重症Rhupus综合征三例临床特征分析及文献复习

Clinical Features of Severe Rhupus Syndrome: Three Cases Report and Literature Review

目的总结类风湿关节炎(RA)与系统性红斑狼疮(SLE)重叠的Rhupus综合征的临床特点,提高临床医师对本病的认识。方法回顾性分析株洲市中心医院收治的3例重症Rhupus综合征患者的临床特点、诊治经过,并检索国内外文献进行总结。结果 3例患者均为中年女性,均以关节炎起病,经过长病程出现SLE特征性表现,除侵蚀性关节炎外,还有严重脏器受累,表现为急性横贯性脊髓炎、重度溶血性贫血、大量心包积液及肾病综合征,均有多种RA及SLE特异性抗体阳性,经过大剂量激素或激素冲击,并联合环磷酰胺等免疫抑制剂治疗,病情明显改善。结论 Rhupus综合征少见,常以RA起病,关节损害严重,脏器受累一般较轻,但需提高警惕Rhupus综合征也可出现重要脏器受累,及时恰当的诊治对改善预后至关重要。

Objective The Rhuptis syndrome has RA symptom and SLE symptom simultaneously. In order to improve the understanding of this disease among clinicians, the clinical features of Rhupus syndrome are summarized. Methods The clinical features, diagnosis and treatment of 3 patients with severe Rhupus syndrome were analyzed retrospectively, and related literatures at home and abroad were reviewed and summarized. Results 3 patients were middle -aged females, the initial manifestation was arthritis. Characteristic symptoms of SLE manifested after a long course, such as erosive osteoarthritis and serious organ involvement （acute transverse myelitis, severe autoimmune hemolytic anemia, a large amount of pericardial effusion and nephrotic syndrome）, many .RA -specific and SLE -specific antibodies were positive, the clinical signs and symptoms improved significantly after high - dose corticosteroids or methylprednisolone pulse therapy combined with immunosuppressant （ such as cyclophosphamide） treatment. Conclusion Rhupus syndrome is relatively rare in clinic, the initial manifestation is often symptom of RA, joint damage is severe, but organ involvement is usually relatively light, but vital organs may be involved, which should evoke clinicians＇ vigilance. Timely and correct diagnosis and treatment is essential to prognosis improvement.