Efficacy and safety of upadacitinib in refractory interstitial lung disease with idiopathic inflammatory myopathies: a retrospective study
Authors' conclusion
UPA significantly improved lung function, reduced glucocorticoid dependence and suppressed systemic inflammation in refractory IIM-ILD with acceptable safety
Study context
- Population
- Patients with refractory IIM-ILD following conventional therapy failure, single centre16 upadacitinib (UPA) with complete paired data
- Participants
- 28 vs 32
- Exposure
- Upadacitinib vs conventional treatment
- UPAn = 28
- Controlsn = 32reference
- Outcome
- Lung function (UPA, baseline vs after treatment)
- Follow-up
- Mean 7.4 months
Methodological detailssingle-centre, propensity score matching (1:2)
- Single-centre
- Propensity score matching (1:2)
Main finding
As reported in the abstract
- Mean FVC increased from 1.84 to 2.13 l
- P = 0.0069
- FVC% increased from 63.5% to 76.77%
- P = 0.0004
Observational data: associations, not effects.
Further findings
Post-treatment IL-6 (PSM analysis)
P = 0.0012
GC dose (PSM analysis)
P = 0.017
- Post-treatment IL-6 (propensity score matching (PSM) analysis): n = 16 UPA vs 32 controls
Inflammatory indicators (UPA, baseline vs after treatment)
- IL-6 decreased 14.06-8.44 pg/ml
- P = 0.039
- Serum ferritin decreased 240.30-188.15 µg/l
- P = 0.018
Pulmonary function tests (PFTs) and high-resolution CT (HRCT) scores (PSM analysis)
- No significant differences between the groups
Safety
- Infections (among the 28 UPA-treated patients)
- 6/28 (21.4%)
- All recovered well
Original publication
Efficacy and safety of upadacitinib in refractory interstitial lung disease with idiopathic inflammatory myopathies: a retrospective study. Rheumatology (Oxford). Published 10 April 2026. doi:10.
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