Scleroderma-Associated Lung Disease Management

Introduction to Management

The American College of Rheumatology recommends that all patients with scleroderma-associated interstitial lung disease (SSc-ILD) be co-managed by both rheumatologists and pulmonologists from the time of ILD diagnosis or treatment initiation 1, 2

Patients with SSc-ILD should be referred to a pulmonologist immediately upon diagnosis or suspicion based on screening tests, need to determine whether treatment is indicated, or initiation of any ILD-directed therapy, as recommended by the American College of Rheumatology 1, 2
The American Thoracic Society suggests that pulmonologists perform high-resolution CT (HRCT) for definitive ILD diagnosis, conduct full pulmonary function tests, and participate in multidisciplinary discussion with rheumatologists, radiologists, and pathologists for accurate diagnosis 1, 2

The American College of Rheumatology recommends referring patients with progressive SSc-ILD despite first-line treatment to a lung transplantation center rather than waiting for further progression 1, 5, 6
The International Society for Heart and Lung Transplantation suggests that specific triggers for transplant referral include need for high-flow oxygen, progressive disease despite optimal medical management, and critical timing considerations such as pre-transplantation evaluation taking considerable time 1, 2

The American College of Rheumatology recommends immediately referring patients with rapidly progressive ILD (RP-ILD) to both pulmonology and considering early lung transplantation evaluation 1, 5, 6
The European Respiratory Society defines RP-ILD as progression from stable disease to respiratory failure within days to weeks 1, 2

The American College of Rheumatology conditionally recommends referring patients with SSc-ILD progression despite first-line treatment to centers with strong SSc-ILD experience and demonstrated positive outcomes for stem cell transplantation (SCT) evaluation 5, 6
The European League Against Rheumatism suggests that SCT should be prioritized only for specialized centers with proven expertise in SSc-ILD to minimize adverse effects and mortality risk 7

The optimal management team includes a rheumatologist, pulmonologist, radiologist, and pathologist, as recommended by the American College of Rheumatology 1, 2

The American College of Rheumatology recommends not delaying pulmonology referral until symptoms become severe and not waiting for disease progression before considering lung transplantation referral in appropriate candidates 1, 2
The American Thoracic Society suggests not referring to SCT centers without documented expertise and positive outcomes in SSc-ILD 7
The European Respiratory Society recommends not managing SSc-ILD with rheumatology alone—co-management is essential for optimal outcomes 1, 2