结缔组织病合并结节病患者的临床特点

Clinical features of patients with connective tissue disease combined with sarcoidosis

目的探讨CTD与结节病共存患者的临床特点,减少漏诊误诊。方法回顾分析1985年1月至2021年12月在北京协和医院收治的结缔组织病合并结节病患者的临床资料、实验室、影像学及病理资料。结果CTD合并结节病患者17例,SS10例,DM2例,原发性胆汁性胆管炎(PBC)2例,SLE1例,RA1例,未分化结缔组疾病(UCTD)1例,平均年龄(55±10)岁,男女比例1:16。CTD先发病8例,结节病先发病3例,同期发病6例。结节病发病时主要表现为:淋巴结肿大(12/17)、肺结节(8/17)、皮下结节(4/17)、皮疹(4/17)、视物模糊(1/17)。治疗:9例患者优先结节病治疗,5例患者按照CTD治疗;3例患者2种疾病同时治疗。预后:经治疗,17例均好转出院。结论CTD和结节病共存时,结节病容易被CTD掩盖,寻找结节病的特异性临床表现及病理特征十分重要。

Objective To investigate the clinical features of patients with coexisting connective tissue disease(CTD)and sarcoisosis and to avoid misdiagnosis and mistreatment.Methods To analyze the clinical manifestations,laboratory data,imaging and pathological features of patients with CTD combined with sarcoidosis in Peking Union Medical College Hospital from January 1985 to December 2021.Results There were 17 patients with CTD(including 10 SS,2 DM,2 PBC,1 SLE,1 RA and 1 UCTD),combined with sarcoidosis,with a mean age of(55±10)years old and the ratio of male-to-female was 1:16.Eight patients were diagnosed as CTD before sarcoidosis,while 3 patients after sarcoidosis.The other 6 patients were diagnosed with the two diseases almost simultaneously.Lymphadenopathy(12/17),pulmonary nodules(8/17),subcutaneous nodules(4/17),rash(4/17)and blurred vision(1/17)were the main manifestations of patients with the onset of nodular disease.Nine patients were treated based on the presentation of sardoisis and 5 patients for CTD;3 patients were treated for both diseases at the same time.All 17 patients discharged with improvement after treatment.Conclusion When sarcoidosis do coexists with CTD,occult CTD might occur.It is important to investigate specific manifestations including pathological features of sarcoidosis and differentiate it from CTD.