CAJ | 학술논문

目的：分析多中心网状组织细胞增生症（multicentric reticulohistiocytosis，MRH）临床表现、病理改变、治疗及随访结果。方法回顾性分析北京协和医院1998年1月至2014年10月确诊为 MRH 患者的临床资料。结果共纳入8例MRH 患者。8例患者均有典型的皮疹，7例有损毁性关节炎，3例合并皮肌炎样皮疹。2例以关节症状为首发表现，误诊为类风湿关节炎。激素联合免疫抑制剂治疗，随诊1个月～10年，患者病情稳定，无伴随肿瘤证据。结论MRH 易误诊，特别是以关节为首发症状的患者。临床上既要鉴别 MRH 与结缔组织疾病，也应注意这2种疾病共存的情况。
목적：분석다중심망상조직세포증생증（multicentric reticulohistiocytosis，MRH）림상표현、병리개변、치료급수방결과。방법회고성분석북경협화의원1998년1월지2014년10월학진위 MRH 환자적림상자료。결과공납입8례MRH 환자。8례환자균유전형적피진，7례유손훼성관절염，3례합병피기염양피진。2례이관절증상위수발표현，오진위류풍습관절염。격소연합면역억제제치료，수진1개월～10년，환자병정은정，무반수종류증거。결론MRH 역오진，특별시이관절위수발증상적환자。림상상기요감별 MRH 여결체조직질병，야응주의저2충질병공존적정황。
Objective To investigate the clinical manifestations,pathological changes,treatment,and outcome of multicentric reticulohistiocytosis (MRH)patients.Methods The clinical data of 8 MRH patients in Peking Union Medical College Hospital from January 1998 to October 2014 were retrospectively analyzed.Results Eight MRH patients were studied.Eight patients had typical skin rash,7 patients had erosive arthritis,3 patients had dermatomyositis-like skin lesions.Two patients with joint symptoms as the first manifestation of MRH, and were misdiagnosed as rheumatoid arthritis.Steroids combined with immunosuppressant therapy were used for treatment;patients were in stable condition during 1 month to 10 years follow-up period.No evidence of tumor was found.Conclusion MRH may be misdiagnosed easily, especially for patients with joint symptoms as the first manifestation.It’s important to different MRH from connective tissue diseases.Rheumatologists should aware that MRH could coexist with an autoimmune disease sometimes.