Follow-up and quality of life of patients with cryopyrin-associated periodic syndromes treated with Anakinra.

Donato Rigante, Loredana Lepore, G Paloni, R Caorsi, Maria Alessio, Nicolino Ruperto, M Cattalini, Alberto Tommasini, Francesco Zulian, A Ventura, Alberto Martini, Marco Gattorno

Risultato della ricerca: Contributo in rivistaArticolo in rivista

86 Citazioni (Scopus)

Abstract

OBJECTIVE: To evaluate the quality of life and long-term follow-up of patients enrolled in the Italian registry of cryopyrin-associated periodic syndromes (CAPS). STUDY DESIGN: Since 2004, 20 patients with CAPS were enrolled in a common registry from different Italian Centers of Pediatric Rheumatology; 14 patients were treated with Anakinra in an open fashion. Both treated and untreated patients were routinely followed according to standard of care. The Child Health Questionnaire (CHQ-PF 50) was used to assess the health-related quality of life. RESULTS: The mean duration of follow-up was 37.5 months. In all treated patients, a complete and persistent control of the inflammatory manifestations was observed with no further progression of the disease. At enrollment in the registry, patients showed a poorer health-related quality of life than healthy children in both physical and the psychosocial summary scores. Treatment was associated with a dramatic and sustained amelioration of a variety of measures of poor quality of life, particularly in those concerning the global health perception, bodily pain-discomfort, and other physical domains. CONCLUSIONS: Long-term IL-1 blockade produces a significant and persistent improvement in the clinical manifestations associated with the disease and on the overall quality of life.
Lingua originaleEnglish
pagine (da-a)310-315
Numero di pagine6
RivistaJournal of Pediatrics
Volume2010
Stato di pubblicazionePubblicato - 2010

Keywords

  • ANAKINRA
  • CRYOPYRINOPATHY
  • QUALITY OF LIFE

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