Lung Transplants in Scleroderma Patients As Safe, Effective As in Other Lung Disease Patients, Study Shows

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by Diogo Pinto |

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scleroderma, DLCO

Patients with scleroderma who undergo lung transplants have similar survival rates to patients who get lung transplants due to other diseases, according to a new study.

Freedom from chronic lung allograft dysfunction — a set of pathological conditions that cause a transplanted lung to stop breathing — is also similar between the two groups of patients.

Findings were reported in the study, “Lung transplantation for scleroderma lung disease: an international, multicenter, observational cohort study,” published in the Journal of Heart and Lung Transplantation.

Scleroderma is a rare autoimmune disease affecting the skin and several internal organs. Due to its multisystemic nature, lung transplants are rarely performed in these patients. However, similar post-transplant outcomes have been reported in recent studies between scleroderma patients and patients with other causes of interstitial lung disease (ILD) — a group of diseases affecting the interstitium, the tissue and space around the air sacs of the lungs.

Pulmonary arterial hypertension (PAH) is another major complication associated with scleroderma. It is not clear if scleroderma-associated PAH influences the outcomes of a lung transplant.

In this multicenter, retrospective, observational study, researchers analyzed the different phases of lung transplants in patients with scleroderma, namely patient selection, rate of survival, and identification of prognostic factors.

The study included 90 patients with scleroderma, all of whom had lung or heart-lung transplants between 1993 and 2016 at 14 European transplant centers. PAH diagnosis was made through right heart catheterization, based on international guidelines.

Survival rates in scleroderma patients were 81% one year after a lung transplant, 68% after three years, and 61% after five years. These survival rates were similar to those reported after lung transplants in patients with other diseases.

The frequency of major post-transplant complications in scleroderma patients, such as primary graft dysfunction (an acute lung injury that occurs within the first 72 hours after lung transplant) and chronic lung allograft dysfunction, was not higher than that reported for other lung diseases requiring transplants.

Researchers found that scleroderma patients who were female and those with PAH were associated with lower survival rates. The risk of death in female scleroderma patients with PAH was three-fold higher than in males without PAH.

“In conclusion, our study adds valuable evidence that scleroderma per se should not be considered a contraindication to lung transplant candidacy. Female gender and PAH were associated with worse survival but not to the extent that these factors should constitute contraindications,” the researchers wrote.

“Further research is necessary to identify patient characteristics and modifiable risk factors allowing both standardized patient selection for lung transplantation and improvements in post-transplant outcomes,” the team said.