Section of Rheumatology, Department of Clinical and Experimental Medicine, University of Ferrara, Ferrara, Italy.
Best Pract Res Clin Rheumatol. 2012 Aug;26(4):543-57. doi: 10.1016/j.berh.2012.07.006.
Multicentric reticulohistiocytosis (MRH) and fibroblastic rheumatism (FR) are uncommon disorders with similar joint and skin manifestations. They are usually included among the non-Langerhans histiocytoses, but recent insights drive some criticism. The diagnosis is often challenging and must be confirmed by the histological typical features. If the skin manifestations are missing, the arthritic complaints may be confused with those of other rheumatic disorders. In these cases, only a careful clinical and radiological evaluation leads to the correct diagnosis. The natural course of the diseases may rapidly develop into disabling manifestations, making an aggressive treatment strongly recommendable. There is emerging evidence that anti-tumour necrosis factor-α agents and bisphosphonates are promising drugs for MRH, while a course of methotrexate and steroids seems to be the best option for FR. Finally, the clinician should be aware that in many cases MRH, but not FR, is associated with a large number of systemic manifestations and with malignancy. This eventuality must be accurately ruled out.
多中心网状组织细胞增生症 (MRH) 和纤维组织细胞性风湿症 (FR) 是两种罕见疾病，它们具有相似的关节和皮肤表现。它们通常被归入非朗格汉斯细胞组织细胞增多症，但最近的研究结果对此提出了一些批评。诊断通常具有挑战性，必须通过组织学的典型特征来确认。如果皮肤表现缺失，关节炎的症状可能与其他风湿性疾病的症状相混淆。在这些情况下，只有仔细的临床和影像学评估才能得出正确的诊断。疾病的自然病程可能迅速发展为致残表现，因此强烈推荐采用积极的治疗方法。有新的证据表明，抗肿瘤坏死因子-α 药物和双膦酸盐类药物对 MRH 具有良好的疗效，而甲氨蝶呤和类固醇类药物的疗程似乎是 FR 的最佳选择。最后，临床医生应该意识到，在许多情况下，MRH 但不是 FR 与大量全身表现和恶性肿瘤相关。必须准确排除这种可能性。
