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Long-term outcome in patients with juvenile dermatomyositis: A case series
G Chevalier1, O Fakih2, A Lhose3
1Service de Dermatologie, Inserm 1098 RIGHT, Université de Franche Comté, Centre Hospitalier Universitaire, 3, boulevard Fleming, 25030 Besançon, France.
Insights
Juvenile dermatomyositis (JDM) patients show good long-term functional outcomes, with minimal skin and muscle damage after extended follow-up. Most patients maintain normal muscle strength and experience mild disability, indicating a positive prognosis for JDM.
Area of Science:
- Rheumatology
- Pediatric Autoimmunity
- Dermatology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children, with known long-term impacts on multiple organs.
- Limited data exists on the long-term prognosis and outcomes for JDM patients.
Purpose of the Study:
- To assess the long-term outcomes in a cohort of juvenile dermatomyositis patients.
- To evaluate the persistent effects of JDM on skin, muscles, and overall patient well-being.
Main Methods:
- A cohort of JDM patients underwent detailed assessments by dermatologists and rheumatologists.
- Validated tools including aCAT, MMT8, CMAS, MDI, CHAQ, and HAQ were used to evaluate disease activity, muscle strength, damage, and disability.
- Long-term outcomes such as growth, puberty, education, vocation, and comorbidities were recorded.
Main Results:
- After a mean follow-up of 14.9 years, most patients (71%) showed minimal active skin disease (aCAT score) and significant skin damage (MDI).
- The majority of patients (71.4%) exhibited normal muscle strength (MMT8 >72), with no severe muscle weakness reported.
- Disability was mild (CHAQ/HAQ index) in 29% of patients, and quality of life scores were generally favorable.
Conclusions:
- Long-term follow-up using validated cutaneous and musculoskeletal scores indicates favorable functional outcomes for juvenile dermatomyositis patients.
- The study suggests that while some long-term damage may persist, JDM patients generally achieve good functional recovery and quality of life.
Background:
Follow-up of juvenile dermatomyositis (JDM) patients has demonstrated the impact of the disease on several organs in the long term.
Objective:
As there is little information on the long-term outcome of JDM, we aimed to assess long-term outcomes in a series of JDM patients.
Methods:
After selection of JDM patients, a consultation with a dermatologist and a rheumatologist was held for each patient. Cutaneous, muscle, and disease damage was assessed using different validated scores including the abbreviated Cutaneous Assessment Tool (aCAT), 8-muscle Manual Muscle Testing (MMT8), Childhood Myositis Assessment Scale (CMAS), Myositis Damage Index (MDI), Childhood Health Assessment Questionnaire (CHAQ), and Health Assessment Questionnaire (HAQ). Long-term disease outcomes were recorded including growth and pubertal development, educational and vocational achievement, and development of comorbidities.
Results:
Seven patients were included in the study. After a mean follow-up of 14.9±8.8 years, the mean aCAT score was 0.57±1.4 and only one patient had a positive aCAT activity score. The mean aCAT damage score was 1.4±1.3 and five (71%) patients had a score of ≥1. Five (71.4%) patients had normal muscle strength with an MMT8 score of >72, and none had severe muscle weakness (MMT8 ≤32, and CMAS<35). The mean total extent of damage according to the MDI was ≥1 in five (71%) patients and mainly involved the skin. Two (29%) patients had mild disability according to the CHAQ/HAQ disability index. In terms of quality of life, no patient had a score of<40 (1 SD below the mean for healthy controls).
Conclusions:
Based on validated cutaneous and musculoskeletal scores, our study demonstrated the good functional outcomes of JDM at long-term follow-up.
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