布基纳法索自身免疫性肌无力的治疗和临床病程

Medecine tropicale et sante internationale Pub Date : 2025-02-21 eCollection Date: 2025-03-31 DOI:10.48327/mtsi.v5i1.2025.646
Lompo Djingri Labodi, Adeline Julie Marie Kyelem, Alassane Zoungrana, M Fabienne Yabtouta Kere, Melody Zeinab Gnampa, Hervé Nacoulma, Christian Napon, Athanase Millogo
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引用次数: 0

摘要

在撒哈拉以南非洲,自身免疫性重症肌无力(AMG)仍然知之甚少,诊断不足(诊断延迟,缺乏有效的诊断和治疗工具),导致功能预后差,死亡率高。本研究的目的是评估布基纳法索AMG的治疗和临床过程。患者和方法:这是一项纵向、多中心研究,于2015年3月至2023年4月进行。它包括有提示肌无力的临床症状的患者,与血清抗乙酰胆碱受体(抗rach)抗体和/或抗肌肉特异性激酶(抗musk)抗体的存在有关,和/或神经肌电图下降bbb10 %,和/或口服抗胆碱酯酶药物治疗试验阳性。采用Epi InfoTM 7.2.5.0软件分析治疗方式及临床进展。结果:共纳入40例AMG患者,以女性为主(60%)。中位发病年龄为25岁(IQ=7)。到神经科会诊和诊断的中位时间分别为21个月(IQ=12)和22个月(IQ=12)。85%的病例为年轻人,35例为全身性。33例患者中分别有22例和4例存在抗rach和抗musk抗体。胸腺增生22例,胸腺瘤6例。所有患者均接受口服抗胆碱酯酶药物对症治疗,36例患者接受糖皮质激素和/或免疫抑制剂(硫唑嘌呤)的背景治疗。9例患者中有4例接受了一个疗程的静脉免疫球蛋白(IVIG)或血浆置换(PE)治疗肌无力危象。40例患者中16例行胸腺切除术。在53个月(IQ=16)的中位门诊随访结束时,纳入研究的40例患者中,6例(15%)死亡,14例(35%)临床缓解稳定,17例(43%)临床部分改善。结论:AMG在布基纳法索存在诊断延迟的问题。几乎所有接受AMG治疗的患者都单独或联合硫唑嘌呤接受抗胆碱酯酶和皮质类固醇治疗。获得IVIG、PE和胸腺切除术的机会仍然有限。死亡率在近六分之一的患者中发生,稳定的临床缓解仅影响约三分之一的患者。为了改善预后,我们需要提供可获得的诊断工具和经证实有效的治疗方法,如胸腺切除术、免疫抑制剂、IVIG和PE。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Treatment and clinical course of autoimmune myasthenia in Burkina Faso].

Introduction: In sub-Saharan Africa, autoimmune myasthenia gravis (AMG) is still poorly known and underdiagnosed (delayed diagnosis, poor availability and accessibility of proven effective diagnostic and therapeutic tools), resulting in a poor functional prognosis and high mortality. The aim of the present study was to evaluate the therapeutic and clinical course of AMG in Burkina Faso.

Patients and methods: This was a longitudinal, multicenter study conducted from March 2015 to April 2023. It included patients with clinical signs suggestive of myasthenia associated with the presence of serum anti-acetylcholine receptor (anti-RACh) antibodies and/or anti-muscle specific kinase (anti-MuSK) antibodies, and/or with the presence of a decrease >10% on electroneuromyography, and/or with a positive therapeutic test to oral anticholinesterase drugs. Data on treatment modalities and clinical evolution were analyzed using Epi InfoTM 7.2.5.0 software. Bivariate analysis with p-value calculation (<0.05) was used to identify factors associated with adverse clinical outcome.

Results: A total of 40 patients with AMG were included, with a female predominance (60%). The median age of onset was 25 years (IQ=7). The median time to neurological consultation and diagnosis was 21 months (IQ=12) and 22 months (IQ=12), respectively. The disease affected young adults in 85% of cases and was generalized in 35 cases. Anti-RACh and anti-MuSK antibodies were present in 22 and 4 of 33 patients, respectively. Thymic hyperplasia and thymoma were found on chest CT in 22 and 6 of 38 patients, respectively. All patients received symptomatic treatment with oral anticholinesterase agents and 36 patients received background treatment with corticosteroids and/or immunosuppressants (azathioprine). Four of 9 patients received a course of intravenous immunoglobulin (IVIG) or plasma exchange (PE) for myasthenic crises. Thymectomy was performed in 16 of the 40 patients. At the end of a median outpatient follow-up of 53 months (IQ=16), of the 40 patients included in the study, 6 (15%) had died, 14 (35%) were in stable clinical remission, and 17 (43%) had partial clinical improvement.

Conclusion: AMG suffers from delayed diagnosis in Burkina Faso. Almost all patients treated for AMG receive anticholinesterase and corticosteroid therapy alone or in combination with azathioprine. Access to IVIG, PE and thymectomy remains limited. Mortality occurs in nearly one in six patients, and stable clinical remission affects only about one third of patients. To improve the prognosis, we need to make available and accessible diagnostic tools and treatments of proven efficacy, such as thymectomy, immunosuppressants, IVIG and PE.

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