结节病并存原发性干燥综合征四例并文献复习

Coexistence of sarcoidosis and primary Sj（o）gren syndrome： a clinical analysis and literature review

回顾性分析4例结节病并存原发性干燥综合征（pSS）的临床特点并复习相关文献.4例患者均为女性,均以呼吸道症状就诊,抗核抗体阳性2例,抗SSA抗体阳性1例,所有患者均有结节病和干燥综合征的组织病理学证据支持诊断.文献复习41例（包括本组报道的4例）,其中女性37例,男性4例,眼干40例,口干38例,肺门、纵隔淋巴结肿大28例,间质性肺炎15例,有呼吸道症状13例,抗核抗体阳性23例,抗SSA抗体阳性19例,抗SSB抗体阳性10例,类风湿因子阳性12例,所有患者均有两种疾病的组织病理学证据.结节病并存pSS时有多系统受累、自身抗体阳性,但最终确诊还需组织病理学依据.

Four patients with coexistence of sarcoidosis and primary Sj（o）gren syndrome （pSS） were retrospectively analyzed.All patients were female,who were referred to our department mainly because of respiratory symptoms.Positive antinuclear antibody （ANA） was detected in 2 patients and anti-Sj（o）grens syndrome A （SSA） antibody positive in 1 patient.All patients presented specific histologic patterns of both sarcoidosis and pSS.Publications related to coexistence of these two diseases were reviewed.Forty-one patients were finally included in the analysis,among whom 37 confirmed patients were from literature search.There were 37 women and 4 men.The main clinical features presentation were xerophthalmia in 40,xerostomia in 38,hilaradenopathies in 28,interstitial lung disease in 15,respiratory symptoms in 13.The main immunologic data were positive ANA in 23,SSA antibody in 19,anti-Sj（o）grens syndrome B antibody in 10 and rheumatoid factor in 12.All patients presented specific histologic patterns of both diseases.Patients with both sarcoidosis and pSS of ten represent multisystemic involvement and positive immunologic parameters,as well as the dual expression of specific histologic characteristics.