Schwarz M I, Mortenson R L, Colby T V, Waldron J A, Lynch D A, Hutt M P, Cherniack R M, King T E
Department of Medicine, National Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado.
Am Rev Respir Dis. 1993 Aug;148(2):507-11. doi: 10.1164/ajrccm/148.2.507.
We report two patients with systemic necrotizing vasculitis (microscopic polyarteritis) and associated recurrent pulmonary capillaritis, in whom progressive irreversible airway dysfunction began approximately 10 yr after disease onset. Their course was characterized by repeated episodes of diffuse alveolar hemorrhage, glomerulonephritis, palpable purpura, and splinter hemorrhages. The lung revealed intraalveolar hemorrhage, neutrophilic infiltration and cellular fragmentation, fibrinoid necrosis of the alveolar interstitium, and parenchymal hemosiderin deposits. No medium-sized vessel involvement, granulomatous inflammation, or bronchiolar obliteration were seen. Renal biopsies revealed focal segmental necrotizing glomerulonephritis, and a cutaneous biopsy in one case showed a leukocytoclastic vasculitis. Immunofluorescent studies of lung and kidney showed minimal or no immunoreactivity. The clinical course and serologic tests did not support another systemic vasculitis, connective tissue disease, or antiglomerular basement membrane antibody disease. The acute episodes responded to antiinflammatory and immunosuppressive therapy. Symptoms, serial pulmonary function tests, and chest imaging documented the development of a progressive irreversible obstructive airway disease. No other predisposing factors were identified. These cases demonstrate the unexpected appearance of an irreversible obstructive airway disease with lung parenchymal hyperinflation after systemic necrotizing vasculitis associated with recurrent pulmonary capillaritis and diffuse alveolar hemorrhage.
我们报告了两名患有系统性坏死性血管炎（显微镜下多动脉炎）及相关复发性肺毛细血管炎的患者，他们在疾病发作约10年后开始出现进行性不可逆气道功能障碍。其病程特点为反复出现弥漫性肺泡出血、肾小球肾炎、可触及的紫癜和裂片样出血。肺部表现为肺泡内出血、中性粒细胞浸润和细胞破碎、肺泡间质的纤维蛋白样坏死以及实质内含铁血黄素沉积。未见中等大小血管受累、肉芽肿性炎症或细支气管闭塞。肾活检显示局灶节段性坏死性肾小球肾炎，1例皮肤活检显示白细胞破碎性血管炎。肺和肾的免疫荧光研究显示免疫反应性轻微或无。临床病程和血清学检查不支持其他系统性血管炎、结缔组织病或抗肾小球基底膜抗体病。急性发作对抗炎和免疫抑制治疗有反应。症状、系列肺功能测试和胸部影像学检查记录了进行性不可逆阻塞性气道疾病的发展。未发现其他诱发因素。这些病例显示，在伴有复发性肺毛细血管炎和弥漫性肺泡出血的系统性坏死性血管炎后，出现了伴有肺实质过度充气的不可逆阻塞性气道疾病，这一情况出乎意料。
