Who should patients with scleroderma-associated lung disease be referred to for management?

Referral Strategy for Scleroderma-Associated Lung Disease

All patients with scleroderma-associated interstitial lung disease (SSc-ILD) should be co-managed by both rheumatologists and pulmonologists from the time of ILD diagnosis or treatment initiation. 1

Primary Referral: Pulmonology

Refer to a pulmonologist immediately upon:

• Diagnosis of SSc-ILD or suspicion based on screening tests (abnormal spirometry, reduced DLCO, or abnormal chest auscultation) 2, 3
• Need to determine whether treatment is indicated, particularly in asymptomatic patients with stable and mild ILD 1
• Initiation of any ILD-directed therapy 1

The pulmonologist's role includes:

• Performing high-resolution CT (HRCT) for definitive ILD diagnosis 2, 4
• Conducting full pulmonary function tests with DLCO and ambulatory desaturation testing 4
• Participating in multidisciplinary discussion with rheumatologists, radiologists, and pathologists for accurate diagnosis 2
• Monitoring disease progression with PFTs every 3-6 months and annual HRCT 5

Lung Transplantation Referral

For progressive SSc-ILD despite first-line treatment, refer early to a lung transplantation center rather than waiting for further progression. 1

Specific triggers for transplant referral:

• Need for high-flow oxygen (this is a severity marker warranting immediate transfer to a transplantation center) 1
• Progressive disease despite optimal medical management 1
• Advanced disease at initial presentation 3

Critical timing considerations:

• Pre-transplantation evaluation takes considerable time 1
• Early referral is warranted even if the transplantation center is not local to the patient 1
• Candidacy for lung transplantation may influence medical therapy options 1

Rapidly Progressive Disease: Urgent Multidisciplinary Referral

For rapidly progressive ILD (RP-ILD), immediately refer to both pulmonology and consider early lung transplantation evaluation. 1

RP-ILD is defined as progression from stable disease to respiratory failure (requiring high-flow oxygen or mechanical ventilation) within days to weeks 1

Stem Cell Transplantation Referral

For SSc-ILD progression despite first-line treatment, conditionally refer to centers with strong SSc-ILD experience and demonstrated positive outcomes for stem cell transplantation (SCT) evaluation. 1

Important caveats:

• SCT should be prioritized only for specialized centers with proven expertise in SSc-ILD to minimize adverse effects and mortality risk 1
• Do NOT refer for SCT as first-line treatment for RP-ILD 1
• Optimal medical management is preferred over SCT as first-line therapy 1

Multidisciplinary Team Composition

The optimal management team includes:

• Rheumatologist (for systemic disease management and immunosuppressive therapy) 1, 2
• Pulmonologist (for ILD-specific assessment and monitoring) 1, 2
• Radiologist (for HRCT interpretation in multidisciplinary discussion) 2
• Pathologist (when tissue diagnosis is needed) 2

Common Pitfalls to Avoid

• Do not delay pulmonology referral until symptoms become severe—patients may have minimal symptoms with significant lung pathology present 6
• Do not wait for disease progression before considering lung transplantation referral in appropriate candidates 1
• Do not refer to SCT centers without documented expertise and positive outcomes in SSc-ILD 1
• Do not manage SSc-ILD with rheumatology alone—co-management is essential for optimal outcomes 1