Gatselis Nikolaos K, Skendros Panagiotis, Ritis Konstantinos, Dalekos George N
Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, University of Thessaly, Larissa, Greece.
First Department of Internal Medicine and Laboratory of Molecular Hematology, School of Medicine, Democritus University of Thrace, Alexandroupolis, Greece.
BMJ Case Rep. 2016 Sep 22;2016:bcr2016216941. doi: 10.1136/bcr-2016-216941.
Familial Mediterranean fever (FMF) is characterised by recurrent, self-limited fever attacks and serositis. Severe liver involvement has rarely been reported. We present two FMF cases of a 55-year-old man and a 20-year-old woman in whom the prevailing manifestations were recurrent unexplained episodes of anicteric hepatitis (man) and recurrent severe jaundice (woman). A long-term history of recurrent self-limited episodes of fever was also claimed in both. After exclusion of infectious, malignant, autoimmune, and liver and biliary diseases, a diagnosis of FMF as confirmed by molecular analysis was established. The patients started colchicine 1 mg/day with immediate resolution of symptoms. During follow-up, no new episodes of fever and exacerbation of liver biochemical parameters have been recorded for 5 and 1 years. Physicians must keep FMF in mind in patients with recurrent episodes of unexplained severe liver impairment and fever and especially in regions like Mediterranean basin where hereditary periodic fever syndromes are common.
家族性地中海热(FMF)的特征为反复出现的自限性发热发作和浆膜炎。严重肝脏受累情况鲜有报道。我们报告了两例FMF病例,一名55岁男性和一名20岁女性,主要表现分别为反复出现不明原因的无黄疸型肝炎发作(男性)和反复出现严重黄疸(女性)。两人均称有长期反复自限性发热发作史。在排除感染性、恶性、自身免疫性以及肝脏和胆道疾病后,经分子分析确诊为FMF。患者开始服用秋水仙碱1毫克/天,症状立即缓解。在随访期间,5年和1年内均未记录到新的发热发作及肝脏生化指标恶化情况。对于反复出现不明原因严重肝功能损害和发热的患者,尤其是在地中海盆地等遗传性周期性发热综合征常见的地区,医生必须考虑到FMF。