PMID- 28090299
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 2045-8932 (Print)
IS  - 2045-8940 (Electronic)
IS  - 2045-8932 (Linking)
VI  - 6
IP  - 4
DP  - 2016 Dec
TI  - Tadalafil therapy for sarcoidosis-associated pulmonary hypertension.
PG  - 557-562
LID - 10.1086/688775 [doi]
AB  - Sarcoidosis-associated pulmonary hypertension (SAPH) is estimated to occur in at 
      least 5% or more of sarcoidosis patients, and it contributes to significant 
      morbidity and mortality. Optimal therapy for SAPH is not well established. We 
      performed a 24-week open-label trial of tadalafil for SAPH at 2 academic medical 
      centers. Subjects were required to have confirmed sarcoidosis plus a right heart 
      catheterization within 12 months of enrollment showing a mean pulmonary artery 
      pressure >/= 25 mmHg, a pulmonary artery wedge pressure </= 15 mmHg, and a calculated 
      pulmonary vascular resistance >/= 3 Wood units. Subjects received 20 mg/day of 
      tadalafil for the first 4 weeks and then 40 mg/day for the subsequent 20 weeks. 
      Sixteen patients were screened, 12 of whom met criteria for enrollment. At 24 
      weeks, there was no overall improvement in 6-minute walk distance (6MWD). Five of 
      the 12 subjects dropped out of the study early (2 for social reasons, 3 for 
      medical reasons). There was no significant change in short form 36, St. George's 
      respiratory questionnaire, or maximum Borg dyspnea scores over the 24 weeks. 
      There were no significant adverse events or laboratory abnormalities clearly 
      related to tadalafil in the cohort. The study did not meet the primary end point 
      of change in 6MWD because of the small sample size. Tadalafil was generally 
      safely administered in this cohort of SAPH patients. There was a relatively high 
      dropout rate but no major adverse events and no clinical worsening. Larger 
      studies are needed to explore this question further. (Trial registration: 
      ClinicalTrials.gov identifier: NCT01324999).
FAU - Ford, H J
AU  - Ford HJ
AD  - Division of Pulmonary and Critical Care Medicine, University of North Carolina, 
      Chapel Hill, North Carolina, USA.
FAU - Baughman, R P
AU  - Baughman RP
AD  - Division of Pulmonary and Critical Care Medicine, University of Cincinnati 
      Hospitals, Cincinnati, Ohio, USA.
FAU - Aris, R
AU  - Aris R
AD  - Division of Pulmonary and Critical Care Medicine, University of North Carolina, 
      Chapel Hill, North Carolina, USA.
FAU - Engel, P
AU  - Engel P
AD  - Pulmonary Hypertension Program, Christ Hospital, Cincinnati, Ohio, USA.
FAU - Donohue, J F
AU  - Donohue JF
AD  - Division of Pulmonary and Critical Care Medicine, University of North Carolina, 
      Chapel Hill, North Carolina, USA.
LA  - eng
SI  - ClinicalTrials.gov/NCT01324999
PT  - Journal Article
PL  - United States
TA  - Pulm Circ
JT  - Pulmonary circulation
JID - 101557243
PMC - PMC5210066
OTO - NOTNLM
OT  - pulmonary hypertension
OT  - sarcoidosis
OT  - tadalafil
EDAT- 2017/01/17 06:00
MHDA- 2017/01/17 06:01
PMCR- 2016/12/01
CRDT- 2017/01/17 06:00
PHST- 2017/01/17 06:00 [entrez]
PHST- 2017/01/17 06:00 [pubmed]
PHST- 2017/01/17 06:01 [medline]
PHST- 2016/12/01 00:00 [pmc-release]
AID - PC2013313 [pii]
AID - 10.1086/688775 [doi]
PST - ppublish
SO  - Pulm Circ. 2016 Dec;6(4):557-562. doi: 10.1086/688775.