Familial inclusion body myositis: A report on two Japanese sisters

Maki Tateyama, Naohiro Saito, Kazuo Fujihara, Yusei Shiga, Atsushi Takeda, Koichi Narikawa, Takafumi Hasegawa, Yuzuru Taguchi, Ryo Sakuma, Yoshiaki Onodera, Ayumu Ohnuma, Muneshige Tobita, Yasuto Itoyama

Research output: Contribution to journalArticlepeer-review

18 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1035-1038
Number of pages4
JournalInternal Medicine
Issue number10
Publication statusPublished - 2003 Oct


  • Azathioprine
  • HLA
  • Inclusion body myositis
  • MRI
  • Muscle biopsy
  • Predonisolone


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