摘要
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。
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.
出处
《中华临床免疫和变态反应杂志》
2015年第3期213-217,F0003,共6页
Chinese Journal of Allergy & Clinical Immunology
