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Review
. 2018 Apr:195:16-27.
doi: 10.1016/j.jpeds.2017.12.053.

Advances in Juvenile Dermatomyositis: Myositis Specific Antibodies Aid in Understanding Disease Heterogeneity

Lauren M Pachman  1 , Amer M Khojah  2
Affiliations
Review

Advances in Juvenile Dermatomyositis: Myositis Specific Antibodies Aid in Understanding Disease Heterogeneity

Lauren M Pachman et al. J Pediatr. 2018 Apr.
No abstract available

Keywords: pathophysiology; physical signs; treatment.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1. Clinical features, common and uncommon, of children with juvenile dermatomyositis
A. Heliotrope rash on the face, characteristic of active JDM B. A very small patch of persistent erythema on cheek in a child as the only rash of JDM positive for MDA-5 C. Erythema of the pinna of the ear in a child as the only sign indicating a flare of JDM positive for p155/140 D. Inflammation in the shawl area on the upper anterior chest indicating both acute and chronic changes E. Linear erythema over the metacarpal-phalangeal (MCP) as well as the proximal (PIP) and distal intercarpal phalangeal (DIP) joints of the hands. Dilated nailfold end row capillary loops are visible. F. Microvasculopathy dilated capillaries on the upper eyelid and eyelid margin in active JDM G. Healing telangiectasia of the eyelid of a child with JDM H. Dilated capillaries close to the edge of the eyelid, resolving; healed medial canthus infarct I. Closely spaced normal nailfold capillary end row loops, showing unit of measure J. Moderate nailfold capillary dropout with vessel tortuosity K. Severe nailfold capillary dropout, with dilated loops and fewer end row loops/mm
Figure 2
Figure 2. Phenotypes associated with the 3 most common myositis specific antibodies (MSAs) in children with myositis: anti-p155/140 (A), anti-MJ (B) and anti-MDA-5 (C)
A) Anti-p155/140, present in 18–30% of idiopathic juvenile inflammatory myopathies display an extensive photosensitive rash which ulcerates, a chronic disease course and generalized lipodystrophy. B) 15–23% of children positive for anti-MJ (NXP2 in the UK) may have disease onset at a younger age, have dysphonia, muscle cramps, atrophy and contractures, with increased weakness, and they are more likely to develop calcifications and gastrointestinal symptoms; their rash often spares the truncal area. C) Anti-MDA-5 is increased in the Japanese population (33%) vs the UK (6%) and is associated with inflammatory lung disease, oral and cutaneous ulcers, arthritis and a milder form of muscle involvement. Adapted with permission from Rider et al (42).
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References

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    1. Pachman LM, Abbott K, Sinacore JM, Amoruso L, Dyer A, Lipton R, et al. Duration of illness is an important variable for untreated children with juvenile dermatomyositis. J Pediatr. 2006;148:247–53. - PubMed
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