Non-renal Microscopic Polyangiitis with Acute Pulmonary Hemorrhage: A Case Report

無腎臟侵犯之顯微性多血管炎合併急性肺出血：病例報告

莊志亮(Chin-Liang Chuang) ； 彭萬誠(Wann-Cherng Perng) ； 簡志峰(Chin-Feng Giian) ； 涂川洲(Chuan-Chou Tu) ； 楊登和(Deng-Ho Yang) ； 王守正(Shou-Cheng Wang)

《胸腔醫學》 28卷4期 (2013/08) Pp. 247-252

摘要

顯微性多血管炎是一種全身小血管壞死性血管炎與antineutrophil cytoplasmic antibodies有關，類似granulomatosis with polyangiitis（GPA），但沒有肉芽腫產生。顯微性多血管炎的發病機制可能是由於血管內皮損傷和血管炎病變所導致。經常以腎臟或肺部侵犯來表現；然而，全身其他部位病變也可能見到。顯微性多血管炎的患者中，超過90%會表現出腎臟侵犯合併腎臟以外不同部位的血管炎。在顯微性多血管炎的病人中，在臨床上以沒有腎臟侵犯來表現是少見的。我們報告一個46歲的男性表現肺纖維化之後十年診斷無腎臟侵犯之顯微性多血管炎，確診之後二年發生由於感染引起血管炎急性惡化合併急性肺出血。

關鍵字

顯微性多血管炎 ； 肺纖維化 ； 肺出血

並列摘要

Microscopic polyangiitis (MPA) is a systemic small-vessel necrotizing vasculitis associated with antineutrophil cytoplasmic antibodies, similar to granulomatosis with polyangiitis (GPA), but without granuloma. The pathogenesis of MPA may be endothelial damage and vasculitic lesions. The manifestations often include renal or pulmonary involvement; however, other systemic lesions might also be observed. Over 90% of MPA patients exhibit renal involvement with extra-renal vasculitis at varying sites. Cases of non-renal MPA are clinically uncommon. We report the case of a 46-year-old male with pulmonary fibrosis, which had persisted for 10 years before the diagnosis of non-renal MPA. The patient also had acute pulmonary hemorrhage due to an infection-induced flare-up of vasculitis, which occurred 2 years after the diagnosis of non-renal MPA.

並列關鍵字

microscopic polyangiitis ； pulmonary fibrosis ； pulmonary hemorrhage