PMID- 19208592
OWN - NLM
STAT- MEDLINE
DCOM- 20090601
LR  - 20181201
IS  - 0315-162X (Print)
IS  - 0315-162X (Linking)
VI  - 36
IP  - 4
DP  - 2009 Apr
TI  - Effect of bosentan on plasma markers of endothelial cell activity in patients 
      with secondary pulmonary hypertension related to connective tissue diseases.
PG  - 760-7
LID - 10.3899/jrheum.080542 [doi]
AB  - OBJECTIVE: To evaluate plasma markers of endothelial cell activity in patients 
      with pulmonary arterial hypertension (PAH) induced by connective tissue diseases 
      (CTD) before and after 3-month administration of bosentan. METHODS: We quantified 
      E, L and P-selectin (sE-S, sL-S, sP-S), thrombomodulin (TM), monocyte-chemotactic 
      protein 1 (MCP-1), human soluble CD40 ligand (sCD40L), and nitric oxide (NO) in 
      18 patients and 18 controls. We evaluated right ventricular systolic pressure 
      (RVSP) and the 6-minute walk test (6-MWT). RESULTS: All plasma markers but sL-S 
      and TM at Time 0 were significantly higher in patients compared with controls. 
      After 3 months of therapy, decreased levels were noted in NO (Time 0 24.05+/-6.01 
      mmol/l, Time 1 13.92+/-3.40 mmol/l; p<0.001) and sCD40L (Time 0 1685.33+/-866 
      pg/ml, Time 1 1055.11+/-630.6 pg/ml; p=0.017). In contrast, sP-S was 
      significantly increased (Time 0 88.36+/-47.76 ng/ml, Time 1 147.21+/-94.43 ng/ml; 
      p=0.021). All patients remained stable in WHO class III, and in 9 patients we 
      noted an improvement in 6-MWT. A correlation was found between Delta of RVSP and 
      6-MWT (r2=0.5355, p<0.001) as well as between Delta-sP-S and both Delta-6-MWT and 
      Delta-RVSP. An increase sP-S level was found in 89% of nonresponder patients, 
      whereas 55% of responders showed a stable or reduced sP-S level (p=0.016 
      responder vs nonresponder). CONCLUSION: Treatment with bosentan for 3 months 
      induced a beneficial effect by restoring endothelial function through a decrease 
      in the markers of endothelial cell activity, leading to stabilization or 
      improvement of severe PAH.
FAU - Cella, Giuseppe
AU  - Cella G
AD  - Department of Medical Sciences, University of Padova Medical Schoo, Via Ospedale 
      Civile 105, 35100 Padova, Italy. giuseppe.cella@unipd.it
FAU - Vianello, Fabrizio
AU  - Vianello F
FAU - Cozzi, Franco
AU  - Cozzi F
FAU - Marotta, Helga
AU  - Marotta H
FAU - Tona, Francesco
AU  - Tona F
FAU - Saggiorato, Graziella
AU  - Saggiorato G
FAU - Iqbal, Omer
AU  - Iqbal O
FAU - Fareed, Jawed
AU  - Fareed J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20090122
PL  - Canada
TA  - J Rheumatol
JT  - The Journal of rheumatology
JID - 7501984
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Biomarkers)
RN  - 0 (Sulfonamides)
RN  - Q326023R30 (Bosentan)
SB  - IM
CIN - J Rheumatol. 2009 Apr;36(4):665-6. PMID: 19342719
MH  - Adult
MH  - Aged
MH  - Antihypertensive Agents/*therapeutic use
MH  - Biomarkers/*blood
MH  - Bosentan
MH  - Connective Tissue Diseases/*complications/physiopathology
MH  - Disease Progression
MH  - Endothelial Cells/*metabolism
MH  - *Endothelium, Vascular/cytology/physiopathology
MH  - Exercise Test
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/*blood/*drug therapy/*etiology/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Sulfonamides/*therapeutic use
MH  - Treatment Outcome
EDAT- 2009/02/12 09:00
MHDA- 2009/06/02 09:00
CRDT- 2009/02/12 09:00
PHST- 2009/02/12 09:00 [entrez]
PHST- 2009/02/12 09:00 [pubmed]
PHST- 2009/06/02 09:00 [medline]
AID - jrheum.080542 [pii]
AID - 10.3899/jrheum.080542 [doi]
PST - ppublish
SO  - J Rheumatol. 2009 Apr;36(4):760-7. doi: 10.3899/jrheum.080542. Epub 2009 Jan 22.