Follow-Up and Quality of Life of Patients with Cryopyrin-Associated Periodic Syndromes Treated with Anakinra

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