Recurrent HyperCKemia with Immunological Involvement of the Endomysial Capillaries in Neuromyelitis Optica

Intern Med. 2020 Dec 1;59(23):3079-3083. doi: 10.2169/internalmedicine.4600-20. Epub 2020 Aug 4.

Abstract

A 55-year-old woman with neuromyelitis optica (NMO) had recurrent myalgias with hyperCKemia. A muscle biopsy suggested nonspecific myopathic changes. Regarding immunohistochemistry, the expression of both major histocompatibility complex class I and myxovirus resistance protein A was observed in the endomysial capillaries, suggesting immunological involvement of these capillaries, whereas both C5b9 (membrane attack complex) and aquaporin 4 immunofluorescence stainings were normal. The present findings led us to conclude that one possible mechanism for hyperCKemia in NMO underlying the immunological involvement of the endomysial capillaries was an as-yet-unidentified factor that triggered damage to the integrity of the sarcolemma and thereby cause CK leakage into the serum.

Keywords: endomysial capillaries; hyperCKemia; major histocompatibility complex class I; myxovirus resistance protein A; neuromyelitis optica.

Publication types

  • Case Reports

MeSH terms

  • Aquaporin 4 / immunology
  • Autoantibodies / blood
  • Creatine Kinase / blood*
  • Female
  • Humans
  • Middle Aged
  • Muscle, Skeletal / blood supply*
  • Neuromyelitis Optica / complications*
  • Neuromyelitis Optica / pathology

Substances

  • Aquaporin 4
  • Autoantibodies
  • Creatine Kinase