Alsarah Ahmad, Alsara Osama, Laird-Fick Heather S
Department of Internal Medicine, Michigan State University, East Lansing, MI, USA.
Department of Cardiovascular Diseases, University of Florida, Gainesville, FL, USA.
Avicenna J Med. 2017 Oct-Dec;7(4):158-163. doi: 10.4103/ajm.AJM_78_17.
Familial Mediterranean fever (FMF) is autoinflammatory disorder characterized by sporadic attacks of fever, peritonitis, pleuritis, and arthritis. It is mainly seen in patients from Mediterranean origins, but it is now reported more frequently in Europe and North America due to immigration. To analyze the data on the cardiovascular manifestations in FMF patients, we searched PubMed using the terms "Familial Mediterranean Fever" or "FMF" in combination with other key words including "cardiovascular diseases" "pericardial diseases" "atherosclerosis" "coronary artery diseases" "cardiomyopathy" "pulmonary hypertension" or "valvular diseases." suggested several mechanisms to explain the cardiac involvements in FMF including the ongoing inflammation and the amyloid deposits in the heart and vessels' walls at the advanced stages of FMF. The course of these manifestations varies widely, but it can associate with poor prognosis in some cases such as with pulmonary hypertension. Interestingly, Colchicine, which is the cornerstone therapy of FMF, plays a vital role in treating and preventing some of these disorders. In this article, In this article, we will discuss the incidence, pathophysiology, and prognosis of the various cardiac manifestations affecting FMF patients.
家族性地中海热(FMF)是一种自身炎症性疾病,其特征为发热、腹膜炎、胸膜炎和关节炎的散发性发作。该病主要见于地中海地区血统的患者,但由于移民原因,现在在欧洲和北美报道得更为频繁。为了分析FMF患者心血管表现的数据,我们在PubMed上使用了“家族性地中海热”或“FMF”等术语,并结合其他关键词进行搜索,这些关键词包括“心血管疾病”“心包疾病”“动脉粥样硬化”“冠状动脉疾病”“心肌病”“肺动脉高压”或“瓣膜疾病”。有人提出了几种机制来解释FMF中的心脏受累情况,包括持续的炎症以及FMF晚期心脏和血管壁中的淀粉样蛋白沉积。这些表现的病程差异很大,但在某些情况下,如肺动脉高压,可能与预后不良有关。有趣的是,秋水仙碱作为FMF的基石疗法,在治疗和预防其中一些疾病方面发挥着至关重要的作用。在本文中,我们将讨论影响FMF患者的各种心脏表现的发病率、病理生理学和预后。