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Sputum acid-fast stain
12%
6/50
Test for anti-myeloperoxidase
20%
10/50
Test for anti-proteinase 3
48%
24/50
Renal arteriogram
Sputum cytology
6%
3/50
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This patient is presenting with history of sinusitis, hemoptysis, and hematuria, suggesting a diagnosis of granulomatosis with polyangitis, which is associated with elevated anti-proteinase 3 levels. Granulomatosis with polyangiitis (formerly known as Wegener’s) is an auto-immune mediated small-vessel vasculitis. It presents with upper respiratory tract symptoms (e.g. epistaxis, oral ulcers, chronic sinusitis, otitis media), lower respiratory tract symptoms (e.g., hemoptysis, cough, and dyspnea), and renal symptoms (e.g., hematuria and red cell casts). A chest radiograph or CT scan will show bilateral, often perihilar, rounded opacities (Figure A). The best initial diagnostic test is to look for elevations in anti-proteinase 3 (PR3-ANCA) or c-ANCA. Figure A is a frontal chest radiograph showing multiple bilateral, perihilar, rounded opacities. Incorrect Answers: Answer 1: Sputum acid-fast stain would be indicated as a diagnostic test for tuberculosis. Although this patient has hemoptysis and night sweats, commonly associated with tuberculosis infections, his renal symptoms and history are more consistent with granulomatosis with polyangiitis. Answer 2: Tests for anti-myeloperoxidase or p-ANCA would be positive in patients with microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss). Microscopic polyangiitis is not associated with nasopharyngeal symptoms. Churg-Strauss is characteristically associated with asthma, skin nodules/purpura, and eosinophilia. Answer 4: A renal arteriogram would be abnormal for polyarteritis nodosa to show renal microaneurysms and spasms. Polyarteritis nodosa normally involves renal and visceral vessels, but the lungs are spared. Answer 5: Sputum cytology may reveal the presence of cancer cells in a patient who has a lung malignancy. Lung cancer more often presents with chronic symptoms such as a lingering cough and weight loss. Bullet Summary: The best initial diagnostic test for granulomatosis with polyangitis (formerly known as Wegener’s) is c-ANCA or anti-proteinase 3 (PR3-ANCA).
4.3
(6)
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