PMID- 30858405
OWN - NLM
STAT- MEDLINE
DCOM- 20200925
LR  - 20210109
IS  - 2045-2322 (Electronic)
IS  - 2045-2322 (Linking)
VI  - 9
IP  - 1
DP  - 2019 Mar 11
TI  - Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary 
      Hypertension.
PG  - 4061
LID - 10.1038/s41598-019-40030-w [doi]
LID - 4061
AB  - The presence of pulmonary hypertension (PH) significantly worsens outcomes in 
      patients with advanced sarcoidosis, but its optimal management is unknown. We 
      aimed to characterize a large sarcoidosis-associated pulmonary hypertension 
      (SAPH) cohort to better understand patient characteristics, clinical outcomes, 
      and management strategies including treatment with PH therapies. Patients at Duke 
      University Medical Center with biopsy-proven sarcoidosis and SAPH confirmed by 
      right heart catheterization (RHC) were identified from 1990-2010. Subjects were 
      followed for up to 11 years and assessed for differences by treatment strategy 
      for their SAPH, including those who were not treated with PH-specific therapies. 
      Our primary outcomes of interest were change in 6-minute walk distance (6MWD) and 
      change in N-terminal pro-brain natriuretic peptide (NT-proBNP) by after therapy. 
      We included 95 patients (76% women, 86% African American) with SAPH. Overall, 70% 
      of patients had stage IV pulmonary sarcoidosis, and 77% had functional class 
      III/IV symptoms. Median NT-proBNP value was elevated (910 pg/mL), and right 
      ventricular dysfunction was moderate/severe in 55% of patients. Median values for 
      mean pulmonary artery pressure (49 mmHg) and pulmonary vascular resistance (8.5 
      Woods units) were consistent with severe pulmonary hypertension. The mortality 
      rate over median 3-year follow-up was 32%. Those who experienced a clinical event 
      and those who did not had similar overall echocardiographic findings, 
      hemodynamics, 6MWD and NT-proBNP at baseline, and unadjusted analysis showed that 
      only follow-up NT-proBNP was associated with all-cause hospitalization or 
      mortality. A sign test to evaluate the difference between NT-Pro-BNP before and 
      after PH therapy produced evidence that a significant difference existed between 
      the median pre- and post-NT-Pro-BNP (-387.0 (IQR: -1373.0-109), p = 0.0495). Use 
      of PH-specific therapy may be helpful in selected patients with SAPH and 
      pre-capillary pulmonary vascular disease. Prospective trials are needed to 
      characterize responses to PH-specific therapy in this subset of patients with 
      SAPH.
FAU - Parikh, Kishan S
AU  - Parikh KS
AUID- ORCID: 0000-0001-9996-8916
AD  - Department of Medicine, Duke University Medical Center, Durham, North Carolina, 
      USA. kishan.parikh@duke.edu.
FAU - Dahhan, Talal
AU  - Dahhan T
AD  - Department of Medicine, Duke University Medical Center, Durham, North Carolina, 
      USA.
FAU - Nicholl, Leigh
AU  - Nicholl L
AD  - Department of Biostatistics and Bioinformatics, Duke University, Durham, NC, USA.
FAU - Ruopp, Nicole
AU  - Ruopp N
AD  - Department of Medicine, Duke University Medical Center, Durham, North Carolina, 
      USA.
FAU - Pomann, Gina-Maria
AU  - Pomann GM
AD  - Department of Biostatistics and Bioinformatics, Duke University, Durham, NC, USA.
FAU - Fortin, Terry
AU  - Fortin T
AD  - Department of Medicine, Duke University Medical Center, Durham, North Carolina, 
      USA.
FAU - Tapson, Victor F
AU  - Tapson VF
AD  - Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, California, 
      USA.
FAU - Rajagopal, Sudarshan
AU  - Rajagopal S
AUID- ORCID: 0000-0002-3443-5040
AD  - Department of Medicine, Duke University Medical Center, Durham, North Carolina, 
      USA.
LA  - eng
PT  - Journal Article
DEP - 20190311
PL  - England
TA  - Sci Rep
JT  - Scientific reports
JID - 101563288
RN  - 0 (Biomarkers)
RN  - DCR9Z582X0 (Epoprostenol)
RN  - JED5K35YGL (Iloprost)
RN  - RUM6K67ESG (treprostinil)
SB  - IM
MH  - Aged
MH  - Biomarkers/blood
MH  - Cardiac Catheterization
MH  - Echocardiography
MH  - Epoprostenol/administration & dosage/analogs & derivatives
MH  - Female
MH  - Hemodynamics/*drug effects
MH  - Humans
MH  - Iloprost/administration & dosage
MH  - Male
MH  - Middle Aged
MH  - Pulmonary Arterial Hypertension/blood/complications/*drug therapy/physiopathology
MH  - Sarcoidosis, Pulmonary/blood/complications/*drug therapy/physiopathology
MH  - Treatment Outcome
MH  - Vascular Resistance/physiology
MH  - Ventricular Dysfunction, Right/physiopathology
PMC - PMC6411962
COIS- Sudarshan Rajagopal (Sudarshan.rajagopal@duke.edu): Consulted for United 
      Therapeutics, Gilead Sciences and Bayer Sciences. Research funded by United 
      Therapeutics and Gilead Sciences.
EDAT- 2019/03/13 06:00
MHDA- 2020/09/26 06:00
PMCR- 2019/03/11
CRDT- 2019/03/13 06:00
PHST- 2018/04/24 00:00 [received]
PHST- 2018/12/31 00:00 [accepted]
PHST- 2019/03/13 06:00 [entrez]
PHST- 2019/03/13 06:00 [pubmed]
PHST- 2020/09/26 06:00 [medline]
PHST- 2019/03/11 00:00 [pmc-release]
AID - 10.1038/s41598-019-40030-w [pii]
AID - 40030 [pii]
AID - 10.1038/s41598-019-40030-w [doi]
PST - epublish
SO  - Sci Rep. 2019 Mar 11;9(1):4061. doi: 10.1038/s41598-019-40030-w.