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伴发重症肌无力的巨大细胞肌炎:文献病例回顾。

Giant cell myositis associated with concurrent myasthenia gravis: a case-based review of the literature.

机构信息

Division of Rheumatology, Tufts Medical Center, 800 Washington St, Boston, MA, 02111, USA.

Medical Scientist Training Program, Tufts Graduate School of Biomedical Sciences, Tufts University School of Medicine, 136 Harrison Avenue, Boston, MA, 02111, USA.

出版信息

Clin Rheumatol. 2021 Sep;40(9):3841-3851. doi: 10.1007/s10067-021-05619-5. Epub 2021 Feb 25.

Abstract

The term "giant cell myositis" has been used to refer to muscle diseases characterized histologically by multinucleated giant cells. Myasthenia gravis is an autoimmune neuromuscular junction disorder. The rare concurrence of giant cell myositis with myasthenia gravis has been reported; however, the clinical and histological features have varied widely. Here, we present such a case and a review of the literature. An 82-year-old woman admitted for subacute, progressive, proximal muscle weakness developed acute-onset dysphagia, dysphonia, and respiratory distress 5 days after admission. Laboratory findings were positive for acetylcholine receptor binding antibodies and striational muscle antibodies against titin. Muscle biopsy demonstrated widespread muscle fiber necrosis with multinucleated giant cells, consistent with giant cell myositis. She died despite treatment with pulse methylprednisolone and plasma exchange. A literature review of the PubMed and Scopus databases from 1944 to 2020 identified 15 additional cases of these co-existing diagnoses. We found that giant cell myositis with myasthenia gravis primarily affects female patients, is typically diagnosed in the 6-7th decades, and is characterized by the presence of thymoma. Muscle histology predominantly shows giant cell infiltrate without granulomas. The onset of myasthenia gravis symptoms may precede, follow, or coincide with symptoms of myositis. Treatment with thymectomy, anticholinesterase inhibitors, or immunosuppressive therapy may lead to favorable clinical outcomes.

摘要

“巨细胞肌炎”一词用于指代肌肉疾病,其组织学特征为多核巨细胞。重症肌无力是一种自身免疫性神经肌肉接头疾病。巨细胞肌炎与重症肌无力同时发生的情况较为罕见,但临床表现和组织学特征差异较大。本文报告了这样一例病例,并进行了文献复习。一名 82 岁女性因亚急性、进行性、近端肌肉无力入院,入院后 5 天出现急性吞咽困难、发音困难和呼吸困难。实验室检查乙酰胆碱受体结合抗体和针对titin 的横纹肌抗体阳性。肌肉活检显示广泛的肌肉纤维坏死伴多核巨细胞,符合巨细胞肌炎。尽管接受了脉冲甲基泼尼松龙和血浆置换治疗,她仍死亡。对 1944 年至 2020 年 PubMed 和 Scopus 数据库的文献进行回顾,共确定了 15 例这些共存诊断的额外病例。我们发现,重症肌无力合并巨细胞肌炎主要影响女性患者,通常在 6-7 十年代诊断,其特征是胸腺瘤存在。肌肉组织学主要表现为巨细胞浸润,无肉芽肿。重症肌无力症状的发作可能早于、晚于或与肌炎症状同时出现。胸腺切除术、胆碱酯酶抑制剂或免疫抑制治疗可能导致良好的临床结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2342/7904393/e0f49b053657/10067_2021_5619_Fig1_HTML.jpg

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