Long-term outcomes in Juvenile Myositis patients

Abstract

Objective-Juvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on long-term outcomes is limited. In this study we investigate correlations between clinical and demographic features with long-term outcomes in a referral population of adult patients with JIIM.Methods-Forty-nine adults with JIIM were assessed at two referral centers between 1994 and 2016. Features of active disease and damage at a cross-sectional assessment were obtained. Regression modeling was used to examine factors associated with long-term outcomes, defined by the presence of calcinosis or a higher adjusted Myositis Damage Index (MDI) score. A multivariable model of MDI was constructed using factors that were statistically significant in bivariate models.Results-At a median of 11.5 [IQR 4.5-18.9] years following diagnosis, median American College of Rheumatology (ACR) functional class was 2 [1.5-3.0], Health Assessment Questionnaire (HAQ) score was 0.4 out of 3.0 [0.0-1.0], and manual muscle testing (MMT) score was 229 out of 260 [212.6-256.8]. Median MDI score was 6.0 [3.5-8.9], with the most commonly

Juvenile Dermatomyositis: what comes next? Long-term outcomes in childhood myositis from a patient perspective

“…It is possible that perceived muscle weakness for example may be impacted by several factors including cardiorespiratory fitness, endurance, physical disability, or psychological wellbeing. However, the fact that over one-third of patients reported muscle weakness at the time of the questionnaire is comparable to other studies that demonstrate impaired muscle strength and cardiorespiratory fitness compared to healthy controls many years after the diagnosis of JDM [27,37,38].…”

“…In comparison with 3 other long term outcome studies reporting the HAQ (median 0, IQR 0–0.3 [ 26 ], median 0.4, IQR 0–1.0 [ 27 ], mean 0.2, SD 0.5, range 0–1.9 [ 2 ]), our cohort had better HAQ scores despite reports of persistently active disease.…”

“…In comparison with 3 other long term outcome studies reporting the HAQ (median 0, IQR 0-0.3 [26], median 0.4, IQR 0-1.0 [27], mean 0.2, SD 0.5, range 0-1.9 [2]), our cohort had better HAQ scores despite reports of persistently active disease. This cohort also cohort had reduced rates of employment compared to the UK general population, in the 18-24 year old age group, and many felt that they had experienced career compromise or difficulties studying at school and/or in the tertiary setting as a result of their disease.…”

“…These data suggest that active muscle disease was perceived to be an important long-term outcome from a patient perspective in this cohort. Although not completely comparable due to different study design (cross-sectional retrospective cohort [ 2 , 26 , 27 ]) and data collected, other published studies have also found persisting disease activity and requirement of immunosuppressive medication use in long-term follow-up of childhood myositis patients. This may indicate that there are a higher number of patients with chronically active disease than previously thought, and that we need to develop strategies to identify patients at the onset of disease, who may need more intensive therapy in order to improve these outcomes.…”

Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

“…Strengths of our study include that 95% of all identified and tracked JDM patients participated in the study; our results are thus less biased toward serious cases compared to other outcome studies [ 42 ]. Since our age- and sex-matched controls were randomly drawn from the Norwegian registry, we believe they reflect the general population.…”