Diffuse Alveolar Hemorrhage With Histopathologic Manifestations Of Pulmonary Capillaritis: Three Cases Report And Literatures Review

OBJECTIVE:To report three cases of diffuse alveolar hemorrhage(DAH) that histopathologic manifestations showed pulmonary capillaritis (PC) and study the clinical diagnosis and treatment process,also to review the related literatureS,in order to improve the diagnosis and treatment of PC and clearly understand the relationship between PC and DAH.METHODS:The clinical,radiological,histological and treatment informations of three cases of PC were retrospectively analyzed and the lung tissues done direct immunofluorescence tests, and also the related literatures was reviewed.RESULTS:The three patients were girls and got ill since school-age.They presented with cough, hemoptysis, shortness of breath, dyspnea and different degree of anemia in symptoms and signs. Serological characteristics of lower hemoglobin in two cases and elevated blood sedimentation rate in case three, throcic high resolution computed tomography (HRCT) characterized by bilateral diffuse pulmonary infiltrate change, lung biopsy showed pulmonary capillaritis.The autoantibodies and immunofluorescence tests of case one and case two were negative, so their preliminary diagnosis were isolated pauciimmune pulmonary capillaritis(IPIPC). However, the autoantibodies of case three showed ANA 1:320 and immunofluorescence test saw granular IgG and a little granular IgA deposit on the alveolar walls under fluorescence microscope.Above all,a preliminary diagnosis of systemic lupus erythematosus (SLE) with PC can be maded. After confirmed the PC, all the case begun to take glucocorticoid therapy though ivdrip or oral.It proved that disease could be control in the follow-up time.Then reviewed the related literatures, there were 21 cases about PC reported in related domestic and foreign medical literatures so far.The clinical manifestations of all 21 cases included respiratory symptoms and extrapulmonary manifestations.The main respiratory symptoms included cough, hemoptysis, wheezing, dyspnea and hypoxemia and so on.73.9% of all the cases had moderate anemia and 41.7% had elevated ESR, chest imaging in different degree of diffuse pulmonary infiltrates, lung biopsy showed PC. By using glucocorticoid or combining immunosuppressant treatment,72.7% processus of the cases were controlled.CONCLUSION:PC is a histopathological type of DAH,and though lung biopsy can diagnosis PC.PC can be divided into two categories,non-immune mediated (isolated pulmonary capillaritis, IPC) and immnune mediated PC associate with systemic disease.The majority of children were under control though fist line treatment that use glucocorticoid or combination of immunosuppressant treatment.