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重症肌无力:长期硫唑嘌呤停药后临床疾病及自身免疫因素的重新激活。

Myasthenia gravis: reactivation of clinical disease and of autoimmune factors after discontinuation of long-term azathioprine.

作者信息

Hohlfeld R, Toyka K V, Besinger U A, Gerhold B, Heininger K

出版信息

Ann Neurol. 1985 Mar;17(3):238-42. doi: 10.1002/ana.410170304.

DOI:10.1002/ana.410170304
PMID:3873207
Abstract

In 15 patients with myasthenia gravis who were in stable clinical remission while receiving azathioprine, we monitored disease severity and serial autoantibody titers before and after discontinuation of azathioprine. Cellular immunoreactivity against tuberculin (PPD) and against Torpedo acetylcholine receptor (AChR) was measured serially in 11 patients. Eight of 15 patients (53%) had a clinical relapse after 3 to 11 months, necessitating the reinstitution of immunosuppressive treatment in 6 patients. Seven patients have remained clinically stable during an observation period of 20 to 40 months. Anti-AChR autoantibody titers correlated closely with the clinical course in the majority of patients, and rose markedly in 7 of the 8 patients who relapsed. Cellular stimulation indices correlated less closely with the clinical severity. Only in 3 patients did the clinical score, antibody titer, and cellular stimulation index rise concurrently. In 4 patients who had high cellular stimulation indices after the discontinuation of azathioprine, it was possible to isolate AChR-reactive inducer/helper T lymphocytes.

摘要

在15例接受硫唑嘌呤治疗且临床病情稳定缓解的重症肌无力患者中,我们在停用硫唑嘌呤前后监测了疾病严重程度和系列自身抗体滴度。对11例患者连续测量了针对结核菌素(PPD)和针对电鳐乙酰胆碱受体(AChR)的细胞免疫反应性。15例患者中有8例(53%)在3至11个月后出现临床复发,其中6例需要重新开始免疫抑制治疗。7例患者在20至40个月的观察期内临床病情保持稳定。在大多数患者中,抗AChR自身抗体滴度与临床病程密切相关,8例复发患者中有7例抗体滴度显著升高。细胞刺激指数与临床严重程度的相关性较弱。仅3例患者的临床评分、抗体滴度和细胞刺激指数同时升高。在4例停用硫唑嘌呤后细胞刺激指数较高的患者中,能够分离出AChR反应性诱导/辅助性T淋巴细胞。

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