与CREST综合征和硬皮病相关的大血管闭塞性疾病。
Large vessel occlusive disease associated with CREST syndrome and scleroderma.
作者信息
Youssef P, Englert H, Bertouch J
机构信息
Department of Rheumatology, Prince Henry Hospital, Sydney, Australia.
出版信息
Ann Rheum Dis. 1993 Jun;52(6):464-6. doi: 10.1136/ard.52.6.464.
OBJECTIVES
To report the cases of three patients with CREST syndrome and one patient with diffuse scleroderma who had severe macrovascular disease and only minimal vascular risk factors.
METHODS
The medical histories, physical examinations, and results of clinical investigations were reviewed in four patients.
RESULTS
These four patients had severe morbidity from macrovascular disease of the arms and legs in the presence of minimal underlying vascular risk factors. These patients represent 11% of the women with scleroderma seen at our hospital since 1974. This is a greater than threefold increase above the expected proportion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen more than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ulnar artery from one patient showed severe luminal narrowing by an acellular material with no evidence of atheroma.
CONCLUSIONS
These cases suggest an association of both the CREST syndrome and scleroderma with macrovascular disease.
目的
报告3例CREST综合征患者和1例弥漫性硬皮病患者的病例，这些患者患有严重的大血管疾病且仅有极少的血管危险因素。
方法
回顾了4例患者的病史、体格检查及临床检查结果。
结果
这4例患者在仅有极少潜在血管危险因素的情况下，因手臂和腿部的大血管疾病而出现严重的发病情况。自1974年以来，这些患者占我院所见硬皮病女性患者的11%。这比人群研究中预期的有症状血管疾病比例高出三倍多。在CREST综合征患者中，大血管疾病首次出现在雷诺现象发作10多年后，而雷诺现象是该疾病的首发表现。1例患者尺动脉的病理标本显示管腔被无细胞物质严重狭窄，无动脉粥样硬化证据。
结论
这些病例提示CREST综合征和硬皮病均与大血管疾病有关。
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