Progressive Pulmonary Fibrosis in SSc-ILD

An expert review in Arthritis & Rheumatology on how to define, predict and treat progressive pulmonary fibrosis (PPF) in systemic sclerosis-associated ILD. It compares the competing definitions of progression (and why they give prevalence estimates from about 14% to 49%), summarises prognosis, screening and monitoring advice, risk factors and biomarkers, and walks through treatment: mycophenolate first, then rituximab, tocilizumab, nintedanib and the newly approved nerandomilast, with combination therapy increasingly used on thin comparative evidence. The authors stress that predicting who will progress is still unsolved and that the PPF label works best as a trigger to escalate treatment.

November 8, 2026 · 16 min