Seronegative granulomatous polyangiitis with central retinal artery occlusion as the initial manifestation.

PURPOSE To report a case of central retinal artery occlusion in a patient with autopsy-verified granulomatous polyangiitis (GP) with negative cytoplasmic antineutrophil cytoplasmic antibody. METHODS Case report. RESULTS A 61-year-old Hispanic man with history of tuberculosis was admitted to the hospital with worsening dyspnea. Two weeks later, he experienced sudden vision loss due to central retinal artery occlusion in his right eye. A CT of the lung revealed multiple opacities. He developed renal failure during his hospital stay. A serum cytoplasmic antineutrophil cytoplasmic antibody test was negative. Pulmonary biopsy disclosed chronic inflammation with no evidence of granuloma formation or vasculitis. He died of acute respiratory distress due to bilateral deep vein thrombosis of his lower extremities. Autopsy revealed GP. CONCLUSION Granulomatous polyangiitis is a multisystem vasculitic disorder. Central retinal artery occlusion as the presenting manifestation of GP is very uncommon. This report demonstrates the difficulty of diagnosing GP, particularly when initial diagnostic assays (including both serology and biopsy) were negative for GP.