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家族性地中海热中的肺部关联

Pulmonary associations in familial Mediterranean fever.

作者信息

Livneh A, Langevitz P, Pras M

机构信息

Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.

出版信息

Curr Opin Pulm Med. 1999 Sep;5(5):326-31. doi: 10.1097/00063198-199909000-00011.

DOI:10.1097/00063198-199909000-00011
PMID:10461539
Abstract

Familial Mediterranean fever (FMF) is a hereditary periodic fever syndrome expressed by acute episodes of fever and painful manifestations. In this report, the pulmonary manifestations of FMF are reviewed, the most prominent of which are chest attacks due to pleuritis. Nephropathic amyloidosis of the AA type, which complicates FMF in most untreated patients, may progress to affect other organs, including the lungs, but this rarely produces noticeable symptoms. The common association between FMF and vasculitis makes pulmonary hemorrhage, infarction, or infiltrates highly possible. These complications, however, have been reported only rarely. Asthma was found to occur less often than expected in patients with FMF, but methodologic faults make this finding doubtful. Finally, the occurrence of mesothelioma in five patients with FMF who were not exposed to asbestos suggests a role for recurrent FMF serositis in the pathogenesis of this malignancy.

摘要

家族性地中海热(FMF)是一种遗传性周期性发热综合征,表现为发热急性发作和疼痛症状。在本报告中,对FMF的肺部表现进行了综述,其中最突出的是胸膜炎引起的胸部发作。AA型肾病性淀粉样变性在大多数未经治疗的患者中使FMF复杂化,可能进展至影响其他器官,包括肺,但这很少产生明显症状。FMF与血管炎之间的常见关联使得肺出血、梗死或浸润很有可能发生。然而,这些并发症仅被罕见报道。研究发现FMF患者中哮喘的发生率低于预期,但方法学缺陷使这一发现存疑。最后,5例未接触石棉的FMF患者发生间皮瘤,提示复发性FMF浆膜炎在这种恶性肿瘤的发病机制中起作用。

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