Frontiers in Medicine (Nov 2020)

Fulminant Course of Neuromyelitis Optica in a Patient With Anti-MDA5 Antibody-Positive Dermatomyositis: A Case Report

  • You-Ri Kang,
  • Kun-Hee Kim,
  • Tai-Seung Nam,
  • Kyung-Hwa Lee,
  • Kyung Wook Kang,
  • Seung-Jin Lee,
  • Seok-Yong Choi,
  • Gopalakrishnan Chandrasekaran,
  • Myeong-Kyu Kim

DOI
https://doi.org/10.3389/fmed.2020.576436
Journal volume & issue
Vol. 7

Abstract

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Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is a myositis-specific marker detected in clinically amyopathic dermatomyositis (DM). DM with anti-MDA5 antibody can be accompanied by rapidly progressive interstitial lung disease (RP-ILD) and other autoimmune disorders. Until now, only one case of neuromyelitis optica (NMO) with anti-MDA5-positive DM has been reported worldwide, in which the patient achieved a favorable outcome with intensive immunotherapy. We report a case of NMO in a patient with anti-MDA5-positive DM complicated by ILD and rheumatoid arthritis. Our patient experienced a fulminant course of NMO, rather than RP-ILD, in the presence of hyperferritinemia, which resulted in profound neurological sequelae despite immunotherapy including rituximab.

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