PMID- 37284520
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230608
IS  - 2045-8932 (Print)
IS  - 2045-8940 (Electronic)
IS  - 2045-8932 (Linking)
VI  - 13
IP  - 2
DP  - 2023 Apr
TI  - Pulmonary vasodilator therapy in sarcoidosis-associated pulmonary hypertension 
      may decrease lung function decline and mortality.
PG  - e12245
LID - 10.1002/pul2.12245 [doi]
LID - e12245
AB  - The efficacy of treating sarcoidosis-associated pulmonary hypertension (SAPH) 
      with pulmonary vasodilator therapy is unclear. The INCREASE trial showed 
      improvement in 6-minute walk distance (6MWD) and in decline in functional vital 
      capacity (FVC) in patients with interstitial lung disease and pulmonary 
      hypertension. We hypothesize that patients with SAPH treated with pulmonary 
      vasodilators have reduced decline in FVC. We retrospectively analyzed patients 
      with SAPH who underwent lung transplantation evaluation. The primary objective 
      was to compare change in FVC between patients with SAPH who received pulmonary 
      vasodilators (treated) and those who did not (untreated). Secondary objectives 
      were to compare the change in 6MWD, change in oxygen requirement, transplant 
      rates, and mortality between treated and untreated SAPH patients. We identified 
      58 patients with SAPH; 38 patients received pulmonary vasodilator therapy, and 20 
      patients did not. Treated SAPH patients had significantly less decline in FVC 
      than untreated SAPH patients (+54 mL vs. -357 mL, p < 0.01). Treated SAPH 
      patients had significantly higher survival than untreated SAPH patients. 
      Receiving PH therapy was significantly associated with a change in FVC (estimate 
      0.36 +/- 0.07, p < 0.01) and decreased mortality (hazard ratio 0.29, confidence 
      interval 0.12-0.67, p < 0.01). Among patients with SAPH, those who received 
      pulmonary vasodilator therapy had significantly less decline in FVC and increased 
      survival. Receiving pulmonary vasodilator therapy was significantly associated 
      with FVC change and decreased mortality. These study findings point towards 
      potential benefit of pulmonary vasodilator therapy in SAPH patients. Further 
      prospective studies are required to fully elucidate the benefits of pulmonary 
      vasodilator therapy in SAPH.
CI  - (c) 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on 
      behalf of Pulmonary Vascular Research Institute.
FAU - Gayen, Shameek
AU  - Gayen S
AUID- ORCID: 0000-0002-0275-7416
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
FAU - Ansari, Sohaib
AU  - Ansari S
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
FAU - Lashari, Bilal H
AU  - Lashari BH
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
FAU - Zhao, Huaqing
AU  - Zhao H
AD  - Center for Biostatistics and Epidemiology Lewis Katz School of Medicine at Temple 
      University Hospital Philadelphia Pennsylvania USA.
FAU - Criner, Gerard J
AU  - Criner GJ
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
FAU - Gupta, Rohit
AU  - Gupta R
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
FAU - James Mamary, Albert
AU  - James Mamary A
AD  - Department of Thoracic Medicine and Surgery Lewis Katz School of Medicine at 
      Temple University of Hospital Philadelphia Pennsylvania USA.
LA  - eng
PT  - Journal Article
DEP - 20230605
PL  - United States
TA  - Pulm Circ
JT  - Pulmonary circulation
JID - 101557243
PMC - PMC10241350
OTO - NOTNLM
OT  - functional vital capacity
OT  - lung transplant
OT  - pulmonary hypertension
OT  - pulmonary vasodilator therapy
OT  - sarcoidosis
COIS- Each author declares that he or she has no commercial associations (e.g. 
      consultancies, stock ownership, equity interest, patent/licensing arrangement 
      etc.) that might pose a conflict of interest in connection with the submitted 
      article.
EDAT- 2023/06/07 13:10
MHDA- 2023/06/07 13:11
PMCR- 2023/06/05
CRDT- 2023/06/07 09:50
PHST- 2023/04/11 00:00 [received]
PHST- 2023/05/09 00:00 [revised]
PHST- 2023/05/19 00:00 [accepted]
PHST- 2023/06/07 13:11 [medline]
PHST- 2023/06/07 13:10 [pubmed]
PHST- 2023/06/07 09:50 [entrez]
PHST- 2023/06/05 00:00 [pmc-release]
AID - PUL212245 [pii]
AID - 10.1002/pul2.12245 [doi]
PST - epublish
SO  - Pulm Circ. 2023 Jun 5;13(2):e12245. doi: 10.1002/pul2.12245. eCollection 2023 
      Apr.