Mortality similar after single- vs double-lung transplant in autoimmune disease with ILD
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WASHINGTON — Patients with systemic autoimmune rheumatic disease who develop interstitial lung disease demonstrate similar 1-year mortality risks following either single- or double-lung transplant, according to data.
The finding, presented at ACR Convergence 2024, suggests that, for patients with late-stage lung disease from ILD and systemic autoimmune rheumatic diseases (SARDs), single-lung transplants are a reasonable strategy to effectively increase the number of lungs available for transplantation, said the lead author.
“In other forms of ILD — we’ll take idiopathic pulmonary fibrosis (IPF) as an example — similar 1-year mortality is demonstrated,” Harry Hurley, MD, of the Albert Einstein College of Medicine, told attendees. “However, there was another study showing longer overall survival in IPF patients who receive double lung transplant (DLT) vs. single lung transplant (SLT). So, our question naturally is, is there a benefit to DLT in SARD ILD patients?”
To compare the outcomes of single-lung vs. double-lung transplants among patients with SARD-ILD, Hurley and colleagues conducted a retrospective cohort study using data from the United Network for Organ Sharing. The study comprised procedures that occurred between May 4, 2005, and Dec. 31, 2019.
The analysis included 606 adults (mean age, 54 years; 59% women) with SARD-ILD, 37% of whom had systemic sclerosis. Stratified Cox models — adjusted for characteristics of the donor, recipient and procedure — were used to compare 1-year mortality rates between the two procedures. Some missing covariate data were filled in through multiple imputation.
Overall, 76% of the cohort underwent double-lung transplant. According to the researchers, the unadjusted mortality rates over 1 year were 17.24 per 100 person-years for single-lung transplant and 14.97 for double-lung transplant.
Additionally, single-lung transplant was not associated with significantly increased 1-year mortality vs. double-lung transplant in either the unadjusted model (HR = 1.19; 95% CI, 0.75-1.19) or the fully adjusted model (HR = 1.49; 95% CI, 0.34-6.57).
Mortality risk between the two groups over 1 year “was comparable,” Hurley said.
“This study is the first of its kind to evaluate the effect of single- and double-lung transplant patients in SARD-ILD,” he added. “We were able to control for a large number of possible confounders, and as a result, we believe this is an accurate reflection of modern-day transplant outcomes in SARD-ILD. In the future, we need to prospectively study adult SARD-ILD patients during the transplant eval process to identify modifiable risk factors and thus improve outcomes.”
Perspective
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David A. McLain, MD, MACR, FACP, FRCP
Lung transplantation is typically considered for patients who have advanced interstitial lung disease associated with connective tissue disease (CTD-ILD) that does not respond to other treatments, indicating end-stage lung disease.
The criteria for transplantation acceptance highlight increased risk factors such as CTD itself, esophageal dysmotility and gastroesophageal reflux disease. These factors, along with the use of corticosteroids and other immunosuppressants, are associated with adverse post-lung transplant outcomes.
Some transplant specialists believe that extrapulmonary flares of CTD can influence patient prognosis, though there are limited studies to substantiate this association. Research indicates that systemic sclerosis, a subtype of CTD, may lead to higher rates of acute rejection due to its association with gastrointestinal dysfunction. However, many of these gastrointestinal issues can now be effectively managed.
Polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD), particularly the acute type, poses challenges during the post-transplantation period, including complications in immunosuppression management and rehabilitation. The 3-year survival rate for acute PM/DM-ILD (27%) is significantly lower than that of the chronic type (79%). Moreover, a diagnosis of acute or subacute PM/DM-ILD, or clinically amyopathic dermatomyositis, is linked to a more than fourfold increase in mortality risk compared with polymyositis alone.
Some lung transplant centers still regard CTD-ILD as a relative contraindication for transplantation. However, numerous studies have shown that outcomes for patients with CTD-ILD are comparable to those with idiopathic pulmonary fibrosis (IPF).
Most prior research examining survival in patients with CTD — such as those with SSc — or IPF who underwent lung transplantation has demonstrated no significant difference in cumulative survival between the two groups. Similarly, studies comparing various forms of CTD to IPF have generally shown similar rates of survival, acute and chronic rejection, and extrapulmonary organ dysfunction.
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Hurley H. Abstract #1694. Presented at: ACR Convergence 2024; Nov. 14-19, 2024; Washington, D.C.
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