Expert Perspectives: Defining and Managing Progressive Pulmonary Fibrosis in Systemic Sclerosis
7 affiliations
- 1Department of Clinical and Molecular Sciences Marche Polytechnic University & Clinica Medica, Marche University Hospital Ancona Italy
- 2University of Michigan Scleroderma Program Ann Arbor Michigan
- 3Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) IRCCS San Raffaele Hospital Vita‐Salute San Raffaele University Milan Italy
- 4Université Claude Bernard Lyon 1, Hospices Civils de Lyon, Centre de référence des maladies pulmonaires rares, Service de pneumologie, ERN‐LUNG, INRAE, UMR754 F‐69000 Lyon France
- 5School of Medicine and Surgery, University of Milano Bicocca, SC Pneumologia, Fondazione IRCCS “San Gerardo dei Tintori,” Monza Italy
- 6Inflammation Fibrosis and Ageing Initiative (INFLAGE), Division of Genetics and Cell Biology IRCCS San Raffaele Scientific Institute Milan Italy
- 7Rheumatology Department Cochin Hospital, Université Paris Cité Paris France
Abstract
Systemic sclerosis–associated interstitial lung disease (SSc‐ILD) is one of the leading causes of morbidity and mortality in SSc, affecting up to three‐quarters of patients. The disease course is highly heterogeneous, ranging from indolent, nonprogressive forms to rapidly progressive pulmonary fibrosis (PPF). Identifying patients at risk for progression remains a key clinical challenge. Routine screening with high‐resolution computed tomography at diagnosis and regular pulmonary function test monitoring are essential for early detection and timely management. Recent efforts have sought to harmonize the definition of PPF across fibrosing ILDs, recognizing shared clinical and pathophysiological mechanisms beyond idiopathic pulmonary fibrosis. Approximately 20% to 30% of patients with SSc‐ILD develop PPF, yet reliable predictors of progression remain elusive. Advances in imaging, functional assessment, and molecular biomarkers hold promise for improving risk stratification. Therapeutic approaches have evolved from conventional immunosuppression toward combination and antifibrotic strategies. Mycophenolate mofetil remains the preferred first‐line therapy, whereas nintedanib is the only antifibrotic agent approved for both SSc‐ILD and PPF, having demonstrated efficacy in slowing lung function decline. Combination therapy, particularly mycophenolate mofetil with nintedanib or biologics such as rituximab and tocilizumab, is gaining traction in clinical practice despite limited comparative data. Emerging antifibrotic and anti‐inflammatory agents offer new therapeutic prospects. This review summarizes current understanding of PPF in SSc‐ILD, focusing on its evolving definition, prognostic determinants, and treatment landscape, and highlights unmet needs for personalized, evidence‐based management.
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BibTeX
@article{benfaremo2026expert,
title = {{Expert Perspectives: Defining and Managing Progressive Pulmonary Fibrosis in Systemic Sclerosis}},
author = {Devis Benfaremo and Dinesh Khanna and Corrado Campochiaro and Vincent Cottin and Fabrizio Luppi and Marco Matucci‐Cerinic and Gianluca Moroncini and Yannick Allanore},
journal = {Arthritis \& Rheumatology (Hoboken, N.j.)},
year = {2026},
month = jun,
doi = {10.1002/art.70237},
url = {https://doi.org/10.1002/art.70237},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Expert Perspectives: Defining and Managing Progressive Pulmonary Fibrosis in Systemic Sclerosis
AU - Devis Benfaremo
AU - Dinesh Khanna
AU - Corrado Campochiaro
AU - Vincent Cottin
AU - Fabrizio Luppi
AU - Marco Matucci‐Cerinic
AU - Gianluca Moroncini
AU - Yannick Allanore
PY - 2026
DA - 2026/06/20
JO - Arthritis & Rheumatology (Hoboken, N.j.)
DO - 10.1002/art.70237
UR - https://doi.org/10.1002/art.70237
AB - Systemic sclerosis–associated interstitial lung disease (SSc‐ILD) is one of the leading causes of morbidity and mortality in SSc, affecting up to three‐quarters of patients. The disease course is highly heterogeneous, ranging from indolent, nonprogressive forms to rapidly progressive pulmonary fibrosis (PPF). Identifying patients at risk for progression remains a key clinical challenge. Routine screening with high‐resolution computed tomography at diagnosis and regular pulmonary function test monitoring are essential for early detection and timely management. Recent efforts have sought to harmonize the definition of PPF across fibrosing ILDs, recognizing shared clinical and pathophysiological mechanisms beyond idiopathic pulmonary fibrosis. Approximately 20% to 30% of patients with SSc‐ILD develop PPF, yet reliable predictors of progression remain elusive. Advances in imaging, functional assessment, and molecular biomarkers hold promise for improving risk stratification. Therapeutic approaches have evolved from conventional immunosuppression toward combination and antifibrotic strategies. Mycophenolate mofetil remains the preferred first‐line therapy, whereas nintedanib is the only antifibrotic agent approved for both SSc‐ILD and PPF, having demonstrated efficacy in slowing lung function decline. Combination therapy, particularly mycophenolate mofetil with nintedanib or biologics such as rituximab and tocilizumab, is gaining traction in clinical practice despite limited comparative data. Emerging antifibrotic and anti‐inflammatory agents offer new therapeutic prospects. This review summarizes current understanding of PPF in SSc‐ILD, focusing on its evolving definition, prognostic determinants, and treatment landscape, and highlights unmet needs for personalized, evidence‐based management.
SN - 2326-5191
SN - 2326-5205
ER -