Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Familial Inclusion Body Myositis: A Report on Two Japanese Sisters
Maki TATEYAMANaohiro SAITOKazuo FUJIHARAYusei SHIGAAtsushi TAKEDAKoichi NARIKAWATakafumi HASEGAWAYuzuru TAGUCHIRyo SAKUMAYoshiaki ONODERAAyumu OHNUMAMuneshige TOBITAYasuto ITOYAMA
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2003 Volume 42 Issue 10 Pages 1035-1038

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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 (DRB 1*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.
(Internal Medicine 42: 1035-1038, 2003)

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© The Japanese Society of Internal Medicine
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