title: Familial inclusion body myositis: a report on two Japanese sisters.
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.
authors: Department of Neurology, Tohoku University School of Medicine, Sendai.
authors: Maki
authors: M
authors: Tateyama
authors: Naohiro
authors: N
authors: Saito
authors: Kazuo
authors: K
authors: Fujihara
authors: Yusei
authors: Y
authors: Shiga
authors: Atsushi
authors: A
authors: Takeda
authors: Koichi
authors: K
authors: Narikawa
authors: Takafumi
authors: T
authors: Hasegawa
authors: Yuzuru
authors: Y
authors: Taguchi
authors: Ryo
authors: R
authors: Sakuma
authors: Yoshiaki
authors: Y
authors: Onodera
authors: Ayumu
authors: A
authors: Ohnuma
authors: Muneshige
authors: M
authors: Tobita
authors: Yasuto
authors: Y
authors: Itoyama
mesh_terms: D000368:Aged; D001706:Biopsy; D005260:Female; D020022:Genetic Predisposition to Disease; D006680:HLA Antigens / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000473:pathology*; D018979:Myositis, Inclusion Body / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D006680:HLA Antigens; D007166:Immunosuppressive Agents