Original articleSkin disease is more recalcitrant than muscle disease: A long-term prospective study of 184 children with juvenile dermatomyositis
Introduction
Juvenile dermatomyositis, a rare autoimmune disease, affects 2.3 to 4.1 US children per million per year.1, 2, 3 Defining features are symmetric proximal muscle weakness and a characteristic skin eruption, most often localized to the face and overlying joints.4 Juvenile dermatomyositis may lead to severe sequelae, including dystrophic calcifications, persistent cutaneous inflammation, and organ involvement with functional limitations.5, 6, 7
Validated in 2003,8 the Disease Activity Score (DAS) continues to be a widely used measure for quantifying skin and muscle activity in juvenile dermatomyositis.9, 10, 11, 12, 13 The 20-point scale uses 11 possible points for muscle and 9 points for skin manifestations. Scored skin features include severity of skin erythema and Gottron papules, distribution, and the presence of erythema and telangiectasia at specific locations (eyelid, periungual region, and palate). The DAS monitors disease severity and response to treatment, and has been shown to predict juvenile dermatomyositis functional outcomes, such as cardiac dysfunction with high early Disease Activity Score–Skin (DAS-S) scores.14 The DAS-S has the strongest correlation with the physician's visual analog scale.15
Prompt initiation of appropriate therapy is linked to optimal outcomes and reduction of long-term disability.16, 17, 18 Although skin features often precede muscle complaints, calcinoses are a later complication. Calcinosis is a poorly understood and feared complication of juvenile dermatomyositis, contributing to significant morbidity. Reported to be present in 30% to 50% of juvenile dermatomyositis patients,19, 20, 21 calcinoses often involve the extremities22 and soft tissues, may be related to repeated local trauma, and are associated with longer disease duration before therapy.23,24 Risk of calcification has been linked to delayed or suboptimal treatment5,25 and the TNFA-308 GA or AA genotype.26
Using long-term tracking of DAS-S and Disease Activity Score–Muscle (DAS-M) scores,8,9 this study compared skin and muscle activity, as well as patterns of calcifications, during the juvenile dermatomyositis disease course in 485 children at a single center.
Section snippets
Patient selection and study design
Data from this single-center cohort study were extracted from the Juvenile Myositis Registry, currently a Research Electronic Data Capture database (hosted by Northwestern University Feinberg School of Medicine),27 begun in July 1971 that includes all juvenile dermatomyositis patients treated at The Ann and Robert H. Lurie Children's Hospital of Chicago through May 2019. Clinical and laboratory data were captured for 184 children and adolescents who met inclusion criteria of diagnosis of
Results
The 184 untreated juvenile dermatomyositis patients (Table I) were predominantly female individuals (75%), non-Hispanic White (72.2%), and of the TNFA-308 GG genotype (69%). The mean duration of untreated disease at enrollment was 10.4 months (standard deviation [SD] 17.5 months). At entry, skin involvement was classified as minimal (Gottron papules only, 0.6%), mild (45.2%), moderate (45.2%), or severe (9.0%). Skin distribution was described as focal (59.1%; limited to joint regions), diffuse
Discussion
Given the collection of data for 48 years, this study provides a historical and comprehensive look at the management of juvenile dermatomyositis. The data confirm that juvenile dermatomyositis patients present with greater skin than muscle involvement, and that skin features require more time for resolution.36 This is an important consideration because persistent skin disease not only significantly affects quality of life but also can foster calcification and ulceration that can be life
Strengths and limitations
With data captured during almost 5 decades, this study is the largest cohort study of untreated juvenile dermatomyositis patients from a single center, to our knowledge. Most patients were treated and evaluated by a single physician (L.M.P.), ensuring uniform scoring. However, the DAS has certain limitations. For example, nailfold capillary dropout is associated with more chronic disease and greater disease severity,17,48 but it is not quantified in the DAS. Our group's ongoing investigation of
Conclusions
This cohort study confirms that both skin and muscle involvement should guide diagnosis and treatment of juvenile dermatomyositis. Clinicians should be aware of eyelid margin capillary dilatation, which tends to persist, in addition to other characteristic skin features that are better recognized. Early and aggressive treatment with therapies such as intravenous methylprednisolone can help limit the severity of skin disease.
