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肺毛细血管炎和弥漫性肺泡出血。多发性肌炎的一种主要表现。

Pulmonary capillaritis and diffuse alveolar hemorrhage. A primary manifestation of polymyositis.

作者信息

Schwarz M I, Sutarik J M, Nick J A, Leff J A, Emlen J W, Tuder R M

机构信息

Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver 80262, USA.

出版信息

Am J Respir Crit Care Med. 1995 Jun;151(6):2037-40. doi: 10.1164/ajrccm.151.6.7767555.

DOI:10.1164/ajrccm.151.6.7767555
PMID:7767555
Abstract

Polymyositis is often complicated by either usual interstitial pneumonitis, diffuse alveolar damage, cellular interstitial pneumonitis, or bronchiolitis obliterans-organizing pneumonia. Pulmonary capillaritis, a distinct interstitial reaction associated with diffuse alveolar hemorrhage, occurs with the systemic vasculitides and some collagen vascular diseases. It has not been described in patients with polymyositis. We describe two patients who developed a relatively acute onset of polymyositis based on the appearance of a severe proximal myopathy, elevated serum creatine phosphokinase levels, compatible electromyographic abnormalities, inflammatory muscle biopsies, and in one case, the presence of serum anti-Jo-1 antibodies. Concomitant with their muscle disease, they developed respiratory failure that proved to be pulmonary capillaritis with varying degrees of diffuse alveolar hemorrhage as well as bronchiolitis obliterans-organizing pneumonia. Although those reactions are reported to occur with other collagen vascular diseases, these two cases are the first reports of pulmonary capillaritis and diffuse alveolar hemorrhage complicating polymyositis.

摘要

多发性肌炎常并发寻常性间质性肺炎、弥漫性肺泡损伤、细胞性间质性肺炎或闭塞性细支气管炎伴机化性肺炎。肺毛细血管炎是一种与弥漫性肺泡出血相关的独特间质反应,见于系统性血管炎和一些胶原血管病。尚未见有多发性肌炎患者发生肺毛细血管炎的报道。我们描述了两名患者,他们基于严重近端肌病的表现、血清肌酸磷酸激酶水平升高、相符的肌电图异常、炎性肌肉活检,以及其中1例患者存在血清抗Jo-1抗体,出现了相对急性起病的多发性肌炎。在其肌肉疾病的同时,他们发生了呼吸衰竭,结果证明是肺毛细血管炎伴不同程度的弥漫性肺泡出血以及闭塞性细支气管炎伴机化性肺炎。尽管据报道这些反应可见于其他胶原血管病,但这两例是多发性肌炎并发肺毛细血管炎和弥漫性肺泡出血的首例报道。

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