Volume 206 Issue 5

There have been marked improvements in treatment options but none have yet been shown to induce remission

Matt Brown · Linda A Bradbury

A collaborative effort to provide real world evidence of therapies for patients with lupus

Sean O'Neill · Eric F Morand · Alberta Hoi

It is important that patients’ broader health needs are addressed

Louisa Chou · Andrew M Briggs · Anita E Wluka

Good technique and a reflective approach assist clinicians to identify an easily missed indicator of ventricular dysfunction

Subha Ramani · Brittany N Weber

An 86-year-old man presented with acute severe neck pain, restricted neck movement and fever. The white cell count, erythrocyte sedimentation rate and C-reactive protein were all elevated. After excluding an infective aetiology, review of computed tomography images of the cervical spine (Figure) showed calcification of the transverse ligament of the atlas with crown-like density around the odontoid process. This was consistent with a diagnosis of crowned dens syndrome, an uncommon manifestation of calcium pyrophosphate dehydrate deposition disease.1,2 The patient’s symptoms were self-limiting over 1 week without the use of anti-inflammatories or corticosteroids. Figure

Matthew Jiang · Sukumar Navanathan

Mindmaps in ophthalmology

Thomas D Walker

If I were a klonopin …

E Kristin Anderson

Biological disease-modifying agents are transforming the treatment of autoimmune rheumatic diseases

Flavia M Cicuttini

Planning for life after work should commence as early as possible

Brian M Draper

If high vaccine coverage is sustained, the higher rates of HPV-related cancer rates in Indigenous women should also decline

Hammad Ali · Hamish McManus · Catherine C O'Connor · Denton Callander · Marlene Kong · Simon Graham · Dina Saulo · Christopher K Fairley · David G Regan · Andrew Grulich · Nicola Low · Rebecca J Guy · Basil Donovan

Delaying retirement may be related to the primacy of work for doctors

Chanaka Wijeratne · Joanne K Earl · Carmelle Peisah · Georgina M Luscombe · Johanna Tibbertsma

SLE should be considered in anyone with inflammatory joint pain with extra-articular features

Vera Golder · Alberta Hoi

While there is no cure for rheumatoid arthritis, treatment has improved to the point where many patients can approach a normal quality of life

Graeme Jones · Peter Nash · Stephen Hall

Rheumatic diseases are frequently complicated by extra-articular manifestations, often affecting the eye; some ocular complications may be rapidly sight-threatening

Colby Hart · Merv Ferdinands · Leslie Barnsley

To the Editor:As 2017 marks the 10-year anniversary of the Australian Scleroderma Interest Group (ASIG), we want to raise awareness of the significant morbidity and mortality associated with systemic sclerosis (SSc), a chronic multisystem autoimmune disorder characterised by vasculopathy and fibrosis, with a particular focus on SSc-related pulmonary arterial hypertension (PAH).1 SSc-PAH is the leading cause of SSc-related death with a survival, once symptomatic, of only 2–3 years without treatment.2 Survival may be improved by annual screening with algorithms incorporating transthoracic echocardiogram and pulmonary function tests — even after adjustment for lead-time bias — and with early implementation of specific PAH therapies available through the Pharmaceutical Benefits Scheme (PBS).3 Thus, annual screening is recommended4 to identify patients who should undergo right heart catheterisation to confirm the diagnosis. Despite the documented benefits of PAH screening, physician adherence in Australia is suboptimal, with over 40% not adhering to annual screening.5 Therefore, the Australian Scleroderma Cohort Study (ASCS) was established in 2007 as a web-based screening platform for the cardiorespiratory manifestations of SSc, particularly PAH. The ASCS is a nationwide project where patients with SSc are recruited from 13 participating centres across Australia. Physicians who are not part of ASIG and care for patients with SSc are invited to refer patients for the screening service. Over the past 9 years, 1636 patients with SSc have been recruited and, as a result of annual screening, 194 patients (11.9%) have been diagnosed with PAH. All patients have received a specific PAH therapy. In Australia, the PBS only subsidises monotherapy (treatment with one PAH therapy at any one time) in patients with PAH who are in the World Health Organization (WHO) functional classes III or IV. International data have shown that patients treated earlier, such as those in the WHO functional class II, before damage to the right ventricle has occurred, have an improved survival rate over those treated later and those in a worse WHO functional class. Furthermore, patients treated with combination therapy (treatment with two specific PAH agents at the same time) have a survival benefit compared with those treated with monotherapy only.6 In the coming years, ASIG will continue to campaign to optimise screening and the treatment of SSc-PAH through efforts directed at closing the evidence–practice and evidence–policy gap in Australia, with the goal of improving outcomes for patients who have this incapacitating disease.

