Macía-Villa Cristina C, Zea-Mendoza Antonio
Rheumatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain.
Clin Rheumatol. 2016 Feb;35(2):527-34. doi: 10.1007/s10067-014-2611-5. Epub 2014 Apr 15.
Multicentric reticulohistiocytosis (MRH) is a rare systemic and devastating disease with main involvement of skin and joints, frequently related with malignancies. Macrophages and TNFα are the main targets of multiple treatment options with variable results. Although MRH has the tendency of self-resolve in an average of 8 years, treatment must be started early to avoid sequelae, mainly in joints with severe deformities and daily life activity impairment. We describe a 50-year-old man with skin and joint involvement, with a non-previously described muscle affectation, that failed to different drugs and achieved skin improvement with infliximab. We review for the first time in medical literature the available therapeutic options of MRH and its outcomes and propose possible future targets.
多中心网状组织细胞增多症（MRH）是一种罕见的全身性破坏性疾病，主要累及皮肤和关节，常与恶性肿瘤相关。巨噬细胞和肿瘤坏死因子α（TNFα）是多种治疗方案的主要靶点，但疗效各异。尽管MRH有平均8年自行缓解的倾向，但必须尽早开始治疗以避免后遗症，尤其是关节严重畸形和日常生活活动受限的情况。我们描述了一名50岁男性，有皮肤和关节受累，伴有此前未描述过的肌肉病变，对多种药物治疗无效，使用英夫利昔单抗后皮肤状况改善。我们首次在医学文献中回顾了MRH现有的治疗选择及其结果，并提出了可能的未来靶点。
