Multicentric reticulohistiocytosis and fibroblastic rheumatism

• Published in: Best practice & research. Clinical rheumatology 2012-08, Vol.26 (4), p.543-557
• Main Authors: Trotta, Francesco, Colina, Matteo, MD, PhD
• Format: Article
• Language: English
• Subjects: Arthritis - diagnosis; Arthritis - drug therapy; Erosive arthritis; Fibroblastic rheumatism; Histiocytosis, Non-Langerhans-Cell - classification; Histiocytosis, Non-Langerhans-Cell - diagnosis; Histiocytosis, Non-Langerhans-Cell - drug therapy; Humans; Multicentric reticulohistiocytosis; Non-Langerhans histiocytoses; Paraneoplastic syndromes; Rare Diseases - classification; Rare Diseases - diagnosis; Rare Diseases - drug therapy; Rheumatic Diseases - classification; Rheumatic Diseases - diagnosis; Rheumatic Diseases - drug therapy; Rheumatology
• ISSN: 1521-6942; 1532-1770
• 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.