Familial inclusion body myositis: a report on two Japanese sisters

Intern Med. 2003 Oct;42(10):1035-8. doi: 10.2169/internalmedicine.42.1035.

Abstract

Familial occurrence of inclusion body myositis is extremely rare, and only a few cases in Western countries have been reported. In these reports, a strong association of this disease with DR3 (DRB1*0301/0302) and the efficacy of immunosuppressants suggested that an immune pathomechanism is involved in the disease. We, for the first time, report two Japanese sisters who suffered myopathy clinicopathologically similar to inclusion body myositis. One sister received corticosteroid and azathioprine and the therapy relieved dysphagia. Both of our patients had DR15(2)/4 (DRB1*1502/0405), suggesting a distinct genetic association with the disease in the Japanese population.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Biopsy
  • Female
  • Genetic Predisposition to Disease
  • HLA Antigens / immunology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Muscle, Skeletal / pathology*
  • Myositis, Inclusion Body / drug therapy
  • Myositis, Inclusion Body / genetics*
  • Myositis, Inclusion Body / immunology*
  • Treatment Outcome

Substances

  • HLA Antigens
  • Immunosuppressive Agents