Worldwide evaluation of Clinical Practice Strategies (CliPS) for lung involvement in Still’s disease within the JIR-CliPS network: a COST action
- Dr Maurine Jouret
- Jul 15, 2025
- 1 min read
First author : Dr Maurine Jouret
Rheumatology Advances in Practice, rkaf073, https://doi.org/10.1093/rap/rkaf073
Published: 03 July 2025
Still’s disease, whether systemic juvenile idiopathic arthritis (sJIA) or adult-onset Still’s disease (AOSD), can be complicated by serious lung involvement—which remains under-recognized and highly variable in management worldwide.
🔎 What we did:
✅ Conducted a global survey of 372 clinicians across five continents via the JIR-CliPS network.
✅ Explored diagnostic practices, biomarkers, and therapeutic strategies for lung disease in Still’s disease.
✅ Highlighted significant regional differences and gaps between real-world practice, registry data, and existing guidelines.
Key takeaways:
🫁 Sixty-nine respondents indicated that they manage patients with lung involvement. Chest CT and echocardiography for pulmonary hypertension are widely used, but advanced biomarkers like IL-18 and interferon signatures remain underutilized in many regions.
Genetic testing for HLA-DRB1*15, a potential risk factor for drug-induced reactions is inconsistently performed showing significant regional variation.
🔬 Despite concerns that biologics might have exacerbated lung inflammation most pediatricians continued their use in alignment with EULAR/PReS guidelines. Emerging therapies, including JAK inhibitors, are gaining ground, especially in refractory cases.
🌐 There’s an urgent need to harmonize diagnostic and therapeutic approaches globally to improve patient outcomes.
By Maurine Jouret - France
Based upon work from COST Action CA21168, Project CliPS - Working Group 5"Systemic Juvenile Idiopathic Arthritis (sJIA) and adult onset still disease(AOSD)". Supported by COST (European Cooperation in Science and Technology). www.cost.eu
SHARING is CARING !
By participating in the worldwide CLIPS project, you help to improve patient management and long-term-outcome.
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