Arimura Y, Minoshima S, Kamiya Y, Motohashi S, Nakabayashi K, Kitamoto K, Nagasawa T, Suzuki K
First Department of Internal Medicine, School of Medicine, Kyorin University, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 1991 Oct;33(10):1011-6.
We report a case of idiopathic crescentic glomerulonephritis with pulmonary hemorrhage associated with anti-myeloperoxidase antibodies (anti-MPO ab). A 74 year-old female was admitted to our hospital because of rapidly progressive glomerulonephritic syndrome and dyspnea with bloody sputum. On admission anti-MPO ab, one of anti-neutrophil cytoplasmic antibodies, were detected but anti-GBM antibodies and immune complexes were not detected. Renal biopsy showed crescentic glomerulonephritis and lung biopsy showed massive alveolar hemorrhage. Both tissue had pauci-immune deposit by immunofluorescence microscopy. Hemodialysis and steroid administration were started. Pulmonary hemorrhage was improved remarkably, but renal failure progressed rapidly to end stage kidney, then hemodialysis was continued. Although subsequent 3 years uneventful maintenance hemodialysis had been performed, she admitted to our hospital again because of progressive dyspnea with hemoptysis after upper respiratory tract infection. On admission anti-MPO ab were detected again and steroid administration was started. Pulmonary hemorrhage was improved with decreased anti-MPO ab titer. While tapering the dosis of steroid, anti-MPO ab again increased and pulmonary hemorrhage recurred. Although pulse methylprednisolone therapy and plasma exchange were performed, respiratory failure progressed rapidly and she died of sepsis. Postmortem examination showed no evidence of systemic vasculitis. In this case, titer of anti-MPO ab was associated with not only idiopathic crescentic glomerulonephritis but also with pulmonary hemorrhage. We tried to detect enzymatically active MPO in serum. Titer of serum MPO was also associated with disease activity and anti-MPO ab. It is suggested that both anti-MPO ab and serum MPO are closely related to the pathogenesis of idiopathic crescentic glomerulonephritis and pulmonary hemorrhage.
我们报告一例伴有肺出血的特发性新月体性肾小球肾炎，与抗髓过氧化物酶抗体（抗MPO抗体）相关。一名74岁女性因快速进展性肾小球肾炎综合征及咯血伴呼吸困难入住我院。入院时检测到抗中性粒细胞胞浆抗体之一抗MPO抗体，但未检测到抗GBM抗体和免疫复合物。肾活检显示新月体性肾小球肾炎，肺活检显示大量肺泡出血。免疫荧光显微镜检查显示两个组织均有寡免疫沉积。开始进行血液透析和给予类固醇。肺出血明显改善，但肾衰竭迅速进展至终末期肾病，随后继续进行血液透析。尽管随后3年进行了平稳的维持性血液透析，但她因上呼吸道感染后咯血伴进行性呼吸困难再次入住我院。入院时再次检测到抗MPO抗体并开始给予类固醇。随着抗MPO抗体滴度降低，肺出血得到改善。在逐渐减少类固醇剂量时，抗MPO抗体再次升高，肺出血复发。尽管进行了甲泼尼龙冲击治疗和血浆置换，但呼吸衰竭迅速进展，她死于败血症。尸检未发现系统性血管炎的证据。在该病例中，抗MPO抗体滴度不仅与特发性新月体性肾小球肾炎有关，还与肺出血有关。我们试图检测血清中具有酶活性的MPO。血清MPO滴度也与疾病活动度及抗MPO抗体有关。提示抗MPO抗体和血清MPO均与特发性新月体性肾小球肾炎及肺出血的发病机制密切相关。
