Inflammatory myopathy with myasthenia gravis Thymoma association and polymyositis pathology

被引：29

作者：

Uchio, Naohiro ^{[1
]}

Taira, Kenichiro ^{[1
]}

Ikenaga, Chiseko ^{[1
]}

Kadoya, Masato ^{[2
]}

Unuma, Atsushi ^{[1
]}

Yoshida, Kenji ^{[6
]}

Nakatani-Enomoto, Setsu ^{[6
]}

Hatanaka, Yuki ^{[5
]}

Sakurai, Yasuhisa ^{[4
]}

Shiio, Yasushi ^{[3
]}

Kaida, Kenichi ^{[2
]}

Kubota, Akatsuki ^{[1
]}

Toda, Tatsushi ^{[1
]}

Shimizu, Jun ^{[1
]}

机构：

[1] Univ Tokyo, Grad Sch Med, Dept Neurol, Tokyo, Japan

[2] Natl Def Med Coll, Dept Internal Med, Div Neurol, Saitama, Japan

[3] Tokyo Teishin Hosp, Div Neurol, Tokyo, Japan

[4] Mitsui Mem Hosp, Dept Neurol, Tokyo, Japan

[5] Teikyo Univ, Sch Med, Dept Neurol, Tokyo, Japan

[6] Fukushima Med Univ, Dept Neurol, Fukushima, Japan

来源：

NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION | 2019年 / 6卷 / 02期

关键词：

MYOCARDITIS; WOMAN; RECEPTOR; DISEASE; IMMUNE; CTLA-4;

D O I：

10.1212/NXI.0000000000000535

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective To provide evidence that idiopathic inflammatory myopathy (IM) with myasthenia gravis (MG) frequently shows thymoma association and polymyositis (PM) pathology and shares clinicopathologic characteristics with IM induced by immune checkpoint inhibitors (ICIs). Methods We analyzed the clinicopathologic features of 10 patients with idiopathic IM and MG identified in 970 consecutive patients with biopsy-proven IM. Results Seven patients (70%) had thymoma. IM and MG were diagnosed with more than 5-year time difference in 6 thymomatous patients and within 1 year in 1 thymomatous and 3 non-thymomatous patients. Seven thymomatous patients showed rhabdomyolysis-like features with respiratory failure (4/7), dropped head (3/7), cardiac involvement (2/7), and positive anti-acetylcholine receptor (anti-AChR) and anti-titin antibodies (7/7 and 4/6, respectively) but rarely showed ocular symptoms (2/7) or decremental repetitive nerve stimulation (RNS) responses (1/7) at IM diagnosis. Three non-thymomatous patients showed acute cardiorespiratory failure with rhabdomyolysis-like features (1/3), positive anti-AChR and anti-titin antibodies (3/2 and 2/2, respectively), and fluctuating weakness of the skeletal muscle without ocular symptoms (3/3). Muscle pathology showed a PM pathology with infiltration of CD8-positive CD45RA-negative T-lymphocytes (9/9), scattered endomysial programmed cell death 1 (PD-1)-positive cells (9/9), and overexpression of programmed cell death ligand 1 (PD-L1) on the sarcolemma of muscle fibers around the infiltrating PD-1-positive cells (7/9). Conclusion Rhabdomyolysis-like features, positive anti-AChR antibody without decremental RNS responses, and PD-L1 overexpression are possible characteristics shared by ICI-induced IM. Frequent thymoma association in patients with idiopathic IM and MG may suggest thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway.

引用

页数：11

共 40 条

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