Abstract
Familial Mediterranean fever (FMF) is the most common of the hereditary periodic fever syndromes. Although the typical clinical course of FMF is characterized by bouts of painful inflammation, this presentation represents only the tip of the iceberg. In many patients inflammation can persist in attack-free periods, as shown by high levels of acute-phase proteins, cytokines and inflammation-induced proteins. This subclinical inflammation puts patients at risk of developing complications such as anemia, splenomegaly, decreased bone mineral density, heart disease and life-threatening amyloid A amyloidosis, among others. In this article, we review the published data on markers and other factors involved in the persistence of inflammation in patients with FMF during attack-free periods, examine the risk factors for the development of this subclinical inflammation, summarize the complications of chronic inflammation in FMF and propose a new strategy for treatment, based on these data.
Key Points
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The hallmark feature of familial Mediterranean fever (FMF) is acute, painful episodes of inflammation, but chronic subclinical inflammation may persist between attacks
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Chronic subclinical inflammation in patients with FMF might lead to complications, including amyloid A amyloidosis, anemia, splenomegaly and decreased bone density
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In addition to the prevention of acute attacks, treatment of FMF should be aimed at decreasing the chronic subclinical inflammation and impeding its deleterious clinical outcome
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Serum amyloid A protein is a sensitive marker of chronic inflammation in FMF that should be measured and the levels taken into account in treatment decisions
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I. Ben-Zvi and A. Livneh contributed equally to researching the data for the article, discussing the content, writing the article and reviewing and/or editing of the manuscript before submission.
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Ben-Zvi, I., Livneh, A. Chronic inflammation in FMF: markers, risk factors, outcomes and therapy. Nat Rev Rheumatol 7, 105–112 (2011). https://doi.org/10.1038/nrrheum.2010.181
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DOI: https://doi.org/10.1038/nrrheum.2010.181
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