Pediatric rheumatology

Advances in JDM: biomarker and MRI sensitivity, outcomes and steroid management in a Dutch national prospective cohort

Rheumatology (Oxford)Published Prospective cohort study

Authors' conclusion

This study demonstrates the superior diagnostic sensitivity of Galectin-9 and CXCL10 over CK, the high sensitivity of MRI and the frequent occurrence of refractory disease and flares despite treatment.

Study context

Population
JDM patients diagnosed 2007–2024 in the study-coordinating centre and 2015–2024 in five other Dutch tertiary referral hospitals65% female
Participants
83 patients
Outcome
Characteristics and outcomes of the Dutch JDM population
Methodological detailsnational, multicentre
  • National
  • Multicentre

Main finding

As reported in the abstract

Characteristics and outcomes
Median age at diagnosis (IQR 4-9), years
6.0
Median follow-up (IQR 1.4-5.8), years
3.2
Proximal muscle weakness at diagnosis
94.9%
Gottron's papules/sign at diagnosis
79.7%
CK abnormal
73.1%
Galectin-9 elevated at diagnosis
98.3%
CXCL10 elevated at diagnosis
93.3%
Muscle oedema on whole-body MRI (30/33)
90.9%
Corticosteroids tapered to 0.2 mg/kg at 6 months
41.5%
≥1 flare (27 patients)
32.5%
Refractory disease course (24 patients)
28.9%
Calcinosis (12 patients)
14.5%
Clinically inactive disease without medication at last follow-up
28.9%
Deaths
0

Observational data: associations, not effects.

Original publication

Advances in JDM: biomarker and MRI sensitivity, outcomes and steroid management in a Dutch national prospective cohort. Rheumatology (Oxford). Published 18 June 2025. doi:10.1093/rheumatology/keaf335

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