Lung transplantation in patients with scleroderma

Systemic sclerosis (SSc) is an autoimmune disorder that involves skin and internal organs. Pulmonary diseases, comprising interstitial lung disease and pulmonary arterial hypertension, are the leading causes of morbidity and mortality in patients with SSc. Lung transplantation in patients with SSc remains controversial owing to a presumed heightened risk in the postoperative period from SSc-related gastroesophageal reflux, renal impairment, and skin fibrosis. In this article, we review the indications and patient selection criteria for lung transplantation in patients with SSc, discuss the implications and recommendations related to single versus bilateral lung transplantation, and review postlung transplantation survival data.

Early aggressive surgical treatment of gastroesophageal reflux disease decreases the rate of bronchiolitis and improves survival in lung transplant patients. Heart transplantations remain rare owing to frequent involvement of other organ systems in SSc patients with cardiac manifestations. The procedure of choice in patients with SSc has not been determined, but given the dearth of donor lungs, single lung transplantation has become commonplace for most SSc patients without severe pulmonary hypertension. Carefully selected patients without extrapulmonary systemic disease experience similar survival after lung transplantation compared to those with other end-stage pulmonary diseases. Patients with SSc undergoing lung transplantation have similar rates of chronic rejection as patients transplanted for nonconnective tissue disease-related interstitial lung disease.

Lung transplantation represents a viable therapeutic option to consider for patients with end-stage lung disease due to SSc who have limited extrapulmonary manifestations.