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Updated: Sep 13, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Rheumatologists' Adherence to EULAR Recommendations for Systemic Sclerosis Treatment: Experience of a Single Center
Slavica Pavlov-Dolijanovic1, Ivan Jeremic2, Milan Bogojevic3
1Faculty of Medicine, Institute of Rheumatology, University of Belgrade, 11000 Belgrade, Serbia.
Insights
Rheumatologists
Area of Science:
- Rheumatology
- Clinical Pharmacology
Background:
- First systemic sclerosis (SSc) treatment guidelines by EULAR/EUSTAR in 2009, updated in 2016 and 2023.
- Assessing rheumatologists' treatment approach evolution post-2016 SSc guideline update.
Purpose of the Study:
- Evaluate changes in SSc treatment strategies after the 2016 EULAR/EUSTAR guidelines.
- Analyze medication use patterns in SSc patients before and after the guideline update.
Main Methods:
- Retrospective analysis of medication use in 378 SSc patients.
- Stratification of patients based on enrollment before (233) and after (145) the 2016 guideline update.
- Comparison of medication prescribing patterns between the two cohorts.
Main Results:
- Low uptake of recommended treatments for Raynaud's phenomenon (RP) and digital ulcers.
- Increased use of phosphodiesterase type 5 (PDE-5) inhibitors and cyclophosphamide post-guideline update for PAH and ILD respectively.
- Consistent use of proton pump inhibitors (PPIs) and prokinetic agents for gastrointestinal involvement, with increased utilization after 2016.
Conclusions:
- Variable rheumatologist adherence to EULAR/EUSTAR SSc guidelines (25%-100%).
- Improved concordance in managing pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and gastrointestinal issues post-2016 update.
- Need for enhanced guideline implementation and monitoring in SSc care.
Abstract:
Background: The European League Against Rheumatism (EULAR), in collaboration with the European Scleroderma Trial and Research group (EUSTAR), published the first set of treatment recommendations for systemic sclerosis (SSc) in 2009, with subsequent updates in 2016 and 2023. Objectives: This study aimed to evaluate how rheumatologists' clinical approaches to the treatment of SSc evolved following the 2016 update of the clinical management guidelines. Methods: Medication use for SSc was analyzed in a cohort of 378 patients. The patients were stratified based on enrollment before (233 patients) and after (145 patients) the guideline update, and medication usage was compared between the two groups. Results: Although all patients presented with Raynaud's phenomenon (RP), only 35% received calcium channel blockers. Medications such as iloprost, phosphodiesterase type 5 (PDE-5) inhibitors, fluoxetine, and bosentan, recommended for the treatment of RP and digital ulcers, were not approved for SSc by the Republic Health Insurance Fund. Treatment for pulmonary arterial hypertension (PAH) was administered to only 16 patients (4.2%), including 2 who received bosentan, 10 who received PDE-5 inhibitors, and 4 who were treated with riociguat. The use of PDE-5 inhibitors increased following the 2016 update of the guidelines. Cyclophosphamide was consistently prescribed for interstitial lung disease (ILD), with an increased frequency observed after the guideline update. No significant differences were observed in the use of methotrexate for skin involvement, ACE inhibitors for scleroderma renal crisis, or antibiotics for gastrointestinal symptoms. Proton pump inhibitors (PPIs) were prescribed to 87.3% of patients with gastrointestinal involvement, with an increase in use of both PPIs and prokinetic agents following the guideline update. Conclusions: Rheumatologists' adherence to the EULAR/EUSTAR guidelines varied considerably, with 25% to 100% of eligible patients receiving the recommended treatments. Concordance improved in the management of PAH, ILD, and gastrointestinal involvement after the 2016 guideline update.
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