Health‐related quality of life of patients with juvenile dermatomyositis: Results from the paediatric rheumatology international trials organisation multinational quality of life cohort study

Abstract

Objective. To investigate the health-related quality of life (HRQOL) change over time, as measured by the Child Health Questionnaire (CHQ), and its determinants in patients with active juvenile dermatomyositis (DM).Methods. We assessed patients with juvenile DM at both baseline and 6 months of followup, and healthy children age <18 years. Potential determinants of poor HRQOL included demographic data, physician's and parent's global assessments, muscle strength, functional ability as measured by the Childhood Health Assessment Questionnaire (C-HAQ), global disease activity assessments, and laboratory markers. Results. A total of 272 children with juvenile DM and 2,288 healthy children were enrolled from 37 countries. The mean ؎ SD CHQ physical and psychosocial summary scores were significantly lower in children with juvenile DM (33.7 ؎ 11.7 versus 54.6 ؎ 4.1) than in healthy children (45.1 ؎ 9.0 versus 52 ؎ 7.2), with physical well-being domains being the most impaired. HRQOL improved over time in responders to treatment and remained unchanged or worsened in nonresponders. Both physical and psychosocial summary scores decreased with increasing levels of disease activity, muscle strength, and parent's evaluation of the child's overall well-being. A C-HAQ score >1.6 (odds ratio [OR] 5.06, 95% confidence interval [95% CI] 2.03-12.59), child's overall well-being score >6.2 (OR 5.24, 95% CI 2.27-12.10), and to a lesser extent muscle strength and alanine aminotransferase level were the strongest determinants of poor physical well-being at baseline. Baseline disability and longer disease duration were the major determinants for poor physical well-being at followup. Conclusion. We found that patients with juvenile DM have a significant impairment in their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment.

2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis

“…Although only the IMACS core set measures were used for adult DM/PM, for further congruence with paediatric core set measures, the experts in adult myositis agreed to include the Short Form-36 33 as a health-related quality-of-life measure to correspond to the PRINTO quality-of-life core set measure, the parent form of the Child Health Questionnaire. [34][35][36] In a post-conference final vote, consensus (74%) was reached on threshold values for minimal, moderate, and major response for adult DM/PM patients, which are ≥20 in the total improvement score for minimal improvement, ≥40 for moderate improvement, and ≥60 for major improvement. In contrast, consensus on the final threshold values for minimal, moderate, and major response for juvenile DM were ≥30, ≥45, and ≥70 points, respectively.…”

The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis

“…The database contains the six PRINTO JDM core set measures 14 assessed longitudinally (0, 6, 12 and 24 months): (1) physician's global assessment of the patient's overall disease activity on a 10 cm visual analogue scale (MD-GLOVAS); 15 (2) muscle strength/endurance on the Childhood Myositis Assessment Scale (CMAS) [16][17][18] or manual muscle testing of eight muscle groups (MMT); 19 (3) global disease activity assessment through the Disease Activity Score (DAS); 20 (4) functional ability through the Childhood Health Assessment Questionnaire (C-HAQ); [21][22][23][24] (5) parent's global assessment of the patient's overall well-being on a 10 cm VAS (Par GLOVAS); 15 21 22 and (6) health-related quality of life using the physical summary score (PhS) of the Child Health Questionnaire (CHQ). 22 25 Additional measures were the Myositis Disease Activity Assessment which combines the Myositis Disease Activity Assessment Visual Analogue Scale (MYOACT) including the physician evaluation of extramuscular activity (MD-ExtraMuscVAS) and muscle activity (MD-MuscVAS) and the Myositis Intention to Treat Activity Index; 26 the psychosocial summary score (PsS) of the CHQ; 22 25 serum muscle enzymes (creatine kinase (CPK), lactate dehydrogenase (LDH), aldolase, aspartate aminotransferase, alanine aminotransferase (ALT)), [27][28][29][30][31] whose results were standardised as previously described; 32 and the Myositis Damage Index.…”

Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatment

“…In terms of health-related quality of life, the Child Health Questionnaire (CHQ) has been examined in a large multi-center cohort by PRINTO, comparing physical and psychosocial components to healthy children [73]. Physical dysfunction scores on the CHQ most related to functional disability, parent’s global assessment of well-being and ALT levels, while psychosocial well-being was most strongly associated with muscle strength and physical dysfunction.…”