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Rheumatol Int. 2018 Jan;38(1):37-50. doi: 10.1007/s00296-017-3854-7. Epub 2017 Nov 1.

Cardiovascular disease in patients with autoinflammatory syndromes.

Author information

1
Academic Research Unit, 2nd Department of Internal Medicine, Kepler University Hospital, Med Campus III, Krankenhausstr. 9, 4020, Linz, Austria. rainer.hintenberger@kepleruniklinikum.at.
2
Academic Research Unit, 2nd Department of Internal Medicine, Kepler University Hospital, Med Campus III, Krankenhausstr. 9, 4020, Linz, Austria.
3
Paracelsus Private Medical University Salzburg, M├╝llner Hauptstra├če 48, 5020, Salzburg, Austria.

Abstract

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.

KEYWORDS:

Autoinflammatory syndrome; Cardiovascular disease; Cardiovascular risk; Periodic fever syndrome; Thrombosis

PMID:
29094181
DOI:
10.1007/s00296-017-3854-7
[Indexed for MEDLINE]

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