PMID- 10616012
OWN - NLM
STAT- MEDLINE
DCOM- 20000128
LR  - 20131121
IS  - 0004-3591 (Print)
IS  - 0004-3591 (Linking)
VI  - 42
IP  - 12
DP  - 1999 Dec
TI  - Systemic sclerosis-associated pulmonary hypertension: short- and long-term 
      effects of epoprostenol (prostacyclin).
PG  - 2638-45
AB  - OBJECTIVE: To evaluate the short- and long-term effects of intravenous 
      epoprostenol in patients with pulmonary hypertension (PH) associated with 
      systemic sclerosis (SSc). METHODS: Sixteen patients with SSc-associated PH and 
      New York Heart Association (NYHA) class III or IV symptomatology underwent right 
      heart catheterization for determination of baseline hemodynamic values. 
      Vasoreactivity was assessed with either inhaled nitric oxide or intravenous 
      adenosine. After a medication washout period, all patients received intravenous 
      epoprostenol in incrementally increasing doses; tolerance was assessed according 
      to symptoms and hemodynamic findings at each dose increment and at the conclusion 
      of the medication trial. Once a stable medication regimen was established, 
      patients were discharged and followed up as outpatients for assessment of 
      symptoms and exercise tolerance as measured by change in the NYHA class. Repeat 
      hemodynamic testing was performed in 4 patients at 1 year and in 2 patients at 2 
      years of treatment. RESULTS: Therapeutic response to epoprostenol, defined by a 
      reduction in the pulmonary vascular resistance of > or =25%, was achieved in the 
      short-term treatment period in 13 of 16 patients (81.3%). Improvement in symptoms 
      and exercise tolerance occurred in all patients, and a significant short-term 
      hemodynamic response was observed. Followup hemodynamic tests revealed persistent 
      favorable responses in all 4 of the patients studied. CONCLUSION: Most patients 
      with PH secondary to SSc manifest favorable hemodynamic responses to epoprostenol 
      in the short term. Long-term epoprostenol was generally well tolerated and 
      provides a potential therapeutic option for patients with PH secondary to SSc.
FAU - Klings, E S
AU  - Klings ES
AD  - The Pulmonary Center, Boston University School of Medicine, Massachusetts 02118, 
      USA.
FAU - Hill, N S
AU  - Hill NS
FAU - Ieong, M H
AU  - Ieong MH
FAU - Simms, R W
AU  - Simms RW
FAU - Korn, J H
AU  - Korn JH
FAU - Farber, H W
AU  - Farber HW
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Arthritis Rheum
JT  - Arthritis and rheumatism
JID - 0370605
RN  - 0 (Antihypertensive Agents)
RN  - DCR9Z582X0 (Epoprostenol)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Antihypertensive Agents/administration & dosage/therapeutic use
MH  - Epoprostenol/administration & dosage/therapeutic use
MH  - Female
MH  - Hemodynamics/drug effects
MH  - Humans
MH  - Hypertension, Pulmonary/*complications/drug therapy
MH  - Injections, Intravenous
MH  - Lung/physiology
MH  - Male
MH  - Middle Aged
MH  - Scleroderma, Systemic/*complications/drug therapy
MH  - Time Factors
MH  - Vascular Resistance/drug effects
EDAT- 2000/01/01 00:00
MHDA- 2000/01/01 00:01
CRDT- 2000/01/01 00:00
PHST- 2000/01/01 00:00 [pubmed]
PHST- 2000/01/01 00:01 [medline]
PHST- 2000/01/01 00:00 [entrez]
AID - 10.1002/1529-0131(199912)42:12<2638::AID-ANR20>3.0.CO;2-X [doi]
PST - ppublish
SO  - Arthritis Rheum. 1999 Dec;42(12):2638-45. doi: 
      10.1002/1529-0131(199912)42:12<2638::AID-ANR20>3.0.CO;2-X.