一名系统性红斑狼疮且狼疮活动轻微的患者出现严重肺动脉高压。

Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity.

作者信息

Kawamura Nobutoshi, Tsutsui Hiroyuki, Fukuyama Kae, Hayashidani Shunji, Koike George, Egashira Kensuke, Abe Yasunobu, Yamamoto Hidetaka, Tsuneyoshi Masazumi, Takeshita Akira

机构信息

Department of Cardiovascular Medicine, Kyushu University, Fukuoka.

出版信息

Intern Med. 2002 Feb;41(2):109-12. doi: 10.2169/internalmedicine.41.109.

DOI:10.2169/internalmedicine.41.109

PMID:11868596

Abstract

Pulmonary hypertension (PH) sometimes occurs in patients with systemic lupus erythematosus (SLE). We report a case of 51-year-old-woman with PH associated with SLE. She had been diagnosed as SLE on the basis of pericardial effusion, hematological disorder, positive antinuclear antibody, and hypocomplementemia. Despite minimal lupus activity, she had marked elevation of pulmonary arterial pressure (101/53 mmHg) and decreased cardiac index (1.5 l/min/m2). Symptoms related to PH were progressive under treatment with oral corticosteroids, oxygen, calcium antagonists, and warfarin. After 17 months of epoprostenol treatment, she died of pulmonary infarction. SLE-associated PH is often severe and progressive even in association with minimal activity.

摘要

肺动脉高压（PH）有时会出现在系统性红斑狼疮（SLE）患者中。我们报告一例51岁女性患者，其患有与SLE相关的PH。她因心包积液、血液系统疾病、抗核抗体阳性及补体降低而被诊断为SLE。尽管狼疮活动轻微，但她的肺动脉压显著升高（101/53 mmHg）且心脏指数降低（1.5 l/min/m²）。在接受口服糖皮质激素、氧气、钙拮抗剂及华法林治疗期间，与PH相关的症状仍不断进展。接受依前列醇治疗17个月后，她死于肺梗死。即使狼疮活动轻微，SLE相关的PH通常仍很严重且呈进行性发展。