Amato M B, Barbas C S, Delmonte V C, Carvalho C R
Pulmonary Division of the Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Brasil.
Am Rev Respir Dis. 1989 Jun;139(6):1539-42. doi: 10.1164/ajrccm/139.6.1539.
Very few cases of pulmonary vasculitis that cannot be classified into a single category of vasculitis have been described. We report the first case of a vasculitic process in which pulmonary involvement with asthma, eosinophilic interstitial infiltrates, and small nodules were seen in association with jaw claudication and temporal arteritis with giant cells found on biopsy. Other signs of systemic involvement were also present such as peripheral neuropathy, hematuria with erythrocytic casts and proteinuria, pericardial effusion, and a dilated cardiomyopathy. The histopathologic picture was complex and unique. The early age of onset, the multisystemic involvement, and the prompt response to cyclophosphamide pointed to a diagnosis of "polyangiitis overlap syndrome," with some aspects of Churg-Strauss syndrome and also temporal arteritis. Physicians should be aware of these polymorphous and life-threatening pulmonary vasculitic syndromes, which require aggressive immunosuppressor therapy.
很少有不能归类为单一血管炎类型的肺血管炎病例被描述。我们报告了首例血管炎病例,该病例中可见肺部受累伴哮喘、嗜酸性间质浸润和小结节,同时伴有颌跛行和活检发现的巨细胞颞动脉炎。还存在其他全身受累的迹象,如周围神经病变、伴有红细胞管型和蛋白尿的血尿、心包积液以及扩张型心肌病。组织病理学表现复杂且独特。发病年龄早、多系统受累以及对环磷酰胺的迅速反应提示诊断为“多血管炎重叠综合征”,兼具变应性肉芽肿性血管炎综合征(Churg-Strauss综合征)和颞动脉炎的某些方面。医生应意识到这些多形性且危及生命的肺血管炎综合征,它们需要积极的免疫抑制治疗。