ERS Research Seminar: The Pulmonary Circulation: Scleroderma-Related Pulmonary Arterial Hypertension, Paris 2008

About
Research Seminars gather a small number (20 to 100) of well-established and preferably young investigators to address scientific topics in depth and help break new ground. Research Seminars take place over two days in easy-to-reach European cities. They are scientific Seminars involving recently published work and research in progress. Participation is fully supported by the budget granted by the ERS.
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Background

Pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with scleroderma.  With a prevalence of scleroderma of 240 patients/million, and a conservative estimate that 10% of patients will develop PAH, scleroderma-related PAH (PAH-SSc) is probably the leading cause of pulmonary hypertension within the category of PAH-related diseases. Therapeutic trials indicate that PAH-SSc has a significantly poorer response to therapy compared with idiopathic PAH. A limited progress in diagnosis and management of PAH-SSc includes lack of specific outcome measures (that factor in the components of the cardiovascular response) and limited knowledge on the phenotypic, genomic and genetic characteristics that predict the development of PAH and the response to therapy and survival.  

The focus of this seminar was to better understand:

  • the vasculopathy characteristics in scleroderma that lead to PAH
  • the value of outcome measures reliable in scleroderma
  • current and future targeted therapy for this devastating disease.
Background  Show

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 [+]  Slide presentation PDF journal article, handout or slides Clinical and demographic factors in scleroderma-related PAH
P. Hassoun (Baltimore, Md, United States of America)
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 [+]  Slide presentation PDF journal article, handout or slides Re-defining end-points in the assessment of pulmonary hypertension
A. Peacock (Glasgow, United Kingdom)
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 [+]  PDF journal article, handout or slides Right ventricular interaction in pulmonary arterial hypertension
A. Vonk Noordegraaf (Amsterdam, Netherlands)
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 [+]  Slide presentation PDF journal article, handout or slides Determinants of survival in scleroderma-related PAH
S. Kawut (New York, Ny, United States of America)
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 [+]  Slide presentation PDF journal article, handout or slides The Role of Inflammation in promoting pulmonary hypertension in idiopatchi and connective tissue disease-associated PAH
M. Humbert (Clamart, France)
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 [+]  Slide presentation PDF journal article, handout or slides Auto-immunity in idiopathic and scleroderma-related PAH
L. Mouthon (Paris, France)
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 [+]  Slide presentation PDF journal article, handout or slides Stimulatory autoantibodies to the PDGF receptor in scleroderma
A. Gabrielli (Ancona, Italy)
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 [+]  Slide presentation PDF journal article, handout or slides The genetics of systemic sclerosis
C. Fonseca (London, United Kingdom)
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 [+]  Slide presentation Emerging therapies in PAH and scleroderma-related PAH
G. Simonneau (Clamart, France)
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 [+]  Slide presentation PDF journal article, handout or slides 10 Role of anti-proliferative therapy in PAH
H. Ghofrani (Giessen, