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弥漫性肺泡出血和 Libman-Sacks 心内膜炎作为可能原发性抗磷脂综合征的表现。

Diffuse alveolar hemorrhage and Libman-Sacks endocarditis as a manifestation of possible primary antiphospholipid syndrome.

机构信息

Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA.

出版信息

J Clin Rheumatol. 2013 Mar;19(2):79-83. doi: 10.1097/RHU.0b013e318278c577.

DOI:10.1097/RHU.0b013e318278c577
PMID:23340096
Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of circulating autoantibodies against phospholipid-binding plasma proteins, leading to an increased risk of thrombosis and pregnancy loss. The most common manifestation of lung disease in APS is pulmonary embolism, which may often be the presenting symptom. We present a 30-year-old man with probable primary APS (with no history of thromboses) presenting with diffuse alveolar hemorrhage, an uncommon presentation. He was also found to have severe mitral valve regurgitation and during valve replacement surgery had cardiac vegetations compatible with a presentation of Libman-Sacks endocarditis. There are only 21 other reported cases of diffuse alveolar hemorrhage occurring as a result of APS. This is the first case of Libman-Sacks endocarditis in the setting of probable APS and alveolar hemorrhage.Diffuse alveolar hemorrhage should be considered as a nonthrombotic manifestation of APS, even in the absence of known thromboses, and may be the presenting symptom.

摘要

抗磷脂综合征(APS)是一种自身免疫性疾病,其特征是存在针对结合磷脂的血浆蛋白的循环自身抗体,从而增加血栓形成和妊娠丢失的风险。APS 中最常见的肺部疾病表现为肺栓塞,这可能经常是首发症状。我们报告了一名 30 岁男性,可能患有原发性 APS(无血栓形成史),表现为弥漫性肺泡出血,这是一种不常见的表现。他还被发现患有严重的二尖瓣反流,在瓣膜置换手术中发现与 Libman-Sacks 心内膜炎表现一致的心脏赘生物。仅有 21 例其他报道的弥漫性肺泡出血是由 APS 引起的。这是可能的 APS 和肺泡出血背景下首例 Libman-Sacks 心内膜炎病例。即使没有已知的血栓形成,弥漫性肺泡出血也应被视为 APS 的非血栓形成表现,并且可能是首发症状。

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Diffuse alveolar hemorrhage and Libman-Sacks endocarditis as a manifestation of possible primary antiphospholipid syndrome.弥漫性肺泡出血和 Libman-Sacks 心内膜炎作为可能原发性抗磷脂综合征的表现。
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