Inflammatory myopathies

Janus kinase inhibition (Janus kinase inhibitors (JAKi)) therapy in refractory anti-synthetase syndrome: A retrospective cohort study

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

JAKi were effective in most refractory ASS patients as evidenced by improved skin rash, myositis, and ILD.

Study context

Population
Refractory anti-synthetase syndrome patients treated with JAKi, October 2020 to June 2023; all had interstitial lung disease (ILD)all with ILD
Participants
20 patients
Exposure
Before vs after JAKi treatment
Outcome
Efficacy (significant improvement in clinical manifestations) and safety of JAKi
Methodological detailsreal-world clinical settings, single-group before–after comparison
  • Real-world clinical settings
  • Single-group before–after comparison

Main finding

As reported in the abstract

Significant improvement in clinical manifestations

Patients with significant improvement in clinical manifestations

14

70 %

Observational data: associations, not effects.

Further findings

Arthritis

P = 0.002

Rash

P = 0.012

Shortness of breath

P = 0.039

Cough

P = 0.012

CK levels (5 patients abnormally elevated prior treatment)

P = 0.043

Myalgia
P = 0.016
Muscular weakness
P = 0.012
Erythrocyte sedimentation rate (ESR)
P = 0.001
C-reactive protein (CRP)
P = 0.023
CT score in ASS-ILD
P = 0.001
GC dose
P = 0.001

Myalgia

Improvement was noted for myalgia (50 % vs. 11.1 %)

Muscular weakness

Improvement was noted for muscular weakness (61.1 % vs. 11.1 %)

ESR

Decrease in ESR

CRP

Decrease in CRP

CT score in ASS-ILD

CT score reduced (188.75 ± 69.67 vs. 156.35 ± 74.62)

GC dose

GC dose significantly reduced (21.42 ± 13.26 mg vs. 11.32 ± 8.59 mg)

Original publication

Janus kinase inhibition (JAKi) therapy in refractory anti-synthetase syndrome: A retrospective cohort study. Semin Arthritis Rheum. Published 3 June 2024. doi:10.1016/j.semarthrit.2024.152474

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