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Joint Bone Spine
Volume 74, n° 6
pages 544-550 (décembre 2007)
Doi : 10.1016/j.jbspin.2007.07.005
Received : 23 July 2007 ;  accepted : 25 July 2007
Auto inflammatory syndromes: Diagnosis and treatment
 

Katia Stankovic , Gilles Grateau
Internal Medicine Department, Reference Center for Inflammatory Amyloidosis and Familial Mediterranean Fever, Tenon Teaching Hospital, Assistance publique-hôpitaux de Paris, Paris 6 University, 4 rue de la Chine, 75970 Paris Cedex 20, France 

Corresponding author. Tel.: +33 1 56 01 70 80; Fax: +33 1 56 01 70 82.
Abstract

Hereditary recurrent fevers are rare genetic diseases characterized by apparently spontaneous attacks of inflammation. They include familial Mediterranean fever (FMF); tumor necrosis factor (TNF) receptor periodic syndrome (TRAPS); hyperimmunoglobulinemia D syndrome (HIDS); and hereditary periodic fevers related to mutations in the CIAS1 (cold induced autoinflammatory syndrome 1) gene, such as Muckle-Wells syndrome, familial cold urticaria, and CINCA/NOMID (chronic infantile neurological cutaneous and articular/neonatal-onset multisystemic inflammatory disease). Musculoskeletal manifestations are common. They may occur as features of the acute inflammatory attacks or persist for longer periods. Among them, the most common include arthritis of the large and medium-sized joints in FMF and CINCA, arthralgia in HIDS, and myalgia or pseudo-fasciitis in TRAPS. The outcome is usually favorable, although joint destruction may develop in CINCA or at the hip in FMF. The recurrent bouts of fever and accompanying clinical manifestations suggest the diagnosis, which can be confirmed by genetic testing. Among differential diagnoses, infection should be considered routinely. The treatment of the inflammatory attacks is nonspecific. New pathophysiological insights have led to the development of promising maintenance treatments designed to reduce the number and severity of the inflammatory attacks and to diminish the risk of secondary amyloidosis.

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Keywords : Hereditary recurrent fevers, Musculoskeletal manifestations




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