What is the most likely diagnosis? A 46-year-old man comes to the clinic with a cough that is occasionally productive of blood, diffuse muscle and joint pain in the upper extremities, and blood in his urine for the past several days. On further questioning, the patient reveals that he has had chronic sinusitis for the past several years. The patient has a lowgrade fever and nasal ulcerations.Laboratory studies show a markedly elevated erythrocyte sedimentation rate, and staining for antibodies to cytoplasmic antigens of neutrophils is positive. Biopsy of a nasal ulceration reveals vasculitis and necrotizing granulomas.

Wegener’s granulomatosis

The correct answer is E. This patient has Wegeners granulomatosis, a disease characterized by necrotizing, granulomatous vasculitis affecting several organs, most notably the upper respiratory tract, lung, and kidney. The hematuria is suggestive of glomerulitis. The presence of granulomas and the dramatic response to immunosuppressive therapy suggest that this disease process may be immunologic in origin. Elevated serum cytoplasmic antineutrophil cytoplasmic antibody is found in 90% of patients and is highly specific for the disease. Other clinical findings include skin rashes, muscle pains, and fever Answer A is incorrect. Patients with Alports syndrome may have hematuria, but the hallmarks are ocular disorders and nerve deafness from defective synthesis of collagen type IV Answer B is incorrect. Giant cell (temporal) arteritis is a type of vasculitis that affects the arteries of the head (especially, of course, the temporal arteries). The highlights of this disease can be remembered by the mnemonic JOE, because patients get Jaw pain and Ocular disturbances from ischemia to the arteries supplying them. Patients also often have markedly elevated Erythrocyte sedimentation rates. The disease is often associated with the presence of polymyalgia rheumatica Answer C is incorrect. Goodpastures syndrome is a type II hypersensitivity reaction against collagen type IV, found in the lungs and the kidneys. The disease commonly presents with concurrent hemoptysis and hematuria. Exposure to hydrocarbon solvents (such as those found in the dry-cleaning industry) and cigarette smoking have been associated with an increased risk of developing Goodpastures syndrome Answer D is incorrect. Takayasus arteritis is a vasculitis characterized by fibrotic thickening of the aortic arch (it also affects the pulmonary arteries, the branches of the aortic arch, and the rest of the aorta in up to one-third of patients). Clinically, patients often have lower blood pressure and weaker pulses in the upper extremities than in the lower extremities; some patients also have ocular disturbances as well.