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JAK inhibitors in refractory dermatomyositis: A case series

L Corbella-Bagot1, X Bosch-Amate1, E Gimeno-Ribes1

  • 1Department of Dermatology, Hospital Clínic de Barcelona, University of Barcelona, Barcelona, Catalunya, Spain.

The Australasian Journal of Dermatology
|June 18, 2024
PubMed

Insights

Janus kinase (JAK) inhibitors, tofacitinib and baricitinib, show promise for refractory dermatomyositis (DM). These JAK inhibitors effectively reduced skin and muscle symptoms with manageable side effects in a small patient cohort.

Area of Science:

  • Rheumatology
  • Immunology
  • Dermatology

Background:

  • Dermatomyositis (DM) is a multisystemic autoimmune disorder with limited treatment options.
  • Refractory DM presents significant challenges in patient management.
  • Janus kinase (JAK) inhibitors are emerging as potential therapies for autoimmune conditions.

Purpose of the Study:

  • To evaluate the efficacy and safety of JAK inhibitors (tofacitinib, baricitinib) in patients with refractory dermatomyositis.
  • To assess the impact of JAK inhibitors on both cutaneous and muscular manifestations of DM.

Main Methods:

  • Retrospective cohort study design.
  • Inclusion of 14 patients diagnosed with refractory dermatomyositis.
  • Assessment of treatment response using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) and muscular symptom evaluation.

Main Results:

  • Significant reduction in CDASI scores, with a median decrease of 21 points and a 76% overall reduction.
  • Complete resolution of muscular symptoms observed in 64% of treated patients.
  • JAK inhibitors demonstrated effectiveness across various subtypes of refractory DM.

Conclusions:

  • Tofacitinib and baricitinib are effective in managing refractory dermatomyositis.
  • JAK inhibitors offer a viable therapeutic option for patients with limited treatment alternatives.
  • Adverse events associated with JAK inhibitors were generally mild and manageable.
Keywords:
JAK inhibitorsbaricitinibdermatomyositisjuvenilerefractorytofacitinib

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