Radisic Marcelo, Santamarina Joaquin, Froment Roberto
Internal Medicine, Sanatorio de la Trinidad (Palermo), Capital Federal, Argentina.
Clin Rheumatol. 2006 Nov;25(6):914-6. doi: 10.1007/s10067-005-0093-1. Epub 2005 Dec 3.
Familial Mediterranean fever (FMF) is a genetic disorder characterized by sporadic, acute attacks of fever and serosal inflammation. Typical manifestations are recurrent febrile episodes of acute instauration for brief duration (1 to 4 days) that is associated with severe pain due to serositis at one or more sites. Abdominal crisis occurs in 95% of the patients. Treatment with colchicine is highly effective as preventive treatment, but it is considered to be ineffective for the treatment of established acute attacks. As mentioned, untreated crisis resolves spontaneously in 1 to 4 days. Prolonged, nonresolving crisis of abdominal pain refractory to nonsteroidal anti-inflammatory drugs (NSAIDs) and opioids, with fever and elevation of acute phase reactants that resolves after the administration of colchicine, is a clinical presentation undescribed hitherto. The aim of this paper is to report a patient with this distinctively unusual clinical presentation of FMF.
家族性地中海热(FMF)是一种遗传性疾病,其特征为散发性急性发热和浆膜炎症。典型表现为短暂的(1至4天)急性复发性发热发作,伴有一个或多个部位因浆膜炎导致的严重疼痛。95%的患者会出现腹部危机。秋水仙碱作为预防性治疗非常有效,但对于已确诊的急性发作治疗被认为无效。如前所述,未经治疗的危机会在1至4天内自行缓解。腹痛持续不缓解、对非甾体抗炎药(NSAIDs)和阿片类药物难治、伴有发热和急性期反应物升高,在给予秋水仙碱后缓解的这种情况,是一种迄今未被描述的临床表现。本文的目的是报告一名具有这种独特异常临床表现的FMF患者。