title: Advanced pulmonary sarcoidosis.
abstract: OBJECTIVE
  To present an update on the most recent contributions in advanced pulmonary sarcoidosis (APS).
  
  
  RESULTS
  Pathology is better described and the differences between fibrosing pulmonary sarcoidosis and usual interstitial pneumonia (UIP) are clarified. Serial spirometry is the most reliable tool for monitoring evolution. Survival may be predicted by an integrative algorithm based on pulmonary function and computed tomography (CT).
  
  
  CONCLUSIONS
  APS is characterized by significant fibrocystic pulmonary lesions at CT and pathology. There are two main patterns of APS, one with predominant central bronchovascular distortion, often associated with airflow limitation, and the other with predominant honeycombing with a different location than in UIP with severe restrictive impairment and very low diffusion capacity of the lung for carbon monoxide. APS may be burnt out but is most often still active as evidenced by several findings, including on F-fluorodeoxyglucose-PET. There is an increased mortality and morbidity with chronic respiratory insufficiency, pulmonary hypertension stemming from multiple mechanisms, chronic pulmonary aspergillosis and extra infections. Acute worsening episodes are frequent. Serial spirometry, particularly forced vital capacity, is the most reliable tool for monitoring evolution. A new elegant algorithm based on pulmonary function and CT may predict survival. Despite important stakes, there is still a lack of therapeutic recommendations. However, the use of antisarcoidosis treatment is most often required at least as a temporary trial. Finally, the effect of pulmonary hypertension treatment has recently been the object of further evaluation.
authors.affiliation: aAP-HP, Hôpital Avicenne, Service de Pneumologie bUniversité Paris 13, EA2363, Sorbonne Paris Cité, Bobigny cAPHP, Laboratoire d'anatomie et cytologie pathologique, Hôpital Tenon dDépartement d'histologie, Université Pierre et Marie Curie Paris 6, Paris, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
authors.forename: Hilario
authors.initials: H
authors.lastname: Nunes
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Bernaudin
mesh_terms: D002908:Chronic Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000473:pathology; D017565:Sarcoidosis, Pulmonary / Q000473:pathology*; D014057:Tomography, X-Ray Computed / Q000379:methods