PMID- 20015974
OWN - NLM
STAT- MEDLINE
DCOM- 20101207
LR  - 20181201
IS  - 1462-0332 (Electronic)
IS  - 1462-0324 (Linking)
VI  - 49
IP  - 3
DP  - 2010 Mar
TI  - Long-term outcome of systemic sclerosis-associated pulmonary arterial 
      hypertension treated with bosentan as first-line monotherapy followed or not by 
      the addition of prostanoids or sildenafil.
PG  - 490-500
LID - 10.1093/rheumatology/kep398 [doi]
AB  - OBJECTIVE: Data on long-term efficacy of bosentan, an oral dual ET receptor 
      antagonist, in SSc-associated pulmonary arterial hypertension (SSc-PAH) are 
      lacking. We aimed to describe the long-term outcome of SSc-PAH treated with 
      first-line monotherapy bosentan followed or not by the addition of prostanoids or 
      sildenafil. METHODS: A prospective analysis of 49 consecutive SSc-PAH patients 
      treated with first-line bosentan was performed. New York Heart Association (NYHA) 
      functional class, 6-min walk distance (6MWD) and haemodynamics were assessed at 
      baseline and after 4 and 12 months. RESULTS: At 4 months, significant 
      improvements in NYHA functional class and haemodynamics were observed with 
      stabilization at 1 year. There was no significant improvement in 6MWD. Overall 
      survival estimates were 80, 56 and 51% at 1, 2 and 3 years, respectively, and 
      were significantly worse than those in a cohort of patients with idiopathic PAH 
      (92, 89 and 79% at 1, 2 and 3 years, respectively; P < 0.0001). Twenty-three 
      patients (47%) died after a mean follow-up of 23 (18) months. In multivariate 
      analysis, baseline and 4-month NYHA functional class and 4-month cardiac index 
      were independent factors associated with overall survival. CONCLUSIONS: In our 
      cohort of consecutive SSc-PAH patients treated with first-line bosentan, 
      improvement in NYHA functional class and haemodynamics was significant after 4 
      months of treatment and stabilized afterwards. One-year overall survival rate was 
      higher than previously reported in historical series. However, long-term 
      prognosis remains poor. Our study underlines the importance of haemodynamic 
      evaluation 4 months after the start of treatment to provide strong parameters 
      associated with survival-like cardiac index.
FAU - Launay, David
AU  - Launay D
AD  - Universite Paris-Sud, Faculte de Medecine, Kremlin-Bice tre, France.
FAU - Sitbon, Olivier
AU  - Sitbon O
FAU - Le Pavec, Jerome
AU  - Le Pavec J
FAU - Savale, Laurent
AU  - Savale L
FAU - Tcherakian, Colas
AU  - Tcherakian C
FAU - Yaici, Azzedine
AU  - Yaici A
FAU - Achouh, Lara
AU  - Achouh L
FAU - Parent, Florence
AU  - Parent F
FAU - Jais, Xavier
AU  - Jais X
FAU - Simonneau, Gerald
AU  - Simonneau G
FAU - Humbert, Marc
AU  - Humbert M
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
DEP - 20091216
PL  - England
TA  - Rheumatology (Oxford)
JT  - Rheumatology (Oxford, England)
JID - 100883501
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Endothelin A Receptor Antagonists)
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
MH  - Adult
MH  - Aged
MH  - Antihypertensive Agents/adverse effects/*therapeutic use
MH  - Bosentan
MH  - Drug Administration Schedule
MH  - Drug Therapy, Combination
MH  - Endothelin A Receptor Antagonists
MH  - Epidemiologic Methods
MH  - Epoprostenol/therapeutic use
MH  - Exercise Test/methods
MH  - Female
MH  - Hemodynamics/drug effects
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/etiology/physiopathology
MH  - Iloprost/therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Piperazines/therapeutic use
MH  - Purines/therapeutic use
MH  - Scleroderma, Systemic/*complications/physiopathology
MH  - Sildenafil Citrate
MH  - Sulfonamides/adverse effects/*therapeutic use
MH  - Sulfones/therapeutic use
MH  - Treatment Outcome
EDAT- 2009/12/18 06:00
MHDA- 2010/12/14 06:00
CRDT- 2009/12/18 06:00
PHST- 2009/12/18 06:00 [entrez]
PHST- 2009/12/18 06:00 [pubmed]
PHST- 2010/12/14 06:00 [medline]
AID - kep398 [pii]
AID - 10.1093/rheumatology/kep398 [doi]
PST - ppublish
SO  - Rheumatology (Oxford). 2010 Mar;49(3):490-500. doi: 10.1093/rheumatology/kep398. 
      Epub 2009 Dec 16.