Pediatric rheumatology

Long-term follow-up of Janus-kinase inhibitor and novel active disease biomarker in juvenile dermatomyositis

Rheumatology (Oxford)Published Retrospective cohort study and cross-sectional study

Authors' conclusion

The preliminary evidence suggested that JAKi is a relatively safe and effective alternative for JDM patients. Cytokine profiles could well reflect the inflammatory status of JDM patients.

Study context

Population
JDM patients, Affiliated Children's Hospital of Capital Institute of Pediatrics (China), November 2017–December 2021
Participants
101 / 128 / 30
Exposure
Janus Kinase Inhibitor (JAKi) vs Cytokine JDM vs Controls
  • JAKin = 101
  • Cytokine JDMn = 128
  • Controlsn = 30reference
Outcome
Outcomes on JAKi
Follow-up
Median 19 months
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

Improved rashes

65.5%

Eliminated GC

39.6%

Observational data: associations, not effects.

Further findings

Cytokines in active JDM

Cytokines significantly elevated in active JDM
12/34
IL-1RA over 793 times the mean of normal values
793
Active JDM with only rashes had lower cytokine levels than active JDM with multiple phenotypes
Anti-NXP2-positive active patients had lower cytokine levels than anti-NXP2-negative

Six-cytokine panel differentiating active vs stable status

AUC
0.8486
P
P < 0.05

Safety

Serious adverse events

No serious adverse events reported

Potential side effects of JAKi

Abnormal leukopoenia
14/95
Cough
16/83
  • Affected over 10% of the JDM patients

Original publication

Long-term follow-up of Janus-kinase inhibitor and novel active disease biomarker in juvenile dermatomyositis. Rheumatology (Oxford). Published 29 July 2022. doi:10.1093/rheumatology/keac399

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