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

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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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  1. Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy

    Donato Rigante, Giusyda Tarantino, Adele Compagnone & Michele Fastiggi

  2. Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy

    Giuseppe Lopalco

  3. Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100, Siena, Italy

    Luca Cantarini

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  1. Donato Rigante
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Rigante, D., Lopalco, G., Tarantino, G. et al. Non-canonical manifestations of familial Mediterranean fever: a changing paradigm. Clin Rheumatol 34, 1503–1511 (2015). https://doi.org/10.1007/s10067-015-2916-z

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