与干燥综合征相关的冷纤维蛋白原血症：一例高剂量皮质类固醇成功治疗的病例。

Cryofibrinogenemia associated with Sjögren's syndrome: a case of successful treatment with high-dose corticosteroid.

作者信息

Yoshida Ken, Yokoyama Toru, Toyokawa Yasuhiko, Yasuda Jun, Kingetsu Isamu, Kurosaka Daitaro, Yamada Akio

机构信息

Division of Rheumatology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo.

出版信息

Intern Med. 2007;46(13):1039-42. doi: 10.2169/internalmedicine.46.6311. Epub 2007 Jul 2.

DOI:10.2169/internalmedicine.46.6311

PMID:17603248

Abstract

Cryofibrinogenemia (CF) has not been often reported as a complication of various rheumatic diseases. We describe a 44-year-old woman with CF associated with Sjögren's syndrome (SS), who developed digital necrotic ulcerations and purpura of the lower legs. Cryoprecipitate was detected in her plasma, and immunoelectrophoresis showed that the cryoprecipitate was cryofibrinogen. Alprostadil was intravenously administered, but the ulceration was aggravated. Subsequently, administration of high-dose prednisolone (PSL) at 60 mg/day was started, and the ulceration remarkably improved. Cryofibrinogen, detected before the administration of high-dose PSL, was negative after PSL. This is the first case presentation of CF associated with SS successfully treated with high-dose corticosteroid.

摘要

冷纤维蛋白原血症（CF）作为各种风湿性疾病的并发症并不常被报道。我们描述了一名44岁患有与干燥综合征（SS）相关的CF的女性，她出现了手指坏死性溃疡和小腿紫癜。在她的血浆中检测到冷沉淀，免疫电泳显示冷沉淀为冷纤维蛋白原。静脉注射前列地尔，但溃疡加重。随后，开始每天给予60mg大剂量泼尼松龙（PSL），溃疡明显改善。在给予大剂量PSL之前检测到的冷纤维蛋白原，在使用PSL后变为阴性。这是首例成功用大剂量皮质类固醇治疗的与SS相关的CF病例报告。