Journal
JOURNAL OF PEDIATRICS
Volume 157, Issue 2, Pages 310-U171Publisher
MOSBY-ELSEVIER
DOI: 10.1016/j.jpeds.2010.02.040
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Funding
- Italian Telethon [GGP09127]
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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-termIL-1 blockade produces a significant and persistent improvement in the clinical manifestations associated with the disease and on the overall quality of life. (J Pediatr 2010;157:310-5).
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