Skin disease is more recalcitrant than muscle disease: A long-term prospective study of 184 children with juvenile dermatomyositis - 10/02/21

Doi : 10.1016/j.jaad.2020.12.032

Andi Wang, BA ^a, Gabrielle A. Morgan, MA ^b, Amy S. Paller, MD ^c, Lauren M. Pachman, MD ^a,^b,^⁎
^a Division of Pediatric Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois
^b Cure JM Center of Excellence in Juvenile Dermatomyositis Care and Research, Stanley Manne Children's Research Institute; and The Ann and Robert H. Lurie Children's Hospital of Chicago Research Center, Cure JM Myositis Center, Chicago, Illinois
^c Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois

^∗Correspondence to: Lauren M. Pachman, MD, Division of Pediatric Rheumatology, The Ann and Robert H. Lurie Children's Hospital of Chicago, 225 E Chicago Ave, Chicago, IL 60611.Division of Pediatric RheumatologyThe Ann and Robert H. Lurie Children's Hospital of Chicago225 E Chicago AveChicagoIL60611

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Abstract

Background

Persistent skin manifestations, especially calcinoses, contribute to morbidity in children with juvenile dermatomyositis.

Objective

To compare the course of skin and muscle involvement and document frequency of calcinosis in juvenile dermatomyositis.

Methods

Prospective cohort study of 184 untreated children with juvenile dermatomyositis (July 1971 to May 2019) at a single children's hospital.

Results

Disease Activity Scores (DASs) were persistently higher for skin versus muscle at all points; clinical inactivity (DAS ≤2) occurred earlier for muscle than skin. Among vascular features for DAS for skin, eyelid margin capillary dilatation was most frequent (54.3%) and persisted longest. Intravenous methylprednisolone reduced DAS for skin more than oral prednisone at 12 months (P = .04). Overall, 16.8% of patients (n = 31) had calcifications, with 4.9% at enrollment. Despite therapy, 25.0% of calcifications recurred and 22.6% failed to resolve; of the latter, 71.4% (n = 5) were present at enrollment. Children with persistent calcifications had longer duration of untreated disease than those whose calcifications resolved (mean 12.5 months) (P < .001). Hydroxychloroquine did not improve DAS for skin (P = .89).

Limitations

DAS does not quantify nailfold capillary dropout.

Conclusions

In juvenile dermatomyositis, skin disease presents with greater activity and is more recalcitrant to therapies than muscle disease. Early and aggressive treatment can limit the severity and persistence of calcifications identified later in the disease course.

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Key words : calcinosis, IV methylprednisolone, juvenile dermatomyositis, oral prednisone, pediatric dermatology, vasculitis

Abbreviations used : DAS, DAS-M, DAS-S, SD

Plan

Introduction

Methods

Patient selection and study design

Outcome measures and treatments

Statistical analysis

Results

Discussion

Strengths and limitations

Conclusions

	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.