Lung transplantation in scleroderma: recent advances and lessons

Area of Science:

• Pulmonology
• Immunology
• Transplantation Medicine

Background:

• Systemic sclerosis (SSc) poses unique challenges for lung transplantation due to its multisystemic nature.
• Historical data indicated increased complication risks, particularly with gastrointestinal, cardiac, and renal involvement.
• Emerging evidence suggests safety in carefully selected SSc patients with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH).

Purpose of the Study:

• To review current data on the feasibility and outcomes of lung transplantation in SSc-related pulmonary disease.
• To highlight areas requiring further investigation in this complex patient group.
• To describe a center's approach to pre- and post-transplant management.

Main Methods:

• Literature review of recent data on lung transplantation in SSc patients.
• Analysis of outcomes in SSc patients compared to other lung disease cohorts.
• Description of a specialized center's evaluation and management protocols.

Main Results:

• Scleroderma diagnosis is not an absolute contraindication for lung transplantation.
• Selected SSc patients demonstrate comparable short- and long-term outcomes to patients with fibrotic lung disease or PAH.
• Cardiac disease is an increasingly recognized cause of morbidity and mortality in SSc patients post-transplant.

Conclusions:

• Lung transplantation is a viable option for carefully selected SSc patients with pulmonary manifestations.
• Further prospective studies are needed to refine pre- and post-transplant evaluation and management strategies.
• Particular attention to cardiac status is essential throughout the peri-transplant period.