Garcia-Gonzalez A, Weisman M H
Department of Medicine, University of California, San Diego 92103.
Semin Arthritis Rheum. 1992 Dec;22(3):139-50. doi: 10.1016/0049-0172(92)90014-5.
Familial Mediterranean fever (FMF) is a disease of unknown etiology and pathogenesis. In addition to fever, arthritis is among its most frequent manifestations. The arthritis of FMF is typically an acute, episodic, self-limited process with no sequelae. The radiographic features of FMF arthritis are usually limited to transient, often severe osteoporosis. Synovial fluid analysis many mimic septic arthritis with very high white blood cell counts; cultures are uniformly negative. The course of FMF is almost always benign, with no residual articular incapacity. Some patients, limited to certain ethnic groups, develop renal amyloidosis. Colchicine therapy modifies the natural history of the disease by decreasing the attack frequency and preventing amyloid deposition. At present, a lipocortin deficiency appears to be the likely candidate for a pathogenic mechanism. An unusual case with dramatic periarticular features (periostitis) and a protracted course with an excellent response to synovectomies is reported here. There is no explanation for the exuberant periarticular bone formation noted in this case, but a variety of recently discovered growth factors may be implicated.
家族性地中海热（FMF）是一种病因和发病机制不明的疾病。除发热外，关节炎是其最常见的表现之一。FMF关节炎通常是一个急性、发作性、自限性过程，无后遗症。FMF关节炎的影像学特征通常仅限于短暂的、往往严重的骨质疏松。滑膜液分析可能类似于败血症性关节炎，白细胞计数非常高；培养结果均为阴性。FMF的病程几乎总是良性的，无残留关节功能障碍。一些仅限于特定种族群体的患者会发展为肾淀粉样变性。秋水仙碱治疗通过降低发作频率和预防淀粉样蛋白沉积来改变疾病的自然病程。目前，脂皮质素缺乏似乎是致病机制的可能候选因素。本文报道了一例具有显著关节周围特征（骨膜炎）且病程迁延、滑膜切除术后反应良好的罕见病例。该病例中出现的过度关节周围骨形成尚无解释，但可能涉及多种最近发现的生长因子。