Mandana Nikpour · Susanna Proudman · Kathleen Morrisroe · Joanne M Sahhar · Wendy Stevens

To the Editor:We present a case that illustrates the need for careful medical evaluation of pigmented lesions, and the potential risks associated with laser treatment by non-medical providers. A 56-year-old nurse presented to the Victorian Melanoma Service for management of a biopsy-proven lentigo maligna on her right cheek. The patient described an 18-month history of a growing pigmented lesion that had initially been treated by a non-medical cosmetic clinic using laser. There had been no formal clinical or dermoscopic assessment of the pigmented lesion before the laser treatment. Although initially there was complete clearance of the pigment, the lesion recurred over the following 12 months (Box), prompting the patient to seek medical advice. Relevant melanoma risk factors included a family history of melanoma and significant prior sun exposure. Pigmented lesions should only be treated by medical experts given that the diagnostic possibilities range from benign to malignant pathologies, including melanoma.1 There is an increasing tendency in the aesthetic industry to treat pigmented lesions with modalities such as laser, as if they were merely a cosmetic problem. The potentially fatal consequences of laser treatment of pigmented lesions performed by untrained providers has been described in the literature.1,2 However, causation of melanoma by laser, with resultant malignant proliferation or transformation, has not been proven.2 Nevertheless, performing laser treatments on undifferentiated pigmented lesions can delay diagnosis and lead to more devastating outcomes, including metastasis.2,3 To maintain patient safety, pigmented lesions should be assessed medically before any cosmetic treatment.2,4 There is a vast array of unregulated, non-medical cosmetic practices that may use destructive treatments, such as laser, for pigmented lesions. It is therefore essential to increase the awareness of the general public in the face of this potential danger. Box – Recurring lesion after laser therapy

Harini Rajgopal Bala · Yan Pan · Rosemary L Nixon

To the Editor:I read with interest the Medical Education article by Elder and Nair1 and I was surprised that it suggested that the assessment of the jugular vein pulsation should be done “in whatever position the patient is in”. I am often shocked by the fact that medical students are taught to look for the jugular venous pulsation with the patient positioned at 45 degrees. In a person who is asymptomatic and lying flat, without any obvious distress and who has no fluid retention clinically, the pressures in the right atrium will be in the range of 2–8 mmHg and it would be impossible to see the top of the jugular venous pulsation if the patient was elevated. In most patients, the jugular venous pulsation should be assessed by examining the patient in the supine position. Elevation should only be undertaken if there is obvious fluid retention or dyspnoea when lying flat. If there is any question after examining the patient in the supine position, then the patient may be elevated to ensure that the correct peak of the jugular venous pulsation is seen. However, if students are taught to examine the jugular venous pulsation only when the patient is sitting up, then it will seldom be seen in a person who has normal right atrial pressure. I believe this concern should be brought to the attention of all clinical educators so that they clearly explain when the jugular venous pulsation should be examined in the supine position and when it is necessary to elevate the patient to the 45 degrees position.

Stanley Peter Woodhouse

In reply

Andrew Elder · Balakrishnan (Kichu) R Nair

Dr Sharmayne Brady combines research into back pain, clinical practice providing holistic care for her patients, and a balanced family life …

