Case of Wegener’s granulomatosis

A 43y old man presents with fever and arthritis. During the past 2 mo, he has been treated four times for a maxillary sinus infection. He also complains of the recent onset of hematuria. Which of the following is the most likely diagnosis?

• a.Churg-Strauss syndrome
• b.Wegener’s granulomatosis
• c.Lofgren syndrome
• d.Sjogren syndrome
• e.Sarcoidosis

** The answer is (b).

Wegener’s granulomatosis involves the upper airways (nasopharynx and sinuses) and the lungs, kidneys, and joints. The diagnosis is made by the clinical picture, a positive antineutrophil cytoplasmic antibody with a cytoplasmic staining pattern (C-ANCA), and biopsy showing necrotizing granulomas. The disease causes a systemic necrotizing arteritis and is fatal without treatment.
The typical history for Churg-Strauss syndrome(allergic angiitis and granulomatosis) is asthma followed by systemic vasculitis with eosinophilia (mnemonic is RAVE: Rhinitis, Asthma, Vasculitis, and Eosinophilia). Lofgren syndrome is a benign form of sarcoidosis that
causes bilateral hilar adenopathy, periarthritis of the ankles, and erythema nodosum of the anterior tibial regions of the lower extremities. Sjogren syndrome is a slowly progressive autoimmune disease that primarily affects middle-aged women; it affects the lacrimal and salivary glands,resulting in xerostomia and dry eyes. It may occur alone (primary) or in
association with other autoimmune diseases such as rheumatoid arthritis or systemic lupus erythematosus.