[A case of Sjögren's syndrome complicated by polymyositis and sarcoidosis with HLA-B7 and DR 8: common causes of susceptibility for these diseases].
We describe a case of a Japanese patient initially presenting with Sjögren's syndrome who later developed polymyositis and sarcoidosis. A 67-year-old woman with a 4 month history of myalgia was admitted in April 1998 for examination. The patient had a 10 year history of symptoms consistent with Sjögren's syndrome. A diagnosis of polymyositis was made based on a biopsy of the muscle and an electromyogram. Positive Shirmer and Rose Bengal tests and results of a minor salivary gland biopsy were all consistent with Sjögren's syndrome. Chest computed tomography detected a bilateral hilar lymphadenopathy. Microscopic examination of a mediastinal lymph node demonstrated multiple noncaseating granulomas with multiple epithelioid cells and Langhans-like giant cells. A diagnosis of sarcoidosis was made based on these findings. Hepatitis C infection was also detected by elevated antibody levels. The patient was given 40 mg/day of oral prednisolone and a remission of her myositis and lymphadenopathy was obtained. The patient exhibited HLA-B7 and HLA-DR 8. HLA-DR 8 is commonly associated with these three disorders, and HLA-B7 is also associated with overlap syndrome in Japanese patients. The present case suggested the possibility of a common etiological background for these three disorders. Furthermore, the importance of genetic background, including HLA phenotype, in determining susceptibility to these disorders was demonstrated.
Koarada
S
S
Department of Internal Medicine, Saga Medical School.
Uchida
M
M
Tada
Y
Y
Ushiyama
O
O
Suzuki
N
N
Ohta
A
A
Nagasawa
K
K
D000368:Aged; D004198:Disease Susceptibility; D005260:Female; D015793:HLA-B7 Antigen / Q000097:blood*; D006684:HLA-DR Antigens / Q000097:blood*; D059810:HLA-DR Serological Subtypes; D006801:Humans; D017285:Polymyositis / Q000150:complications* / Q000276:immunology; D012507:Sarcoidosis / Q000150:complications* / Q000276:immunology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology
D015793:HLA-B7 Antigen; D006684:HLA-DR Antigens; D059810:HLA-DR Serological Subtypes; C041559:HLA-DR8 antigen