Arimura Y, Minoshima S, Tanaka U, Fujii A, Kobayashi M, Nakabayashi K, Kitamoto K, Nagasawa T
First Department of Internal Medicine, Kyorin University, School of Medicine, Tokyo.
Ryumachi. 1995 Feb;35(1):46-55.
In order to examine the clinical features of pulmonary involvement in patients with myeloperoxidase specific-antineutrophil cytoplasmic antibody (MPO-ANCA), 46 MPO-ANCA positive patients with collagen-vascular disease and glomerulonephritis were investigated. Twenty eight patients (60.8%) had pulmonary involvement without tumor and infection, 20 (43.5%) of MPO-ANCA positive patients had interstitial pneumonitis, 11 (23.9%) had pulmonary hemorrhage, 3 had asthma (6.5%) and 7 had both interstitial pneumonitis and pulmonary hemorrhage. Patients with pulmonary involvement were older (mean age 61.1) and they had higher inflammatory acute phase reactants and higher mortality (42.9%) than patients without pulmonary involvement. Pulmonary hemorrhage revealed on both lung fields in 10 patients, 7 patients required artificial respirator and 6 died. Titer of MPO-ANCA increased paralleled with progression of pulmonary hemorrhage. Interstitial fibrosis revealed predominantly lower and lateral side of the lung. It is suggested that MPO-ANCA may be closely related to the pathogenesis of pulmonary hemorrhage and interstitial pneumonia. Careful management is needed in patients with pulmonary involvement, especially pulmonary hemorrhage in patients with MPO-ANCA.
为了研究髓过氧化物酶特异性抗中性粒细胞胞浆抗体（MPO-ANCA）阳性患者肺部受累的临床特征，对46例患有胶原血管病和肾小球肾炎的MPO-ANCA阳性患者进行了调查。28例患者（60.8%）有肺部受累，无肿瘤和感染，20例（43.5%）MPO-ANCA阳性患者有间质性肺炎，11例（23.9%）有肺出血，3例（6.5%）有哮喘，7例同时有间质性肺炎和肺出血。肺部受累患者年龄较大（平均年龄61.1岁），与无肺部受累患者相比，他们的炎症急性期反应物水平更高，死亡率也更高（42.9%）。10例患者双肺野出现肺出血，7例患者需要使用人工呼吸器，6例死亡。MPO-ANCA滴度随肺出血进展而升高。间质性纤维化主要出现在肺的下侧和外侧。提示MPO-ANCA可能与肺出血和间质性肺炎的发病机制密切相关。对于肺部受累患者，尤其是MPO-ANCA阳性的肺出血患者，需要进行仔细的管理。
