sarcoidosis and transplant

While in most patients with sarcoidosis, the condition is self-limited or well controlled with anti-inflammatory medical management, a small fraction of patients exhibit progressive disease with fibrosis and complicating pulmonary hypertension (APS) poorly responsive to treatment that warrants assessment for transplantation. Review of large registries of patients with APS have shown that they have higher mortality while awaiting transplantation and are less likely to receive a transplant than patients with other forms of pulmonary fibrosis. It is not clear to what extent this observation indicates imperfect performance of scoring systems used to prioritize organ allocation or to what extent this finding may reflect inequities in health care access related to socioeconomic status or even inherent racial bias. All of these possibilities are under evaluation, and adjustment in organ allocation methodology may show reductions in this apparent disparity in coming years.

It is estimated that 10% to 15% of patients with sarcoidosis present with or proceed to manifest significant pulmonary fibrosis, most often determined radiologically. This justifies a designation of APS. Most of these patients can be managed medically, and United States data from the International Thoracic Organ Transplant Registry indicate that only 2.4% of all lung transplants are performed in patients with a diagnosis of APS. Guidelines for transplantation for APS are similar to those for other forms of interstitial lung disease (ILD), including dyspnea or functional limitation, supplemental oxygen requirement, poor lung function (eg, FVC less than 80%, DLCO less than 40% predicted, sarcoidosis-associated pulmonary hypertension), and failure to improve after a trial of medical management. These guidelines may be reconsidered because patients with APS have a higher mortality while awaiting transplantation and are less likely to undergo transplantation than other patients with ILD.

The majority of patients with APS receiving lung transplantation undergo a double rather than single lung procedure, in large part owing to the significant incidence of pulmonary hypertension. Because of the potential for cardiac involvement in sarcoidosis, cardiac evaluation in these patients is particularly important as part of transplant planning. Although patients with APS have higher primary graft dysfunction, prolonged postoperative need for mechanical ventilation, prolonged length of stay, and higher 30-day mortality than patients with other forms of ILD undergoing transplantation, long-term outcomes and median survival are similar to other groups (choice D is incorrect). Histopathologic recurrence of granulomas in the allograft is frequently seen but often without associated symptoms and without survival implications.1

Footnotes

  1. SEEK Questionnaires ↩