• B Acad Nat Med Paris · Jun 2005

    Review

    [Hereditary recurrent fever syndromes].

    • Gilles Grateau.
    • Médecine Interne, Hôpital Tenon, 4, rue de la Chine, 75970 PARIS Cedex 20.
    • B Acad Nat Med Paris. 2005 Jun 1; 189 (6): 1235-46; discussion 1246-8.

    AbstractFour diseases presenting mainly as intermittent bouts of inflammatory symptoms have been clinically and genetically characterized. At the head of this group is familial Mediterranean fever, which affects thousands of patients of Mediterranean ancestry. The other three entities are the tumor necrosis factor receptor superfamily 1A-associated periodic fever syndrome (TRAPS) with a dominant mode of inheritance; hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); and the most recently recognized entity, which includes Muckle Wells syndrome, familial cold urticaria, and the chronic infantile neurological cutaneous and articular (CINCA) syndrome. Proper diagnosis of these entities is needed to begin specific clinical and therapeutic management.

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