Pediatric rheumatology

Treatment outcomes in 63 cases of juvenile dermatomyositis-associated calcinosis

Clin Exp RheumatolPublished Retrospective cohort study

Authors' conclusion

Despite wide variations in treatment, many patients showed calcinosis improvement over time, especially those treated with IVIG.

Study context

Population
Patients with juvenile dermatomyositis (JDM) and calcinosis treated between 2003 and 2019 at 11 CARRA institutions
Participants
63 patients11 institutions
Exposure
Association of treatment outcomes with patient, disease and treatment characteristics (e.g. Intravenous immunoglobulin (IVIG) vs treatments without IVIG)
Outcome
Calcinosis improvement
Methodological detailsmulti-institutional, multivariable Cox models, Kaplan-Meier estimates
  • Multi-institutional
  • Multivariable Cox models
  • Kaplan-Meier estimates

Main finding

As reported in the abstract

Overall improved calcinosis

79%

n=50

Observational data: associations, not effects.

Further findings

Calcinosis improvement by anti-NXP2 or anti-MDA5 autoantibody

Did not significantly affect probability of any calcinosis improvement
P = 0.30

Patient and disease characteristics

Median (IQR) age at JDM diagnosis, years
7.8 (4.1-11.1)
Median (IQR) age at calcinosis diagnosis, years
9.4 (5.7-13.3)
Calcinosis present at JDM diagnosis
32% (n=20)
Active JDM at calcinosis diagnosis
76% (47/62)
Anti-NXP2 (most common myositis autoantibody)
38%, 12/32
Unique treatment regimens
103

Original publication

Treatment outcomes in 63 cases of juvenile dermatomyositis-associated calcinosis. Clin Exp Rheumatol. Published 31 October 2024. doi:10.55563/clinexprheumatol/35pbbq

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