EDITORIAL COMMENT: We accepted this case report not only because readers could encounter a patient with Wegener granulomatosis, but also because the use of cyclophosphamide from 22 weeks gestation did not harm the fetus. We have asked the authors to provide readers with a 100–200 word summary of the aetiology, clinical features and complications of Wegener granulomatosis. Author's response: Wegener granulomatosis is a systemic necrotizing granulomatous vasculitis of unknown aetiology with a predilection for the kidney and upper respiratory tract, especially the nose and sinuses. It classically causes acute renal failure, epistaxis, and pulmonary haemorrhage. It can however affect any organ and untreated has an 82% 1‐year mortality. It can occur at any age but is more common in elderly patients. It is very strongly associated with cytoplasmic antineutrophil cytoplasmic antibody (cANCA) which is directed against a serine proteinase 3 antigen and can be used to follow the course of the disease. Unless the patient has very localized disease, treatment always involves cyclophosphamide and high dose steroids ± plasmapheresis to obtain remission. This would then be followed by a prolonged course of azathioprine and steroid treatment.
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