Background/Purpose: Lung transplantation is a life-saving option for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) and interstitial lung disease (SSc-ILD) patients. Yet, there is risk of post-transplantation mortality. The objective of this study was to evaluate survival of SSc patients post-lung transplantation. We secondarily evaluated SSc lung transplant recipient characteristics, and compared post-lung transplantation survival of SSc patients to non-SSc patients (idiopathic PAH, and ILD).

Methods: A systematic review of MEDLINE, EMBASE, Cochrane Central Registry of Controlled Trials and CINAHL (all inception to 2012) was performed to identify studies evaluating post-lung transplant survival in SSc compared to non-SSc patients. Two reviewers independently abstracted study and survival data using a standardized form.

Results:

226 citations were screened to identify 7 observational studies reporting SSc patients who underwent single lung, double lung, or heart-lung transplantation. Mean age at transplantations ranged 46-53 years. SSc post-transplantation survival ranged 69%-91% at 30-days, 69%-85% at 6-months, 59%-93% at 1-year, 49%-80% at 2-years, and 46%-79% at 3-years. ILD post-transplant survival was 80% at 30-days, 80%-90% at 6-months, 59%-83% at 2-years, and 69% at 3-years. IPAH post transplant survival was 79% at 30-days, 79%-90% at 6-months, and 74%-90% at 1-year. The reporting of overlapping cohorts potentially including the same patients precluded meta-analysis. Causes of death in SSc patients, when reported, included graft failure (n=6), infection (n=8), cardiac events (n=3), hemorrhagic stroke (n=1), respiratory failure (n=3), malignancy (n=2), pulmonary hypertension (n=1), complications of bronchiolitis obliterans syndrome (BOS) (n=1), anesthetic complication (n=1), and scleroderma renal crisis (n=1). There were no reports of recurrence of SSc in the lung allograft.

Conclusion:

SSc survival post-lung transplantation is very good, and improving with time. The short-term and intermediate-term survival post-lung transplantation are similar to IPAH and ILD patients requiring lung transplantation. Future researchers should delineate the access process for lung transplantation and report the occurrence of acute rejection, infection, bronchiolitis obliterans syndrome, renal dysfunction and dialysis, gastroparesis, and need for tube feeding.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/survival-after-lung-transplantation-in-systemic-sclerosis-a-systematic-review/