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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 14  |  Issue : 2  |  Page : 177-185
Unusual Presentations of Wegener's granulomatosis: Pitfalls in Early Diagnosis

Department of Medicine, Aga Khan University Hospital, Karachi, Pakistan

Correspondence Address:
Syed Mansoor Ahmed Shah
Department of Medicine, Aga Khan University Hospital, Stadium Road, PO Box 3500, Karachi – 74800
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PMID: 18209443

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Wegener's Granulomatosis (WG) is a disorder characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Although the cause is unknown, there is strong evidence that this is an autoimmune disease and immunosuppressive therapy with cyclophosphamide and corticosteroids efficiently relieves the symptoms and, prevents relapses. We report on four cases of WG that presented with unusual features. The first patient presented with vague joint pain, blackish discoloration of the skin and leg ulcers. The second patient presented as fever of unknown origin and gingival hyperplasia. The third patient presented with hearing loss while the fourth presented with arthritis, conjunctivitis and painful swelling of the ear. It is emphasized that WG is a complex disease and can involve multiple organ systems. Early recognition and institution of proper treatment are crucial for optimal outcome.

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