A rare case of sporadic inclusion body myositis and rheumatoid arthritis exhibiting ectopic lymphoid follicle-like structures: a case report and literature review

Kazutoshi Konomatsu, Rumiko Izumi, Naoki Suzuki, Yoshiki Takai, Yuko Shirota, Ryoko Saito, Hiroshi Kuroda, Masashi Aoki

Research output: Contribution to journalArticlepeer-review

Abstract

Sporadic inclusion body myositis (sIBM) is a degenerative, intractable, inflammatory myopathy with an immune pathomechanism. We report on a case of a 44-year-old Japanese man who began developing progressive muscle weakness at age 40. Rheumatoid arthritis symptoms manifested at 43 with strongly positive anti-cyclic citrullinated peptide antibodies. Along with typical sIBM pathology, a muscle biopsy revealed dramatic inflammation with prominent perivascular B-cell infiltration forming ectopic lymphoid follicle-like structures (ELFLSs). Exome sequencing identified no causative variants of hereditary myopathy or immune disorders. A combination of immunotherapy slowed the progression of the muscular symptoms. This unusual form of sIBM, including earlier age at onset, a partial response to immunotherapy, and a histopathology presenting B-cell infiltrate with ectopic lymphoid follicle-like structures, indicates a possible association of rheumatoid arthritis and heterogeneity with the autoimmune involvement of sIBM. We review the clinical and pathological features of patients with rheumatoid arthritis associated sIBM in the literature.

Original languageEnglish
Pages (from-to)870-876
Number of pages7
JournalNeuromuscular Disorders
Volume31
Issue number9
DOIs
Publication statusPublished - 2021 Sep

Keywords

  • Ectopic lymphoid follicle-like structure
  • Perivascular B-cell infiltration
  • Rheumatoid arthritis
  • Sporadic inclusion body myositis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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