PMID- 19118270
OWN - NLM
STAT- MEDLINE
DCOM- 20090707
LR  - 20211020
IS  - 1931-3543 (Electronic)
IS  - 0012-3692 (Print)
IS  - 0012-3692 (Linking)
VI  - 135
IP  - 6
DP  - 2009 Jun
TI  - Treatment of sarcoidosis-associated pulmonary hypertension. A two-center 
      experience.
PG  - 1455-1461
LID - S0012-3692(09)60347-0 [pii]
LID - 10.1378/chest.08-1881 [doi]
AB  - BACKGROUND: Pulmonary hypertension (PH) is a common complication of sarcoidosis 
      that is associated with increased mortality. The pathogenesis of PH in 
      sarcoidosis is uncertain, and the role of pulmonary arterial hypertension 
      (PAH)-specific therapies remains to be determined. METHODS: We conducted a 
      retrospective study of patients with sarcoidosis and PH at two referral centers. 
      New York Heart Association (NYHA) functional class, exercise capacity, 
      hemodynamic data, pulmonary function tests, and survival were collected and 
      analyzed. RESULTS: Twenty-two sarcoidosis patients treated with PAH-specific 
      therapies were identified. After a median of 11 months of follow-up, NYHA class 
      was improved in nine subjects. Mean 6-min walk distance (n = 18) increased by 59 
      m (p = 0.032). Patients with a higher FVC experienced a greater increment in 
      exercise capacity. Among 12 patients with follow-up hemodynamic data, mean 
      pulmonary artery pressure was reduced from 48.5 +/- 4.3 to 39.4 +/- 2.8 mm Hg (p 
      = 0.008). The 1- and 3-year transplant-free survival rates were 90% and 74%, 
      respectively. CONCLUSIONS: PAH-specific therapy may improve functional class, 
      exercise capacity, and hemodynamics in PH associated with sarcoidosis. 
      Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to 
      verify this apparent benefit. Mortality among the study population was high, 
      highlighting the need for urgent evaluation at a lung transplant center.
FAU - Barnett, Christopher F
AU  - Barnett CF
AD  - Critical Care Medicine Department, Clinical Center, National Institutes of 
      Health, Bethesda, MD. Electronic address: cbarnett@ucsd.edu.
FAU - Bonura, Eric J
AU  - Bonura EJ
AD  - Department of Internal Medicine, Georgetown University, Washington, DC.
FAU - Nathan, Steven D
AU  - Nathan SD
AD  - Inova Fairfax Hospital, Falls Church, VA.
FAU - Ahmad, Shahzad
AU  - Ahmad S
AD  - Inova Fairfax Hospital, Falls Church, VA.
FAU - Shlobin, Oksana A
AU  - Shlobin OA
AD  - Inova Fairfax Hospital, Falls Church, VA.
FAU - Osei, Kwabena
AU  - Osei K
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 
      Baltimore, MD.
FAU - Zaiman, Ari L
AU  - Zaiman AL
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 
      Baltimore, MD.
FAU - Hassoun, Paul M
AU  - Hassoun PM
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 
      Baltimore, MD.
FAU - Moller, David R
AU  - Moller DR
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 
      Baltimore, MD.
FAU - Barnett, Scott D
AU  - Barnett SD
AD  - Inova Fairfax Hospital, Falls Church, VA.
FAU - Girgis, Reda E
AU  - Girgis RE
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 
      Baltimore, MD.
LA  - eng
GR  - Intramural NIH HHS/United States
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, N.I.H., Intramural
DEP - 20081231
PL  - United States
TA  - Chest
JT  - Chest
JID - 0231335
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Piperazines)
RN  - 0 (Purines)
RN  - 0 (Sulfonamides)
RN  - 0 (Sulfones)
RN  - BW9B0ZE037 (Sildenafil Citrate)
RN  - DCR9Z582X0 (Epoprostenol)
RN  - JED5K35YGL (Iloprost)
RN  - Q326023R30 (Bosentan)
SB  - IM
CIN - Chest. 2009 Jun;135(6):1410-2. PMID: 19497891
MH  - Administration, Inhalation
MH  - Administration, Oral
MH  - Aged
MH  - Antihypertensive Agents/*administration & dosage
MH  - Bosentan
MH  - Cohort Studies
MH  - Drug Therapy, Combination
MH  - Epoprostenol/administration & dosage
MH  - Female
MH  - Follow-Up Studies
MH  - Hemodynamics/*drug effects/physiology
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/*etiology/mortality
MH  - Iloprost/administration & dosage
MH  - Kaplan-Meier Estimate
MH  - Linear Models
MH  - Male
MH  - Middle Aged
MH  - Piperazines/administration & dosage
MH  - Probability
MH  - Purines/administration & dosage
MH  - Respiratory Function Tests
MH  - Retrospective Studies
MH  - Risk Assessment
MH  - Sarcoidosis/*complications/diagnosis/mortality
MH  - Severity of Illness Index
MH  - Sildenafil Citrate
MH  - Statistics, Nonparametric
MH  - Sulfonamides/administration & dosage
MH  - Sulfones/administration & dosage
MH  - Survival Rate
MH  - Treatment Outcome
PMC - PMC2821289
EDAT- 2009/01/02 09:00
MHDA- 2009/07/08 09:00
PMCR- 2010/06/01
CRDT- 2009/01/02 09:00
PHST- 2009/01/02 09:00 [entrez]
PHST- 2009/01/02 09:00 [pubmed]
PHST- 2009/07/08 09:00 [medline]
PHST- 2010/06/01 00:00 [pmc-release]
AID - S0012-3692(09)60347-0 [pii]
AID - 08-1881 [pii]
AID - 10.1378/chest.08-1881 [doi]
PST - ppublish
SO  - Chest. 2009 Jun;135(6):1455-1461. doi: 10.1378/chest.08-1881. Epub 2008 Dec 31.