Background/Purpose: In Juvenile Dermatomyositis (JDM), dystrophic calcifications, associated with increased morbidity and mortality, have been reported for 20-30% of patients. There are few laboratory indicators of disease activity, and a duration of untreated disease (DUD) greater than 4.5 months is associated with normalization of muscle enzymes (CK, Aldolase, LDH, SGOT), commonly used to guide response to therapy. Objective: To determine the current frequency of dystrophic calcifications in children with JDM.

Methods: Patients with definite/probable JDM (overlap syndromes excluded) seen between 2000 and 2010 at the Ann and Robert H. Lurie Children’s Hospital enrolled (IRB #2012-12858).  At the first visit of 90 children, 52 were untreated and 38 were previously treated.  Demographic data were obtained (Table), and their clinical inflammation assessed by disease activity scores (DAS) for skin and muscle.  Diagnosis was confirmed by laboratory testing, and MRI directed muscle biopsy.

	Untreated (N=52)	Treated (N=38)	P-value
White/Hispanic (N, %)	46 (88%)	36 (95%)	0.51
Female (N, %)	40 (77%)	28 (74%)	0.91
Age at onset (year,mean±SD)	5.8±3.17	6.2±3.4	0.52
Disease duration (month,mean±SD)	10.4±16.2	9.6±13.4	0.79
DAS skin (mean±SD)	5.8±1.3	4.9±2.3	0.055
DAS muscle (mean±SD)	4.0±2.6	4.1±2.8	0.91

Results: Of the 52 untreated patients, 5 (9.6%) had calcinosis at presentation. The 52 children were initially treated with oral prednisone (47), IV prednisone (45), methotrexate (48), hydroxychloroquine (11), cyclosporin (1), mycophenolate (2), IVIG (0), and alendronate (0).  During their disease course, the following medications were used: oral prednisone (50), IV methlyprednisone (45), methotrexate (48), hydroxychloroquine (21), cyclosporin (10), mycophenolate (29), IVIG (6), and alendronate (0).  At follow-up 4/5 of the children’s calcifications had resolved and the fifth one had diminished in size. None of the children with initially untreated JDM developed new calcifications. Of the 38 children previously treated, 7 (18%) had calcifications.  Medications taken: oral prednisone (25), IV methylprednisone (5), methotrexate (25), hydroxychloroquine (20), cyclosporin (3), mycophenolate (2), IVIG (5), and alendronate (2).  At follow-up, of the 7 children with calcifications:  2 improved in size (1 totally resolved), 3 remained the same, one developed more advanced calcification (from moderate to severe), and one developed 36 deposits months later.

Conclusion: In untreated JDM without calcifications, no deposits developed during a mean follow-up period of <4 years. Of the 5 untreated JDM with calcifications, 4/5 resolved and the one diminished. Of the seven treated JDM referred with calcifications, only one totally resolved, and one developed new calcifications after 36 months of therapy. These data suggest that early immunosuppressive therapy may impair the development of dystrophic calcifications in JDM, for only 7.0% of this total group had calcifications. None of the 52 initially untreated children without calcifications developed them at a later time.  We speculate that dystrophic calcifications in JDM are preventable.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/decreased-frequency-of-dystrophic-calcifications-in-children-with-juvenile-dermatomyositis-a-10-year-study/