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

Donato Rigante, Adele Compagnone, Giuseppe Lopalco, Luca Cantarini

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

17 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)1503-1511
Numero di pagine9
RivistaCLINICAL RHEUMATOLOGY
Volume2015
DOI
Stato di pubblicazionePubblicato - 2015

Keywords

  • Familial Mediterranean fever

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