To the Editor: In their recent Snapshot, Sharma and colleagues reported an interesting case of a woman presenting with prolonged fever and inflammation of external ears, nose, and throat.1 On the basis of negative antineutrophil cytoplasmic antibodies (ANCAs), a diagnosis of relapsing polychondritis was made and the possibility of Wegener’s granulomatosis “ruled out”. Corticosteroid and azathioprine were given to the patient accordingly.

However, patients with Wegener’s granulomatosis can present with limited otolaryngological symptoms with or without positive ANCAs.2,3 As a rule, ANCAs are present in 90% of patients with the generalised form of the disease, but in only 60% of those with the limited form.4 Conversely, some cases of relapsing polychondritis can have a positive test result for ANCAs.4 A case has been reported of a Wegener’s granulomatosis patient with otolaryngological manifestations that led to an initial diagnosis of relapsing polychondritis, but who subsequently developed pulmonary and renal involvement.3

While a routine biopsy to exclude Wegener’s granulomatosis in patients with inflammatory otolaryngological symptoms may not be practical, with a limited period of follow-up, a diagnosis of relapsing polychondritis should be made cautiously and only provisionally. This is true even in cases such as the patient described by Sharma and colleagues with 6 months of illness and 7 months of follow-up, as pulmonary and/or renal manifestations could take years to follow. We believe that negative ANCAs should never be used to rule out Wegener’s granulomatosis, especially in its limited form.