Koarada S, Uchida M, Tada Y, Ushiyama O, Suzuki N, Ohta A, Nagasawa K
Department of Internal Medicine, Saga Medical School.
Nihon Rinsho Meneki Gakkai Kaishi. 2000 Apr;23(2):141-7. doi: 10.2177/jsci.23.141.
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.
我们描述了一例最初表现为干燥综合征，后来发展为多发性肌炎和结节病的日本患者。一名67岁女性，有4个月的肌痛病史，于1998年4月入院检查。该患者有10年符合干燥综合征的症状病史。基于肌肉活检和肌电图诊断为多发性肌炎。施墨试验和孟加拉玫瑰红试验阳性以及小唾液腺活检结果均符合干燥综合征。胸部计算机断层扫描发现双侧肺门淋巴结肿大。纵隔淋巴结显微镜检查显示多个非干酪样肉芽肿，有多个上皮样细胞和朗汉斯样巨细胞。基于这些发现诊断为结节病。通过抗体水平升高还检测到丙型肝炎感染。给予患者口服泼尼松龙40mg/天，其肌炎和淋巴结病得到缓解。该患者表现出HLA - B7和HLA - DR8。HLA - DR8通常与这三种疾病相关，HLA - B7在日本患者中也与重叠综合征相关。本病例提示这三种疾病可能有共同的病因背景。此外，还证明了包括HLA表型在内的遗传背景在确定对这些疾病易感性方面的重要性。
