恶性高血压与肺出血幻灯片.pptVIP

IgA肾病、恶性高血压与肺出血 北京协和医院 IgA肾病与肺出血 Pulmonary capillaritis and alveolar hemorrhage. Update on diagnosis and management. Pulmonary vascular inflammatory disorders may involve all components of the pulmonary vasculature, including capillaries. The principal histopathologic features of pulmonary capillaritis include capillary wall necrosis with infiltration by neutrophils, interstitial erythrocytes, and/or hemosiderin, and interalveolar septal capillary occlusion by fibrin thrombi. Immune complex deposition is variably present. Patients often present clinically with diffuse alveolar hemorrhage, which is characterized by dyspnea and hemoptysis; diffuse, bilateral, alveolar infiltrates on chest radiograph; and anemia. Pulmonary capillaritis has been reported with variable frequency and severity as a manifestation of Wegeners granulomatosis, microscopic polyarteritis, systemic lupus erythematosus, Goodpastures syndrome, idiopathic pulmonary renal syndrome, Behcets syndrome, Henoch-Schonlein purpura, IgA nephropathy, antiphospholipid syndrome, progressive systemic sclerosis, and diphenylhydantoin use. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. 34 patients with biopsy-confirmed diffuse pulmonary hemorrhage (DPH). DPH syndromes were encountered: antibasement membrane antibody disease (four cases); idiopathic pulmonary hemorrhage (four cases); WG(five cases); probable WG (six cases); systemic necrotizing vasculitis otherwise unclassified (three cases); systemic lupus erythematosus (two cases); rheumatoid arthritis (one case); seronegative juvenile rheumatoid arthritis (one case); IgA nephropathy (one case); idiopathic glomerulonephritis (two cases--one with and one without immune complexes); and unclassified pulmonary-renal syndromes (five cases). Capillaritis was found in lung biopsy samples from 30 of the 34 patients (88%) and included patients with every type of DPH syndrome. Am J Surg Pathol. 1990 Dec;14(12):