Familial inclusion body myositis: a report on two Japanese sisters.
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.
Tateyama
Maki
M
Department of Neurology, Tohoku University School of Medicine, Sendai.
Saito
Naohiro
N
Fujihara
Kazuo
K
Shiga
Yusei
Y
Takeda
Atsushi
A
Narikawa
Koichi
K
Hasegawa
Takafumi
T
Taguchi
Yuzuru
Y
Sakuma
Ryo
R
Onodera
Yoshiaki
Y
Ohnuma
Ayumu
A
Tobita
Muneshige
M
Itoyama
Yasuto
Y
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
D006680:HLA Antigens; D007166:Immunosuppressive Agents