An expert overview of pulmonary fibrosis in sarcoidosis.

IF 2.9 3区 医学 Q2 RESPIRATORY SYSTEM Expert Review of Respiratory Medicine Pub Date : 2023-02-01 DOI:10.1080/17476348.2023.2183193
Rohit Gupta, Jin Sun Kim, Robert P Baughman
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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.

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结节病肺纤维化的专家综述。
晚期肺结节病是指肺结节病的表型,通常导致肺功能明显丧失,呼吸衰竭或死亡。大约20%的结节病患者可能发展到这种状态,主要是由晚期肺纤维化驱动的。晚期纤维化常伴有结节病的并发症,包括感染、支气管扩张和肺动脉高压。涉及领域:本文将重点介绍结节病肺纤维化的发病机制、疾病的自然史、诊断和潜在的治疗选择。在专家意见部分,我们将讨论重大疾病患者的预后和处理。专家意见:虽然一些肺结节病患者通过抗炎治疗保持稳定或改善,但其他患者会发展为肺纤维化和进一步的并发症。虽然晚期肺纤维化是结节病死亡的主要原因,但尚无基于证据的纤维化结节病治疗指南。目前的建议是基于专家共识,通常包括与结节病、肺动脉高压和肺移植专家的多学科讨论,以促进对此类复杂患者的护理。目前评估治疗的工作包括使用抗纤维化疗法治疗晚期肺结节病。
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来源期刊
CiteScore
6.80
自引率
2.60%
发文量
90
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
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