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

Donato Rigante, Loredana Lepore, Giulia Paloni, Roberta Caorsi, Maria Alessio, Nicola Ruperto, Marco Cattalini, Alberto Tommasini, Francesco Zulian, Alessando 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
RivistaTHE JOURNAL OF PEDIATRICS
Volume2010
Stato di pubblicazionePubblicato - 2010

Keywords

  • ANAKINRA
  • CRYOPYRINOPATHY
  • QUALITY OF LIFE

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