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硬皮病中的大量心包积液:五例病例回顾

Massive pericardial effusion in scleroderma: a review of five cases.

作者信息

Satoh M, Tokuhira M, Hama N, Hirakata M, Kuwana M, Akizuki M, Ichikawa Y, Ogawa S, Homma M

机构信息

Division of Rheumatology, Keio University School of Medicine, Tokyo, Japan.

出版信息

Br J Rheumatol. 1995 Jun;34(6):564-7. doi: 10.1093/rheumatology/34.6.564.

DOI:10.1093/rheumatology/34.6.564
PMID:7633799
Abstract

Medical records of five patients with scleroderma (SSc), each of whom had pericardial effusion with an estimated volume of more than 200 ml, were reviewed to study the clinical and immunological significance of massive pericardial effusion in SSc. Diffuse SSc (4/5), with a wide area of pigmentation (4/5), flexion contracture (4/5), oesophageal hypomotility (5/5), pulmonary fibrosis (4/5) and autoantibodies to topoisomerase I (3/5) were the common features in this group. High protein, lactate dehydrogenase and low white blood cell count were the characteristics of pericardial fluid. None of the patients had signs of acute pericarditis. Four of the five cases died within 9 months of the diagnosis of pericarditis; two with renal failure, one with cardiac tamponade and another with sudden death. The pericarditis in diffuse SSc, especially in cases with anti-topoisomerase I, may be characterized by a chronic form of pericarditis with poor prognosis, often complicated by renal failure.

摘要

回顾了5例硬皮病(SSc)患者的病历,这些患者均有心包积液,估计积液量超过200ml,以研究SSc中大量心包积液的临床和免疫学意义。弥漫性SSc(4/5)、色素沉着广泛(4/5)、屈曲挛缩(4/5)、食管动力不足(5/5)、肺纤维化(4/5)以及抗拓扑异构酶I自身抗体(3/5)是该组患者的常见特征。高蛋白、乳酸脱氢酶以及低白细胞计数是心包积液的特点。所有患者均无急性心包炎的体征。5例中有4例在诊断心包炎后9个月内死亡;2例死于肾衰竭,1例死于心脏压塞,另1例猝死。弥漫性SSc中的心包炎,尤其是抗拓扑异构酶I阳性的病例,可能表现为慢性心包炎,预后较差,常并发肾衰竭。

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