Cate Swannell

Three researchers from the Garvan Institute of Medical Research have received fellowships from Cancer Institute NSW to support their investigations into new therapeutic strategies for cancer. Dr Angela Chou was awarded an Early Career Fellowship. She is researching a new targeted drug in pancreatic cancer which, in initial preclinical testing, showed promise in a subgroup of tumour cells with specific genetic traits. Dr Chou plans to test this drug in a newly established model of invasive pancreatic cancer, and to explore its combination with different chemotherapies currently in clinical use, with the future aim of improving response and survival in pancreatic cancer patients. Dr David Gallego-Ortega received a Career Development Fellowship to support his work, which focuses on making immunotherapies an effective treatment for breast cancer. Immunotherapies work by “re-educating” the immune system to recognise and combat cancer cells. In breast cancers, however, the immune system’s function is “dialled down” and immunotherapies can’t work effectively. Dr Gallego-Ortega’s work will use state-of-the-art technology at single-cell level to investigate how this happens, and how to reverse it, so that the immune system can effectively target breast cancer cells. Dr Marina Pajic was awarded a Career Development Fellowship. Dr Pajic’s previous genomic analyses of pancreatic tumours identified a number of new genetic drivers of pancreatic cancer and this funding will support the preclinical assessment of several new drugs that individually target these drivers. A key aim of Dr Pajic’s work is to develop a more efficient strategy for the testing of new therapeutics that will accelerate progress into clinical trials, and ultimately lead to personalised therapy for pancreatic cancer patients. https://www.garvan.org.au/news-events/news/nsw-government-funding-for-garvan-scientists-to-research-new-treatments-for-breast-and-pancreatic-cancer Dr Anne Bruestle, from the John Curtin School of Medical Research at the ANU, has been awarded the Gordon Ada Early Career Researcher Award, and has presented the Gordon Ada Oration: Th17 cells in neuro inflammation: What we can learn from animal models of multiple sclerosis. The annual award, established in 2012 in honour of Emeritus Professor Gordon Ada, recognises early career researchers at JCSMR who have made major contributions to biomedical research. Awardees receive $1000 and are invited to present their work as the annual Gordon Ada Oration. The award capped a good month for Dr Bruestle who earlier was awarded $200 000 from MS Research Australia to develop new techniques to treat multiple sclerosis. The new project will look at how different immune cells interact with neutrophils, a type of immune cell that can combat infection by expelling their DNA to kill bacteria. The process known as neutrophil extracellular traps or NETs forms an important part of the body’s defence against infection. NETs can also mistakenly kill the body’s own cells, leading to a variety of diseases. Dr Bruestle said her team will look at how NETs induce neuronal inflammation and how new drugs could inhibit the process. The final stage of the project aim is to take the laboratory findings to patients and examine the level of neutrophils and NETs in the blood of people with MS. http://jcsmr.anu.edu.au/news-events/news/gordon-ada-early-career-researcher-award-presented-dr-anne-bruestle http://jcsmr.anu.edu.au/news-events/news/jcsmr-researcher-awarded-200000-multiple-sclerosis-research Associate Professor Peter Stanwell, from the University of Newcastle, has been awarded a Fulbright Professional Scholarship. With a 20+ year history in magnetic resonance imaging (MRI), A/Professor Stanwell will use his Fulbright Scholarship to explore the underlying changes that occur in the brain after traumatic brain injury, and how these changes predispose some individuals to later-life cognitive deterioration and neurodegeneration. There is concern amongst health care professionals that milder forms of repetitive brain injury may have long term adverse effects on cognition and development of dementia in later, adult life in high risk groups. A/Professor Stanwell’s recent work combining CLARITY light sheet microscopy and advanced MRI techniques has explored ultrastructural changes that occur following brain injury. Working with global experts in advanced MRI imaging and analysis and light sheet fluorescence microscopy, he will build on this preliminary work and help researchers gain new insights in this area. As well, he hopes to identify evidence-based MRI biomarkers to optimise medical treatment of patients following traumatic brain injuries. A/Professor Stanwell will work with A/Professor Alex Lin at Brigham and Women’s Hospital, and Dr Susan Whitfield-Gabrielli at the McGovern Institute for Brain Research at MIT to achieve these goals. http://www.newcastle.edu.au/newsroom/featured-news/uon-researcher-awarded-fulbright-professional-scholarship Professor Peter Macdonald from the Victor Chang Institute has been honoured with the NSW Ministerial Award for Cardiovascular Research Excellence for his significant contribution to improving treatment for heart failure, in particular, identifying strategies to minimise donor heart injury, reduce transplant allograft rejection and increase awareness of organ donation. He also commits his time to his translational research laboratory for testing new strategies for mechanical assistance for patients awaiting transplantation and identifying procedures for minimising donor heart injury. Also from the Victor Chang Institute, Dr Joshua Ho has been awarded the NSW Ministerial Award for a Rising Star in Cardiovascular Research. Dr Ho is a computational biologist who has already made significant contributions to biomedical research. In his very short career to date, he has already made a great contribution to mentoring of four current PhD students and two Honours students. Dr Ho has also made significant contributions to strengthening bioinformatics research locally as a founding member of the