Living-Donor Lobar Lung Transplantation May Be The Answer to Scleroderma-Related Complications

In a study entitled “A Case Report of Living-donor Lobar Lung Transplantation for Scleroderma-associated Usual Interstitial Pneumonia: Eight Years and Counting” researchers suggest living-donor lobar lung transplantation as a viable alternative for critically ill patients with scleroderma-associated usual interstitial pneumonia. The study was published in the journal Transplantation Proceedings.

Scleroderma, also known as systemic sclerosis, is an autoimmune disorder characterized by skin thickening, a process known as fibrosis. This process, however, is not confined to the skin and in severe cases can reach internal organs such as the kidneys, heart, intestine and lungs. In the lungs, a critical life-limiting complication of scleroderma arises called interstitial lung disease — a group of diseases of inflamed or scarred lungs that will eventually require lung transplantation. These pulmonary complications are usually successfully treated with deceased-donor lung transplantation. Notably, an alternative for critically ill patients that are at risk of dying while in a waiting list for cadaveric donors is living-donor lobar lung transplantation (LDLLT) — a procedure where two donors (usually friends of family members) donate a lobe to the recipient.

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Here, the authors present a case study of a 47-year-old woman with scleroderma-associated interstitial pneumonia who submitted to LDLLT after waiting on a list for deceased-donor lung transplantation for three years. The patients’ health was declining while waiting for the lung transplant (a total of nine years), with several complications arising, including mild sclerodactyly, periungual erythema, Raynaud’s phenomenon, and gastroesophageal reflux, with positive antinuclear autoantibodies. The high Panel Reactive Antibody score together with the patient’s low stature were obstacles to the deceased-donor lung transplantation, since a high score suggests a higher risk for organ rejection. The patient underwent LDLLT and the authors report that after 8 years post-transplantation the patient has benefitted, with an absence of significant esophageal compromise.

The authors highlight that living-donor lobar lung transplantation is an alternative for patients with scleroderma-associated interstitial pneumonia and esophageal pathology, two features that usually exclude them from lung transplantation. The authors also emphasize the longevity of the allograft and how future reports on patients’ outcomes after LDLLT are critical in identifying groups of patients where the procedure can actually make a difference in patients’ survival.