Advanced Pulmonary Sarcoidosis

In sarcoidosis, pulmonary manifestations are almost universal; however, their severity ranges from asymptomatic to respiratory failure and death. Approximately 20% of patients progress to advanced pulmonary sarcoidosis (APS), a disease phenotype that is driven mostly by pulmonary fibrosis and associated complications, including bronchiectasis, chronic pulmonary aspergillosis, and pulmonary hypertension, which may result from multiple mechanisms. APS may be burnt out but is often active as confirmed by 18F-fluorodeoxyglucose-PET. APS is a major cause of morbidity and mortality in patients with sarcoidosis, but its natural history is largely unknown and evidence-based treatment guidelines are lacking. Here, we review the major patterns of APS with emphasis on clinical manifestations, pathophysiology, and management, although this is based on expert opinion and may include, based on the predominant disease phenotype, anti-inflammatory/immunosuppressive treatment, antifibrotics, pulmonary rehabilitation, antibiotics, vasodilators, and, in highly selected patients, lung transplantation.