Outcomes of lung transplantation for scleroderma versus other indications: Insigts from a single center

TL;DR

Lung transplants for scleroderma patients have similar survival rates to other conditions, despite more post-surgery complications.

Contribution

The study provides new evidence that lung transplantation for scleroderma has comparable survival outcomes to other indications.

Findings

Scleroderma patients had higher pulmonary pressure and esophageal issues but similar survival rates.

They experienced more post-transplant complications like delayed chest closure and longer ICU stays.

Adjusted analysis showed no significant difference in chronic lung dysfunction or survival between groups.

Abstract

Scleroderma is an autoimmune disease affecting the skin and internal organs, with pulmonary disease being the leading cause of mortality. Lung transplantation is a potential therapy, but its indication has been limited by concerns about complications, such as esophageal dysmotility.

A retrospective analysis was performed on 959 lung transplant patients from 2011 to 2023, including 77 with scleroderma-related lung disease. Survival rates, post-transplant complications, such as chronic lung allograft dysfunction, and acute cellular rejection rates.

Scleroderma patients had higher mean pulmonary arterial pressure (32 vs. 24 mmHg, p < 0.001) and increased esophageal dysmotility (85% vs. 27%, p < 0.001). Double lung transplantation was more common (99% vs. 87%, p = 0.003). Scleroderma patients experienced higher rates of delayed chest closure (44% vs. 25%, p < 0.001), severe primary graft dysfunction at 72 hours (30% vs. 17%, p = 0.006), and longer mechanical ventilation (median 7 vs. 4 days, p = 0.002). They also required more gastrojejunostomy tubes (79% vs. 20%, p < 0.001) and had longer ICU stays (median 12 vs. 8 days, p = 0.007). However, adjusted competing risks regression showed no significant association between scleroderma and chronic lung allograft dysfunction (HR 0.69 [0.33 – 1.46], p = 0.31) or survival (HR 0.90 [0.56 – 1.45], p = 0.68).

Our findings suggest that lung transplantation might be an important therapeutic option for patients with scleroderma, showing outcomes similar to those of patients with different underlying conditions.

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