood (ISAAC) study. For example, in the four Australian centres that took part, there was a 19% difference between the highest and lowest prevalence. For sleep disturbance and speech limitation, this was 40%.4 There is even greater variation in large cities. In Mumbai (Bombay), for example, reported current wheeze varied by more than 100% in different parts of the city. Large cities are not homogeneous, with large variations in many factors that may affect wheezing, such as housing conditions, smoking prevalence, diet and variable access to healthcare. Over time, with gentrification and changes in zoning, factors may vary even in the same narrow geographic location. Robertson et al also note that awareness of asthma in the community is a determinant of the prevalence of reported symptoms. The pool of individuals with a history of symptoms in any population will be larger than the proportion who report symptoms in a defined period. In a resurvey of 700 young adults, all of whom had 4 years previously responded positively to at least one of three asthma questions, only two-thirds reported symptoms. In the first survey, 28% had responded positively to all three questions, and in the second, 29%. However, only 60% were the same individuals.5 Just how large this pool is has recently been clarified, at least in New Zealand. In a 26-year follow-up of a birth cohort in Dunedin, 73% reported wheezing on at least one occasion and 51% on at least two.6 Given that there is likely to be some loss to recall, wheezing at some time between birth and adulthood appears virtually universal. Clearly, most of this wheezing is occasional, trivial, inconsequential, and a normal phenomenon. It is now easy to see how a change in diagnostic and hence societal emphasis on wheezing, as opposed to bronchitis, with attendant changes in treatment, can enhance recall for asthma in cross-sectional surveys. Perhaps this emphasis is now stabilising and the diagnostic label “asthma” is being applied slightly less frequently in Melbourne. The strengths of the ISAAC approach — simple questionnaires requiring minimal funding — allow large-scale international comparisons of children (in fact, this is the only way that such large population studies can be conducted), but interpreting relatively small changes over time in a very asthma-“savvy” environment like Australia is more difficult. Although the ISAAC approach clearly shows that reported asthma symptoms are far more frequent in Australia, New Zealand and the United Kingdom compared with, say, Albania or India (more than 10-fold), smaller differences over relatively short time intervals in individual countries are harder to interpret. In the past 30 years there have been many cross-sectional studies reporting an increase in the prevalence of asthma symptoms over time. In only two has this been accompanied by measures of airway hyperresponsiveness. In the first, in the United Kingdom, Burr et al showed that current asthma symptom reporting had doubled over a 15-year period, but exercise-induced fall in peak expiratory flow rate had not changed.7 In the second, Peat et al showed a doubling of current symptoms and airway hyperresponsiveness, predominantly among atopic children, suggesting an increase in asthma relating to greater allergen exposure.8 There is a real need for the measurement of objective markers over time in similar populations. The advent of easily obtainable markers of airway inflammation, such as exhaled nitric oxide or constituents of breath condensate, may allow population studies of airway inflammation to be quantified and tracked over time. Perhaps it is time to abandon our inconvenient population model of asthma as a disease, just as doctors did for essential hypertension in the 1950s.9 Nature provides no obvious support for asthma and we can neither define nor measure it accurately; indeed, it remains a mystery because it is largely an illusion. The late Geoffrey Rose (Professor of Epidemiology at the London School of Hygiene and Tropical Medicine) suggested that we should seek the answers to “disease” by exploring populations rather than atypical minorities.10 We need to consider applying this concept to airway inflammation. Now that appropriate tools are becoming available, we should measure airway inflammation and airway responses in large populations and explore the environmental and genetic factors that affect the airway at a population level. This might reveal that it is not just the upper end of the distribution of airway inflammation (which we arbitrarily and inconsistently call asthma) that varies by environment, and over time, but the whole distribution. In the meantime, Robertson and colleagues have shown that parent-reported asthma symptoms of young Melbourne children have declined in the past decade. This may or may not be an early signal for a real decline in asthma prevalence. What it does suggest is that asthma prevalence has not increased, which in itself is a welcome observation.

Julian Crane MB BS, FRCP, FRACP