AB1363-HPR THE INVESTIGATION OF THE QUALITY OF LIFE AND FUNCTIONAL ABILITIES IN PATIENTS WITH JUVENILE SCLERODERMA
Authors: Arzu Dag, Ela Tarakci, Amra Adrovic, Ozgur Kasapcopur
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. There are two main disease forms: juvenile localized scleroderma (JLS) and juvenile systemic sclerosis (JSS). These conditions share common pathophysiologic features which are mainly characterized by inflammation and fibrosis of the skin. In JLS, fibrosis involves restricted areas of the skin, whereas in JSS it also affects the internal organs. <h3>Objectives</h3> There have been few studies of quality of life in pediatric scleroderma and these focused predominantly on self-perception and the influence of skin involvement. Our cross-sectional study aimed to describe the influence of juvenile scleroderma on functional ability and quality of life in relation to clinical and demographic measures. <h3>Methods</h3> 30 patients (26 girls, 4 boys) with JS between the ages of 6-18 years and 30 healthy controls (20 girls, 10 boys) with similar age and gender were included in our study. Patients with either localized scleroderma or systemic sclerosis (SSc) attending pediatric rheumatology clinics, together with their parents were asked to complete a set of 3 validated measures. Children completed their functional abilitiy status with Childhood Health Assessment Questionnaire (CHAQ) and Jebson Taylor Hand Function Test (JTHFT). The quality of life were evaluated with Scleroderma Health Assessment Questionnaire (SHAQ). Clinical and demographic data were provided by consultant pediatric rheumatologists and physiotherapists. <h3>Results</h3> The mean age of the JS group was 14.06 3.24 years (86% female, 14% male; while the%53 localized scleroderma,%47 SSc) and of the control group was 12.43 3.24 years. There were significantly differences between the two groups in functional ability scores (JTHFT), (CHAQ) and quality of life scores (SHAQ) (p&lt;0,05). In JS grup, the total-CHAQ score was 0.470,63 (range 03, 0 indicating no impairment), the JTHFT-Total left hand and right hand scores were 44,059,83 and 41,497,42 second respectively, and the median SHAQ-Total score was 0,34 (range 06, 0 indicating no impairment) <h3>Conclusion</h3> Functional ability disabilities in JS patients cause limitations in daily living activity. Scleroderma had only a moderate effect on quality of life and functional abilites as measured by the 3 validated instruments. Although a small number of children reported greater impairment, this is an encouraging finding, given its potential disfiguring and debilitating effects. <h3>References</h3> [1] Lam C, Young N, Marwaha J, McLimont M, Feldman BM. Revised versions of the Childhood Health Assessment Questionnaire (CHAQ) are more sensitive and suffer less from a ceiling effect. Arthritis Care Res. 2004; 51(6):881-9. [2] Reedman SE, Beagley S, Sakzewski L, Boyd RN. The Jebsen Taylor Test of Hand Function: A Pilot Test-Retest Reliability Study in Typically Developing Children. Phys Occup Ther Pediatr. 2016; 36(3):292-304. [3] Rocha, L. F., Marangoni, R. G., Sampaio-Barros, P. D., Levy-Neto, M., Yoshinari, N. H., Bonfa, E.,. .. &amp; Kowalski, S. C. Cross-cultural adaptation and validation of the Brazilian version of the Scleroderma Health Assessment Questionnaire (SHAQ). Clinical rheumatology. 2014; 33(5):699-706. *The co-author Ela Tarakcı was supported to participate to EULAR 2019 by Turkish Academy of Sciences as a winner of the Young Scientists Award Programme (GEBIP) 2018. <h3>Disclosure of Interests</h3> None declared