PMID- 17099027
OWN - NLM
STAT- MEDLINE
DCOM- 20061214
LR  - 20131121
IS  - 0012-3692 (Print)
IS  - 0012-3692 (Linking)
VI  - 130
IP  - 5
DP  - 2006 Nov
TI  - Sarcoidosis-associated pulmonary hypertension: outcome with long-term 
      epoprostenol treatment.
PG  - 1481-8
AB  - RATIONALE: Pulmonary hypertension is a known complication of sarcoidosis and is 
      associated with increased mortality. Little is known about the outcome of 
      sarcoidosis-associated pulmonary hypertension, including response to treatment. 
      OBJECTIVE: To determine the characteristics and outcome of patients with 
      sarcoidosis-associated pulmonary hypertension treated with IV epoprostenol. 
      DESIGN: Retrospective chart review of all cases of pulmonary hypertension with a 
      concomitant diagnosis of sarcoidosis evaluated in the Boston University Pulmonary 
      Hypertension Center from 2000 to 2004. MEASUREMENTS: Data collected included 
      patient demographics, sarcoidosis stage, pulmonary function, echocardiography 
      results, treatment, baseline and posttreatment hemodynamic measurements, and 
      clinical outcome. RESULTS: Eight patients were identified; four of the patients 
      had stage IV pulmonary sarcoidosis. Pulmonary function test results were notable 
      for severe diffusion impairment (mean diffusion capacity of the lung for carbon 
      monoxide, 30% of predicted), with only mild-to-moderate restrictive physiology 
      (mean FVC, 59% of predicted). Seventy-five percent of patients required 
      supplemental oxygen at the time of presentation. All patients had moderate or 
      severe pulmonary hypertension and were New York Heart Association (NYHA)/World 
      Health Organization (WHO) class III or IV. A vasodilator trial with epoprostenol 
      was performed in seven of the eight patients; six of the seven patients had a 
      significant hemodynamic response (> 25% reduction in pulmonary vascular 
      resistance). All but one of the responders (five of six patients) continued on 
      therapy. Average clinical improvement was one to two NYHA/WHO classes at a mean 
      follow-up of 29 months (range, 15 to 49 months). CONCLUSIONS: In patients with 
      sarcoidosis-associated pulmonary hypertension, the severity of pulmonary vascular 
      disease occurs out of proportion to lung function abnormalities. The majority of 
      our patients responded to epoprostenol; survival may be improved in this group.
FAU - Fisher, Kimberly A
AU  - Fisher KA
AD  - Pulmonary Center, Boston University School of Medicine, Boston, MA, USA. 
      FisherK@ummhc.org
FAU - Serlin, David M
AU  - Serlin DM
FAU - Wilson, Kevin C
AU  - Wilson KC
FAU - Walter, Robert E
AU  - Walter RE
FAU - Berman, Jeffrey S
AU  - Berman JS
FAU - Farber, Harrison W
AU  - Farber HW
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Chest
JT  - Chest
JID - 0231335
RN  - 0 (Antihypertensive Agents)
RN  - DCR9Z582X0 (Epoprostenol)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Antihypertensive Agents/adverse effects/*therapeutic use
MH  - Blood Pressure/drug effects/physiology
MH  - Dose-Response Relationship, Drug
MH  - Epoprostenol/adverse effects/*therapeutic use
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/*etiology/physiopathology
MH  - Infusions, Intravenous
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Sarcoidosis, Pulmonary/*complications/physiopathology
MH  - Treatment Outcome
MH  - Vascular Resistance/drug effects/physiology
EDAT- 2006/11/14 09:00
MHDA- 2006/12/15 09:00
CRDT- 2006/11/14 09:00
PHST- 2006/11/14 09:00 [pubmed]
PHST- 2006/12/15 09:00 [medline]
PHST- 2006/11/14 09:00 [entrez]
AID - S0012-3692(15)37326-8 [pii]
AID - 10.1378/chest.130.5.1481 [doi]
PST - ppublish
SO  - Chest. 2006 Nov;130(5):1481-8. doi: 10.1378/chest.130.5.1481.