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A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma

  • Review Article
  • Published:
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Abstract

Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

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Abbreviations

ALT:

Alanine aminotransferase

ANA:

Anti-nuclear antibody

AST:

Aspartate aminotransferase

CARRA:

Childhood Arthritis and Rheumatology Research Alliance

CMAS:

Childhood Myositis Assessment Scale

CPK:

Creatinine phosphokinase

CRP:

C-reactive protein

DLCO:

Diffusion capacity for carbon monoxide

DMARDS:

Disease modifying anti-rheumatic drugs

dsDNA:

Double-stranded DNA

ECG:

Electrocardiogram

EMG:

Electromyography

ENA:

Extractable nuclear antigens

ESR:

Erythrocyte sedimentation rate

HRCT:

High resolution computerised tomography

IIM:

Idiopathic inflammatory myopathy

ILD:

Interstitial lung disease

IMACS:

International Myositis Assessment & Clinical Studies Group

IVIG:

Intravenous immunoglobulin

JDM:

Juvenile dermatomyositis

JLS:

Juvenile localised scleroderma

JSSc:

Juvenile systemic scleroderma

LDH:

Lactate dehydrogenase

LoSCAT:

Localised scleroderma cutaneous assessment tool

MCTD:

Mixed connective tissue disease

MMF:

Mycophenolate mofetil

MMT:

Manual muscle testing

MRI:

Magnetic resonance imaging

MSA:

Myositis specific antibodies

MTX:

Methotrexate

PFTS:

Pulmonary function tests

RCT:

Randomised controlled trial

RF:

Rheumatoid factor

SLE:

Systemic lupus erythematosus

STIR:

Short T1 inversion recovery

UK:

United Kingdom

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Contributions

Both authors contributed equally to the writing of this review and approved the final draft. LJM will act as guarantor for this paper.

Conflict of Interest

None.

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Authors and Affiliations

  1. Department of Rheumatology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK

    Liza J. McCann & Clare E. Pain

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  1. Liza J. McCann
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  2. Clare E. Pain
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Correspondence to Clare E. Pain.

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McCann, L.J., Pain, C.E. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma. Indian J Pediatr 83, 163–171 (2016). https://doi.org/10.1007/s12098-015-1907-z

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  • DOI: https://doi.org/10.1007/s12098-015-1907-z

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