Conflicts of interest
None disclosed.
References (54)
- et al.
Advances in juvenile dermatomyositis: myositis specific antibodies aid in understanding disease heterogeneity
J Pediatr
(2018) - et al.
Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification
J Pediatr
(1983) - et al.
Juvenile dermatomyositis: latest advances
Best Pract Res Clin Rheumatol
(2017) - et al.
Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial
Lancet
(2016) - et al.
Aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis
J Am Acad Dermatol
(2002) - et al.
Research Electronic Data Capture (REDCap) - a metadata-driven methodology and workflow process for providing translational research informatics support
J Biomed Inform
(2009) - et al.
Classification and treatment of the juvenile idiopathic inflammatory myopathies
Rheum Dis Clin North Am
(1997) - et al.
Clinical status and cardiovascular risk profile of adults with a history of juvenile dermatomyositis
J Pediatr
(2011) - et al.
Duration of illness is an important variable for untreated children with juvenile dermatomyositis
J Pediatr
(2006) - et al.
US incidence of juvenile dermatomyositis, 1995–1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry
Arthritis Care Res
(2003)
Incidence and prevalence of idiopathic inflammatory myopathies in Korea: a nationwide population-based study
J Korean Med Sci
The incidence of juvenile dermatomyositis: results from a nation-wide study
Br J Rheumatol
The presentation, assessment, pathogenesis, and treatment of calcinosis in juvenile dermatomyositis
Curr Rheumatol Rep
Disease Activity Score for children with juvenile dermatomyositis: reliability and validity evidence
Arthritis Care Res
Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis
Rheumatology (Oxford)
Retrospective analysis of infliximab and adalimumab treatment in a large cohort of juvenile dermatomyositis patients
Arthritis Res Ther
Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), Physician Global Damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI)
Arthritis Care Res (Hoboken)
Quality of life in adults with juvenile-onset dermatomyositis: a case-control study
Arthritis Care Res (Hoboken)
In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity
Ann Rheum Dis
Comparison of the utility and validity of three scoring tools to measure skin involvement in patients with juvenile dermatomyositis
Arthritis Care Res (Hoboken)
Juvenile dermatomyositis: new insights and new treatment strategies
Ther Adv Musculoskelet Dis
Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis
Arthritis Rheum
Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features
Arthritis Rheum
Clinical outcomes in juvenile dermatomyositis
Curr Opin Rheumatol
The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the first 5 yr
Rheumatology
Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry
Arthritis Care Res
Patterns of calcification in childhood dermatomyositis
AJR Am J Roentgenol
Cited by (10)
[Translated article] Juvenile Dermatomyositis During the SARS-CoV-2 Pandemic: Acral and Oral Mucosal Involvement
2023, Actas Dermo-SifiliograficasJuvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care
2023, Nature Reviews RheumatologyCessation of Immunomodulatory Medication Use in Dermatomyositis: A Single-Center Cohort Study
2023, Arthritis Care and ResearchJuvenile Dermatomyositis
2023, A Clinician's Pearls and Myths in Rheumatology: Second Edition
Funding sources: Supported by the Vivian Allison and Daniel J. Pachman Fund (Pachman), the Cure JM Foundation (Morgan, Pachman), and R-21 AR077565 from the National Institute of Arthritis, Metabolic and Skin Diseases (Pachman). Research Electronic Data Capture is supported at Feinberg School of Medicine by the Northwestern University Clinical and Translational Science (NUCATS) Institute. NUCATS is funded in part by a Clinical and Translational Science Award grant from the National Institutes of Health UL1TR001422.
The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
IRB approval status: Reviewed and approved by the Lurie Children's Institutional Review Board (IRB 2019-2734).
Reprints not available from the authors.