Academic Research Unit, 2nd Department of Internal Medicine, Kepler University Hospital, Med Campus III, Krankenhausstr. 9, 4020, Linz, Austria.
Paracelsus Private Medical University Salzburg, Müllner Hauptstraße 48, 5020, Salzburg, Austria.
Rheumatol Int. 2018 Jan;38(1):37-50. doi: 10.1007/s00296-017-3854-7. Epub 2017 Nov 1.
Autoinflammatory syndromes (AIS) are characterized by recurring events of inflammation, leading to a variety of organ manifestations and fever attacks. A subgroup of AIS is commonly referred to as hereditary periodic fever syndromes (HPFS). There is substantial evidence that autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus are strongly associated with cardiovascular morbidity and mortality. The link between AIS and cardiovascular disease is not that clear, even if the concept of continuous inflammation as a risk factor for cardiovascular disease is widely accepted. Research on the association of AIS and cardiovascular disease is increasing within the last years. In this review, we will discuss the association of several AIS with cardiovascular disease. Based on the rarity of some entities, lack of data, however, led to exclusion of some rare AIS. Especially, for Behcet's disease (BD), adult-onset Still's disease (AOSD), and Familial Mediterranean fever (FMF), there is an association with a number of cardiovascular abnormalities. BD is the AIS, which is most strongly associated with manifestation in the arterial and venous system. AOSD is strongly associated with cardiac inflammation (peri-/myocarditis). FMF patients are likely to suffer from serositis. Of note, there seems to be a link between variants of AOSD as well as FMF and idiopathic recurrent acute pericarditis.
自身炎症性疾病（AIS）的特征是反复发作的炎症，导致多种器官表现和发热发作。AIS 的一个亚组通常被称为遗传性周期性发热综合征（HPFS）。有大量证据表明，类风湿关节炎和系统性红斑狼疮等自身免疫性疾病与心血管发病率和死亡率密切相关。尽管连续炎症作为心血管疾病的一个危险因素的概念被广泛接受，但 AIS 与心血管疾病之间的联系并不那么明确。
近年来，人们对 AIS 与心血管疾病之间的关联进行了越来越多的研究。在这篇综述中，我们将讨论几种 AIS 与心血管疾病的关联。基于一些疾病的罕见性以及数据的缺乏，我们排除了一些罕见的 AIS。特别是，对于贝赫切特病（BD）、成人Still 病（AOSD）和家族性地中海热（FMF），它们与许多心血管异常有关。BD 是与动脉和静脉系统表现最密切相关的 AIS。AOSD 与心脏炎症（心包炎/心肌炎）密切相关。FMF 患者可能患有胸膜炎。值得注意的是，AOSD 和 FMF 的变异体以及特发性复发性急性心包炎之间似乎存在联系。