Australian Bioinformatics and Computational Biology Society (2014) and through his organisation of workshops and conferences. http://www.victorchang.edu.au/home/news-events/news/news-detail/?news_name=time-to-shine-for-victor-chang-researchers Professor Garry Jennings AO has been named as the new Executive Director of Sydney Health Partners, an advanced health research and translation centre, made up of Sydney, Northern Sydney and Western Sydney Local Health Districts; the Sydney Children’s Hospitals Network (Westmead); the University of Sydney; and nine affiliated independent medical research institutes, including the Children’s Medical Research Institute. Professor Jennings served as the director of Baker IDI Heart and Diabetes Institute in Melbourne for 14 years and most recently as the interim CEO of the National Heart Foundation. He has had a long association with the National Heart Foundation as a former board Member and chair of its Cardiovascular Health Advisory Committee. He will also remain active in his clinical and research activities. As a leading cardiologist, Professor Jennings has also had a long career in clinical practice including as the director of Cardiology at The Alfred Hospital, Melbourne. Professor Jennings also played a key role in the transformation of The Alfred campus as Foundation Chair of the Alfred Medical Research and Education Precinct. http://www.cmri.org.au/News/Latest-News/CMRI-Welcomes-Appointment-of-New-Executive-Directo Funded by the Mark Hughes Foundation (MHF), radiation oncologist Dr Mike Fay from the University of Newcastle has been named the inaugural recipient of a 3-year Hunter Medical Research Institute Mid-Career Research Fellowship dedicated to brain cancer. As a clinical expert in advanced imaging for brain tumours, Dr Fay’s research is developing scanning markers and targeted therapies for cancer cells that resist current treatments. Having trained in medical oncology before changing to radiation oncology, Dr Fay has worked in more than 30 hospitals across five countries, including the US and Germany. His research interests were sparked by a study aimed at predicting toxicity from radiotherapy – his paper won a prize from the Royal Australian and New Zealand College of Radiologists and was published in the International Journal of Radiation Oncology Biology and Physics. Along with collaborators, Dr Fay is studying high grade brain tumours that have oxygen-deprived (hypoxic) regions requiring three times as much radiation dose to treat. He believes that critical factors to treatment resistance will be found there. http://www.newcastle.edu.au/newsroom/featured-news/new-research-fellowship-dedicated-to-brain-cancer Wesley Medical Research has awarded 10 researchers specialising in the treatment and management of neurological diseases for their contribution to improving the care and quality of life of patients. The neuroscientists were awarded for researching new therapies and management techniques that may slow the progression of neurological diseases. Emeritus Professor Mervyn Eadie AO was presented with a Lifetime Achievement award for his work for over 50 years on the treatment of conditions like epilepsy and migraines. Dr Susanna Mantovani, who was recognised as one of the Emerging Leaders in Neurology Research, is comparing the sleep patterns of neurodegenerative patients with the healthy population to find ways to slow the progression of neurological diseases like motor neurone disease. Senior Research Fellowships were awarded to Professor Pamela McCombe, Professor John O’Sullivan, Professor Rob Henderson and Dr Noel Saines. Dr Richard Gordon, Dr Trent Woodruff, Dr Shyuan Ngo and Dr Frederik Steyn were awarded as Emerging Leaders in Neurology Research. Doherty Institute postdoctoral scientist Dr Amy Chung is one of six recipients internationally and the only Australian, to be awarded a prestigious Mathilde Krim Fellowship in Basic Biomedical Research for her HIV vaccine research. The fellowships are presented annually by The Foundation for AIDS Research (amfAR), in honour of the organisation’s founding chairman, Dr Mathilde Krim, to support bright young scientists seeking innovative solutions to HIV and AIDS. Based at the University of Melbourne in Professor Stephen Kent’s laboratory, Dr Chung will use her Krim Fellowship, USD$150 000 over 2 years, to study the mechanisms by which different types of antibodies interact, with the aim of producing an effective HIV vaccine. http://www.doherty.edu.au/news-events/news/dr-amy-chung-awarded-prestigious-amfar-krim-fellowship Professor Julie Quinlivan, a practising gynaecologist and obstetrician, and professor of medicine at the University of Notre Dame, has been appointed as the Director of the Professional Services Review (PSR), for the next 3 years. The PSR is an independent statutory authority that investigates whether health practitioners have provided inappropriate services that attract benefits under the Medicare Benefits Scheme. It also looks at whether medicines have been inappropriately prescribed under the Pharmaceutical Benefits Scheme. In doing so, the PSR plays an integral role in the regulatory framework of Australia’s health care system, to protect the integrity of both schemes. The Australian Medical Association has endorsed Professor Quinlivan’s appointment. http://www.health.gov.au/internet/ministers/publishing.nsf/Content/health-mediarel-yr2017-hunt006.htm The University of Queensland’s new Centre for the Business and Economics of Health has opened its doors. Acting director, Professor Luke Connelly said the centre will lead research into cost-effective health care solutions, and connect researchers and resources from UQ’s Faculty of Business, Economics and Law, Faculty of Health and Behavioural Sciences, Mater Health Services and the Mater Research Institute. A donation from two UQ alumni – Fidelity Worldwide Investments Head of Australian Equities Paul Taylor and his wife Sue – kick-started the centre in 2016. https://www.uq.edu.au/news/article/2017/02/new-uq-research-centre-puts-spotlight-health-spending

Cate Swannell