Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 205, Issue 5, Pages 495-506Publisher
AMER THORACIC SOC
DOI: 10.1164/rccm.202106-1366CI
Keywords
sarcoidosis; advanced sarcoidosis; advanced pulmonary sarcoidosis; pulmonary fibrosis; sarcoidosis-associated pulmonary hypertension
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Advanced sarcoidosis is a form of sarcoidosis that poses a significant risk of organ function loss or death, with the lung being the most commonly affected organ. It can lead to complications such as pulmonary fibrosis and bronchiectasis. Although it affects a small proportion of patients, it is the leading cause of poor outcomes.
The term advanced sarcoidosis is used for forms of sarcoidosis with a significant risk of loss of organ function or death. Advanced sarcoidosis often involves the lung and is described as advanced pulmonary sarcoidosis (APS), which includes advanced pulmonary fibrosis, associated complications such as bronchiectasis and infections, and pulmonary hypertension. Although APS affects a small proportion of patients with sarcoidosis, it is the leading cause of poor outcomes, including death. Here we review the major patterns of APS with a focus on the current management as well as potential approaches for improved outcomes for this most serious sarcoidosis phenotype.
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