Non-canonical manifestations of familial Mediterranean fever: a changing paradigm

Clinical Rheumatology
Donato RiganteLuca Cantarini

Abstract

Paroxysmal crises of fever and systemic inflammation herald familial Mediterranean fever (FMF), considered as the archetype of all inherited systemic autoinflammatory diseases. Inflammatory bouts are characterized by short-term and self-limited abdominal, thoracic, and/or articular symptoms which subside spontaneously. Erysipelas-like findings, orchitis, and different patterns of myalgia may appear in a minority of patients. In recent years, many non-classical manifestations have been reported in the clinical context of FMF, such as vasculitides and thrombotic manifestations, neurologic and sensory organ abnormalities, gastrointestinal diseases, and even macrophage activation syndrome. As FMF left unrecognized and untreated is ominously complicated by the occurrence of AA-amyloidosis, it is highly desirable that diagnosis of this autoinflammatory disorder with its multiple clinical faces can be contemplated at whatever age and brought forward.

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Citations

Dec 4, 2015·Clinical Rheumatology·Donato RiganteLuca Cantarini
Jan 10, 2017·Expert Review of Clinical Immunology·Donato Rigante
Jun 19, 2017·Clinical Rheumatology·Mansour Alghamdi
Feb 23, 2020·Mediators of Inflammation·Carla GaggianoLuca Cantarini
Apr 20, 2019·International Journal of Molecular Sciences·Alessandra BettiolFlorenzo Iannone
Feb 14, 2017·Frontiers in Immunology·Carlos de Torre-MinguelaPablo Pelegrín

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