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[对一名特发性血小板减少性紫癜患儿出现自身抗体并随后发展为伴有中枢神经系统表现的狼疮的九年随访]

[Nine-year's follow up on the appearance of autoantibodies in a child with idiopathic thrombocytopenic purpura subsequently developing lupus with central nervous system manifestations].

作者信息

Kagitani Maki, Makino Shigeki, Kinjo Yoshiko, Hirano Suzue, Tabushi Yoko, Sasaki Masako, Takeuchi Tohru, Hanafusa Toshiaki, Murata Takuji

机构信息

First Department of Internal Medicine, Osaka Medical College, Takatsuki-city.

出版信息

Ryumachi. 2002 Jun;42(3):591-6.

Abstract

We describe a 23-year-old female who developed SLE 9 years after asymptomatic idiopathic thrombocytopenic purpura (ITP) with positive antinuclear antibody (ANA). Although the platelet count was normal before the onset of SLE, the titer of ANA was gradually increased and also autoantibodies, including antibodies to SS-A/Ro, single-stranded DNA (ss-DNA) and nuclear ribonucleoprotein (RNP) changed to positive. At 23 years of age, the patient was admitted to our hospital because of fever, butterfly rash and polyarthritis. Anti double-strand DNA (ds-DNA) antibody and anti Smith antigen (Sm) antibody were positive and the platelet count and titer of complements were decreased. The patient was diagnosed as SLE and treated with 60 mg/day of prednisolone. Despite steroid therapy, psychiatric symptoms appeared. Additional treatments with steroid pulse therapy and double filtration plasmaphresis resulted in the improvement of SLE including the central nervous system manifestations. This case suggested that increased titer of ANA and the appearance of antibodies to SS-A, ss-DNA, RNP, ds-DNA and Sm in ITP patients predict the development of SLE. Routine checkup of autoantibodies is needed to manage ITP with positive ANA.

摘要

我们描述了一名23岁女性,她在无症状特发性血小板减少性紫癜(ITP)伴抗核抗体(ANA)阳性9年后患上系统性红斑狼疮(SLE)。尽管在SLE发病前血小板计数正常,但ANA滴度逐渐升高,包括抗SS-A/Ro、单链DNA(ss-DNA)和核糖核蛋白(RNP)抗体在内的自身抗体也转为阳性。23岁时,患者因发热、蝶形红斑和多关节炎入住我院。抗双链DNA(ds-DNA)抗体和抗史密斯抗原(Sm)抗体呈阳性,血小板计数和补体滴度降低。该患者被诊断为SLE,并接受了60毫克/天的泼尼松龙治疗。尽管进行了类固醇治疗,但仍出现了精神症状。额外的类固醇脉冲疗法和双重滤过血浆置换治疗使包括中枢神经系统表现在内的SLE病情得到改善。该病例表明,ITP患者ANA滴度升高以及抗SS-A、ss-DNA、RNP、ds-DNA和Sm抗体的出现预示着SLE的发生。对于ANA阳性的ITP患者,需要进行自身抗体的常规检查。

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