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伴有干燥综合征的家族性CRST综合征

Familial CRST syndrome with sicca complex.

作者信息

Frayha R A, Tabbara K F, Geha R S

出版信息

J Rheumatol. 1977 Spring;4(1):53-8.

PMID:886553
Abstract

Two patients, a mother and daughter, each with the CRST syndrome, developed Sjögren's syndrome. Both patients had mitochondrial antibodies, smooth muscle antibodies, and a raised serum IgM without clinical evidence of liver disease. This family constitutes the first record of the familial coexistence of the CRST syndrome with Sjögren's syndrome, and the second evidence of vertical inheritance of scleroderma. It is suggested that patients with the CRST syndrome be studied for Sjögren's syndrome and for autoimmune liver disease.

摘要

两名患有CRST综合征的患者,一位母亲和女儿,均患上了干燥综合征。两名患者均有抗线粒体抗体、抗平滑肌抗体,血清IgM升高,但无肝病的临床证据。这个家族是CRST综合征与干燥综合征家族共存的首例记录,也是硬皮病垂直遗传的第二个证据。建议对CRST综合征患者进行干燥综合征和自身免疫性肝病的研究。

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