A case of anti-titin antibody positive nivolumab-related necrotizing myopathy with myasthenia gravis

Aiko Isami, Ayaka Uchiyama, Yuichi Shimaoka, Shigeaki Suzuki, Izumi Kawachi, Nobuya Fujita

Research output: Contribution to journalArticle


A 53-year-old man suffering from squamous cell lung cancer presented with bilateral ptosis and bulbar palsy a month after initial treatment with the immune checkpoint inhibitor nivolumab. The symptoms showed worsening from midday, suggesting myasthenia gravis (MG), although anti-AChR antibody was negative. Although no muscle weakness was detected, the CK level was elevated to 5,255 IU/l, and MRI of the thigh revealed inflammation of the bilateral rectus femoris muscle. A muscle biopsy showed signs of necrotizing myopathy with expression of sarcolemmal HLA class I and accumulation of macrophages, CD4, CD8, and CD20-positive lymphocytes. Positivity for anti-titin antibody, one of the anti-striated muscle antibodies, was evident. The patient was diagnosed as having nivolumab-related necrotizing myopathy with myasthenia gravis, an immune-related adverse event (irAE). Treatment with prednisolone rapidly ameliorated the symptoms, and the serum CK level normalized. There have been several reports of nivolumab-related myositis with MG. On the basis of the muscle pathology and antibody data, we were able to clarify that necrotizing myopathy was related to the pathogenesis of this case.

Original languageEnglish
Pages (from-to)431-435
Number of pages5
JournalRinsho shinkeigaku = Clinical neurology
Issue number7
Publication statusPublished - 2019 Jul 31



  • anti-striated muscle antibody
  • immune-related adverse events (irAE)
  • myasthenia gravis
  • myopathy
  • nivolumab

ASJC Scopus subject areas

  • Clinical Neurology

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