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

Loredana Lepore, Giulia Paloni, Roberta Caorsi, Maria Alessio, Donato Rigante, Nicola Ruperto, Marco Cattalini, Alberto Tommasini, Francesco Zulian, Alessando Ventura, Alberto Martini, Marco Gattorno

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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.

Original languageEnglish
JournalJournal of Pediatrics
Volume157
Issue number2
DOIs
Publication statusPublished - 2010

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)

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