Health-related quality of life in children with PFAPA syndrome

Abstract

Nonetheless, fever attacks have been shown to have a substantial impact on daily activities, schooling, and family functioning [10]. [...]the health-related quality of life (HRQOL) is a prominent issue, even though this has not yet been studied in PFAPA patients [11]. Table 1 Parent proxy-reported and child self-reported PedsQL™ scores of pediatric patients with PFAPA and FMF controls Scale PFAPA patients FMF controls P Number Mean score Number Mean score TOTAL 33 62.7 27 76.4 <  0.01 Pre-school age children (2–7 years) Self-reported Total score 15 66.6 8 80.3 0.01 Physical health summary score 15 66.6 8 81.2 0.02 Psychosocial health summary score 15 65.6 8 80.4 0.02 Proxi-reported Total score 23 68.9 14 76.9 0.12 Physical health summary score 23 69.4 14 77 0.12 Psychosocial health summary score 23 68.5 14 75.5 0.27 School age children and youth(8–18 years) Self-reported Total score 8 58 13 74 0.06 Physical health summary score 8 62.5 13 72.7 0.6 Psychosocial health summary score 8 57.2 13 75.5 0.03 Proxi-reported Total score 10 57.4 13 72.3 0.08 Physical health summary score 10 58.8 13 71.8 0.07 Psychosocial health summary score 10 54.8 13 72.8 0.18 The scores for the general quality of life and physical and psychosocial functioning were similar between pre-school and school age children with PFAPA in the self-reported subscales (p,= 0.23, p = 0.65, and p = 0.28, respectively). Table 2 Parent proxy-reported and child self-reported fatigue scores of pediatric patients with PFAPA and FMF controls Scale PFAPA patients FMF controls P Number Mean score Number Mean score Total 33 66.5 27 80.3 < 0.01 Pre-school age children (2/5–7 years) Self-reported Total fatigue score 15 60.4 8 78.3 0.01 General fatigue 15 70 8 84.4 0.02 Sleep/rest fatigue 73 50.5 0.02 Cognitive fatigue 81.4 61.1 0.01 Proxy-reported Total Fatigue score 23 69.3 14 74.9 0.27 General fatigue 23 65.8 14 73.3 0.2 Sleep/rest fatigue 23 65.9 14 74.5 0.25 Cognitive fatigue 23 76.3 14 80.8 0.33 School age children and youth (8–18 years) Self-reported Total Fatigue score 8 62 13 77.5 0.04 General fatigue 8 61.9 13 77.8 0.06 Sleep/rest fatigue 8 66.4 13 76.5 0.09 Cognitive fatigue 8 57.8 13 77.8 0.07 Proxy-reported Total Fatigue score 10 64.1 13 76.6 0.13 General fatigue 10 61.5 13 73.1 0.16 Sleep/rest fatigue 10 66.6 13 74.3 0.09 Cognitive fatigue 10 64.6 13 82.1 0.07 Significantly lower scores were reported in all sub-domains in preschool-age children, whereas the parent proxy-report did not find a significant difference for either age group, although the scores were systematically lower for the PFAPA group (Table 2). Additionally, Press et al. reported that the HRQOL of parents living with a child with FMF was also less than that of parents of healthy children [11]. [...]we compared the quality of life of children with PFAPA to that of patients with the only other autoinflammatory disease evaluated in the literature, FMF.