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[重症肌无力的治疗]

[Treatment of myasthenia gravis].

作者信息

Wakata N

机构信息

Fourth Department of Internal Medicine, Toho University.

出版信息

Rinsho Shinkeigaku. 1999 Dec;39(12):1222-5.

Abstract

The strategy of myasthenia gravis has been progressed during recent 30 years. Extended thymectomy and alternate-day prednisolone administration remarkably improved therapeutic effect in myasthenia gravis. Besides of these therapies, plasmapheresis and immunoadsorption plasmapheresis extremely improved mortality in myasthenia gravis. 1. Corticosteroid therapy Usually initial doses of PSL is 20-30 mg every other day in the morning after thymectomy, and increased 5 mg every on-day. Maximum PSL doses is 80-100 mg alternate-day, then this doses will be maintained over 4 weeks. After these procedures, PSL is gyradually decreased and discontinued within 2-3 years. 2. Thymectomy Extended transsternal thymectomy is the most useful method, because many authors have reported that re-thymectomy reveals re-appearance of thymus. 3. Invasive thymoma Although this type of thymoma has been called malignant thymoma, it should be called as invasive thymoma since any thymoma can invade surrounding tissues for a long subclinical period. Strategy of treating invasive thymoma is PSL administration, radiation and chemotherapy. Chemotherapy should be performed depend on histopathological findings of thymoma. CHOP therapy is useful for the lymphocytic cell predominant type of thymoma, however CHOP + cis-platin therapy is useful for the epithelial cell predominant type. Treatment of myasthenia gravis has been improved, however it is still unclear that how to treat invasive thymoma, and how to prevent re-appearance of symptoms by decrease or discontinuity of PSL administration.

摘要

重症肌无力的治疗策略在最近30年里取得了进展。扩大胸腺切除术和隔日给予泼尼松显著提高了重症肌无力的治疗效果。除了这些治疗方法外,血浆置换和免疫吸附血浆置换极大地降低了重症肌无力的死亡率。1. 皮质类固醇治疗 通常在胸腺切除术后,PSL的初始剂量为隔日早晨20 - 30毫克,并在服药日每次增加5毫克。PSL的最大剂量为隔日80 - 100毫克,然后维持该剂量4周以上。经过这些步骤后,PSL逐渐减量并在2 - 3年内停药。2. 胸腺切除术 扩大经胸骨胸腺切除术是最有效的方法,因为许多作者报道再次胸腺切除术会发现胸腺重新出现。3. 侵袭性胸腺瘤 虽然这种类型的胸腺瘤曾被称为恶性胸腺瘤,但由于任何胸腺瘤在很长的亚临床期都可能侵袭周围组织,所以应称为侵袭性胸腺瘤。治疗侵袭性胸腺瘤的策略是给予PSL、放疗和化疗。化疗应根据胸腺瘤的组织病理学结果进行。CHOP方案对淋巴细胞为主型胸腺瘤有用,然而CHOP + 顺铂方案对上皮细胞为主型胸腺瘤有用。重症肌无力的治疗已经有所改善,然而如何治疗侵袭性胸腺瘤以及如何通过减少或停用PSL来预防症状复发仍不清楚。

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