{"title":"An expert overview of pulmonary fibrosis in sarcoidosis.","authors":"Rohit Gupta, Jin Sun Kim, Robert P Baughman","doi":"10.1080/17476348.2023.2183193","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Areas covered: </strong>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.</p><p><strong>Expert opinion: </strong>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.</p>","PeriodicalId":12103,"journal":{"name":"Expert Review of Respiratory Medicine","volume":null,"pages":null},"PeriodicalIF":2.9000,"publicationDate":"2023-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Expert Review of Respiratory Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1080/17476348.2023.2183193","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
引用次数: 1
Abstract
Introduction: 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.
期刊介绍:
Coverage will include the following key areas:
- Prospects for new and emerging therapeutics
- Epidemiology of disease
- Preventive strategies
- All aspects of COPD, from patient self-management to systemic effects of the disease and comorbidities
- Improved diagnostic methods, including imaging techniques, biomarkers and physiological tests.
- Advances in the treatment of respiratory infections and drug resistance issues
- Occupational and environmental factors
- Progress in smoking intervention and cessation methods
- Disease and treatment issues for defined populations, such as children and the elderly
- Respiratory intensive and critical care
- Updates on the status and advances of specific disease areas, including asthma, HIV/AIDS-related disease, cystic fibrosis, COPD and sleep-disordered breathing morbidity