Pathologic Features of Anti-Mi-2 Dermatomyositis

Jantima Tanboon, Michio Inoue, Shinya Hirakawa, Hisateru Tachimori, Shinichiro Hayashi, Satoru Noguchi, Shigeaki Suzuki, Naoko Okiyama, Manabu Fujimoto, Ichizo Nishino

Research output: Contribution to journalArticlepeer-review

Abstract

OBJECTIVE: To identify the characteristic pathologic features of dermatomyositis (DM) associated with anti-Mi-2 autoantibodies (anti-Mi-2 DM). METHODS: We reviewed 188 muscle biopsies from patients (1) pathologically diagnosed with DM through the sarcoplasmic expression for the myxovirus-resistant protein A and (2) serologically positive for 1 of 5 DM-specific autoantibodies (DMSAs) (anti-Mi-2, n = 30; other DMSAs, n = 152) or negative for all 5 DMSAs (n = 6). We then compared the histopathologic and immunohistochemical features of patients with anti-Mi-2 DM to those with non-Mi-2 DM and patients with anti-synthetase syndrome (ASS) (n = 212) using the t test, Fisher exact test, and a logistic regression model. RESULTS: Patients with anti-Mi-2 DM showed significantly higher severity scores in muscle fiber and inflammatory domains than non-Mi-2 DM patients. The presence of perifascicular necrosis, increased perimysial alkaline phosphatase activity, and sarcolemmal membrane attack complex deposition was more frequent in patients with anti-Mi-2 DM (p < 0.01). After Bonferroni correction, there were no significant differences in the percentages of the features mentioned above between the patients with anti-Mi-2 DM and those with ASS (p > 0.01). CONCLUSION: Perifascicular necrosis and perimysial pathology, features previously reported in ASS, are common in patients with anti-Mi-2 DM. Our findings not only assist in differentiating anti-Mi-2 DM from other DM subtypes but also suggest the possibility of an overlapping mechanism between anti-Mi-2 DM and ASS. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the muscle biopsies of DM patients with anti-Mi-2 autoantibodies are more likely to demonstrate higher severity scores in muscle fiber and inflammatory domains.

Original languageEnglish
Pages (from-to)e448-e459
JournalNeurology
Volume96
Issue number3
DOIs
Publication statusPublished - 2021 Jan 19

ASJC Scopus subject areas

  • Clinical Neurology

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