Rhupus综合征的临床特点

Clinical Features of Rhupus Syndrome

目的探讨Rhupus综合征的临床特点。方法收集2000年1月至2013年3月北京协和医院40例Rhupus综合征患者(Rhupus组)资料,并按1∶2抽取同期无合并类风湿关节炎(rheumatoid arthritis,RA)的系统性红斑狼疮(systemic lupus erythematosus,SLE)患者作对照组,比较两组临床特点。结果 Rhupus综合征患者占同期SLE住院患者的0.93%(40/4301);病程中位数7.0年;77.5%以RA起病。Rhupus组病程长于对照组,其影像学均有骨侵蚀表现。Rhupus组多发性关节炎、对称性关节炎、手关节肿痛、关节畸形、类风湿结节、红细胞沉降率、C反应蛋白升高的发生率及类风湿因子、抗环瓜氨酸肽抗体的阳性率均高于对照组,差异均有统计学意义(均P<0.05)。Rhupus组蝶形红斑、肾脏及神经系统受累、补体C3下降的发生率及SLE疾病活动指数评分均低于对照组,差异均有统计学意义(均P<0.05)。结论 Rhupus综合征少见,多以RA起病,其RA相关表现严重但SLE相关表现较轻,并具有独特的临床表现。特异性抗体和影像学检查有助于诊断。

To analyze the clinical features of Rhupus syndrome. Methods Medical records of 40 Rhupus syndrome who were admitted to Peking Union Medical College Hospital （PUMCH） from 2000 to 2013 were systematically reviewed. Eighty lupus patients without RA coexistence during the same period were randomly selected as the controls. The clinical and laboratory variables were compared between the two groups. Results Forty patients with Rhupus syndrome constituted O. 93% of all the 4301 hospitalized SLE patients during the same period. In our Rhupus patients, SLE with Rheumatoid arthritis （RA） onset was 31 （77.5%）. As compared with lupus patients without coexisting RA, Rhupus patients had longer disease duration（P 〈 0. 05）. With respect to arthritis, rhupus patients were presenting with active, multiple, symmetric joint involvement with more joint deformity and rheumatoid nodules; RF and/or anti-CCP positivity and ESR/CRP levels was significantly higher than in lupus patients without coexisting RA （P 〈 0. 05 ）. Rhupus patients had significantly less butterfly rash, kidney and central nerve system involvement and hypoeomplementemia, SLEDAI score（ P 〈0. 05 ）. Conclusion Rhupus syndrome is relatively rare in clinic, but more common presents as RA initially and with its own unique clinical manifestations characterized by more severe RA-related features while mild SLE-related presentations. Specific autoantibodies and imaging examinations are helpful to the diagnose of this disease.