An expert overview of pulmonary fibrosis in sarcoidosis

ABSTRACTIntroduction Advanced pulmonary sarcoidosis refers to phenotypes of pulmonary sarcoidosis that often lead to significant loss of lung function, respiratory failure, or death. Around 20% of patients with sarcoidosis may progress to this state which is mainly driven by advanced pulmonary fibrosis. Advanced fibrosis often presents with associated complications of sarcoidosis including infections, bronchiectasis, and pulmonary hypertension.Areas covered This article will focus on the pathogenesis, natural history of disease, diagnosis, and potential treatment options of pulmonary fibrosis in sarcoidosis. In the expert opinion section, we will discuss the prognosis and management of patients with significant disease.Expert opinion While some patients with pulmonary sarcoidosis remain stable or improve with anti-inflammatory therapies, others develop pulmonary fibrosis and further complications. Although advanced pulmonary fibrosis is the leading cause of death in sarcoidosis, there are no evidence-based guidelines for the management of fibrotic sarcoidosis. Current recommendations are based on expert consensus and often include multidisciplinary discussions with experts in sarcoidosis, pulmonary hypertension, and lung transplantation to facilitate care for such complex patients. Current works evaluating treatments include the use of antifibrotic therapies for treatment in advanced pulmonary sarcoidosis.KEYWORDS: Sarcoidosispulmonary fibrosisadvanced pulmonary sarcoidosisrespiratory failurelung transplantation Article highlights 10-20% of patients with sarcoidosis may develop pulmonary fibrosis.Advanced pulmonary sarcoidosis refers to phenotypes of pulmonary sarcoidosis that often lead to significant loss of lung function, respiratory failure, or death. This is driven mainly by advanced pulmonary fibrosis.Morbidity and mortality are high due to complications involving bronchiectasis, infections, and pulmonary hypertension.Recognition of underlying disease process(es) is the key to individualized treatment. A multidisciplinary approach involving experts in pulmonary sarcoidosis, pulmonary hypertension, and lung transplantation is crucial in providing individualized for patients with progressive and severe fibrosis.PFT, HRCT, FDG-PET/CT, and potential serum biomarkers have been used to monitor patients, identify inflammation, and assess response to anti-inflammatory therapies.Management of pulmonary fibrosis is not standardized and relies on expert-opinion. Anti-inflammatory, antifibrotic, and pulmonary vasodilator therapy can be useful on a case-by-case basis but needs further evaluation in patients with advanced pulmonary sarcoidosis.Declaration of interestRP Baughman received grant support for clinical trials from Bayer, Gilead, Genetech, Novartis, Mallinckrodt, Actelion, Bellephron; acted a consultant for Actelion, Mallinckrodt, Kinevant, aTyr, Methelial, Abbivie, Bellephron; is on the speakers bureau for Mallinckrodt, Boehringer Ingelheim, United Therapeutics. R Gupta has acted as a consultant for Mallinckrodt. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.Reviewer disclosuresPeer reviewers on this manuscript have no relevant financial or other relationships to disclose.Article highlights 10-20% of patients with sarcoidosis may develop pulmonary fibrosis.Advanced pulmonary sarcoidosis refers to phenotypes of pulmonary sarcoidosis that often lead to significant loss of lung function, respiratory failure or death. This is driven mainly by advanced pulmonary fibrosis.Morbidity and mortality are high due to complications involving bronchiectasis, infections, and pulmonary hypertension.Recognition of underlying disease process(es) is the key to individualized treatment. A multidisciplinary approach involving experts in pulmonary sarcoidosis, pulmonary hypertension, and lung transplantation is crucial in providing individualized for patients with progressive and severe fibrosis.PFT, HRCT, FDG-PET/CT and potential serum biomarkers have been used to monitor patients, identify inflammation, and assess response to anti-inflammatory therapies.Management of pulmonary fibrosis is not standardized and relies on expert-opinion. Antiinflammatory, antifibrotic and pulmonary vasodilator therapy can be useful on a case-bycase basis but needs further evaluation in patients with advanced pulmonary sarcoidosis.Additional informationFundingThis paper was not funded.