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Rev Med Interne. 2018 Apr;39(4):256-264. doi: 10.1016/j.revmed.2018.02.002. Epub 2018 Mar 7.

[Tumor necrosis receptor associated periodic syndrome (TRAPS): State of the art].

[Article in French]

Author information

1
Service de médecine interne, hôpital Tenon, 4, rue de la Chine, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 75020 Paris, France; Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, (CEREMAIA), France; INSERM UMRS_933, hôpital Trousseau, université Pierre-et-Marie-Curie (UPMC)-Paris 6, Assistance publique-Hôpitaux de Paris, 75012 Paris, France.
2
Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, (CEREMAIA), France; Service de rhumatologie pédiatrique, université de Paris Sud, CHU de Bicêtre, Assistance publique-Hôpitaux de Paris, 94270 le Kremlin-Bicêtre, France.
3
Service de médecine interne, hôpital Tenon, 4, rue de la Chine, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 75020 Paris, France; Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, (CEREMAIA), France.
4
Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, (CEREMAIA), France; Laboratoire de génétique, CHU de Montpellier, 191, avenue du Doyen-Gaston-Giraud, 34090 Montpellier, France.
5
Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, (CEREMAIA), France; Service de pédiatrie générale, centre hospitalier de Versailles, 179, rue de Versailles, 78150 Le Chesnay, France. Electronic address: vhentgen@ch-versailles.fr.

Abstract

Tumour necrosis receptor associated periodic syndrome (TRAPS) is a rare cosmopolitan dominant autosomal disease that belongs to the group of recurrent autoinflammatory syndromes. TRAPS is characterized by recurrent bouts of fever lasting more than 7 days, with arthralgia, myalgia, abdominal pain, erythematous rash and sometimes ocular symptoms. During flares, raised inflammatory markers are constant. The age of onset may occur during childhood but also during adulthood. TRAPS is caused by mutations in the TNF receptor 1 (TNFRSF1A) gene that may occur in most of the populations over the world. In the majority of patients, history shows affected relatives, even if sporadic cases do exist. Management of TRAPS usually involves corticosteroid therapy during inflammatory flares. The most severe cases require a treatment with biological agents (mainly interleukin 1 inhibitors). The prognosis of TRAPS is overall good; the main risk is represented by the development of secondary inflammatory amyloidosis. This risk is greatest in patients with structural mutations leading to conformation abnormalities of the TNFRSF1A receptor. Regular clinical and biological monitoring is essential in the follow-up of TRAPS patients.

KEYWORDS:

Autoinflammatory syndrome; Inflammasome; Interleukin1; Interleukine 1; Syndrome auto-inflammatoire; TNFRFS1A; TNFRSF1A; TRAPS; TRAPS syndrome

PMID:
29525418
DOI:
10.1016/j.revmed.2018.02.002
[Indexed for MEDLINE]

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