Sarcoidosis is a multisystem inflammatory disease characterized by noncaseating granulomas of unknown etiology [1,2]. Up to 90 % of patients with sarcoidosis have pulmonary involvement [2,3]. About two-thirds of patients enter remission over years but the remaining may experience chronic disease with significant impairment in lung function [4]. Advanced pulmonary sarcoidosis (APS) is a subset of pulmonary sarcoidosis that includes advanced pulmonary fibrosis, complications of bronchiectasis, and pulmonary hypertension, leading to respiratory failure and/or death [5,6]. APS occurs in approximately 10–20 % of patients with pulmonary sarcoidosis [[7], [8], [9]]. Common radiographic findings include fibrocystic lesions in the upper and perihilar/peribronchial regions, bronchial distortions, and paracicatricial emphysema (Fig. 1) [10,11]. Although overall mortality in sarcoidosis is believed to be 1–5 %, patients with APS have significantly higher mortality of 11–21 % [5,[12], [13], [14]].

Treatment of APS is based on expert opinion due to limited evidence-based data. Management needs to be personalized based on the complication(s) of pulmonary sarcoidosis in individual patients and may include the use of anti-inflammatory medications, antifibrotics, pulmonary vasodilators, anti-microbial agents, and supportive care [6]. However, lung transplantation remains the last resort to improve quality of life in patients with APS.

Lung transplantation is an evolving field worldwide, aimed to provide maximum benefit for all potential recipients. The International Society for Heart and Lung Transplantation (ISHLT) has reported that nearly 70,000 patients underwent lung transplantation since its inception in 1998–2018, of which greater than 55 % was performed in North America [15]. Since its inception, 31.8 % of all adult lung transplant procedures listed by diagnosis comprised of ILD (26.1 % idiopathic interstitial pneumonia (IIP) and 5.7 % non-IIP ILD [16]. Yet, sarcoidosis only comprised of 2.4 % of all lung transplantation and 7.6 % of ILD (combined IIP and non-IIP) [16]. Currently, there are no specific transplantation guidelines for sarcoidosis. In this review, we will discuss lung transplantation in sarcoidosis with specific reference to APS when applicable.