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Wegener's granulomatosis is an uncommon multi-systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and general focal necrotizing vasculitis (commonly known as Wegener's triad). The diagnosis of Wegener's granulomatosis is suggested from the clinical and laboratory findings and from the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) although the absence of ANCA does not exclude the diagnosis. We described a case of 27-year-old man with pansinusitis, mild azotemia and initial respiratory tract symptoms such as foul smelling nasal discharge and epistaxis. Later he developed fever, poly-arthritis, abdominal pain and haematuria. There were multiple painful oral ulcers and skin showed multiple palpable purpuric rash. C-ANCA was positive. He was treated with IV methyl prednisolone 1gm daily for 3 days followed by oral prednisolone 1mg/kg body weight and oral cyclophosphamide. His condition improved dramatically and on follow up after 3 months he was reasonably well. In this report, we wanted to emphasize that Wegener's granulomatosis, although rare, should be considered in the above clinical scenario and treatment should be initiated as soon as possible.

Citation

M A Islam, A K Azad, M S Islam, M N Shazzad, S A Haq. Wegener's granulomatosis - a case report. Mymensingh medical journal : MMJ. 2013 Jan;22(1):196-9

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PMID: 23416832

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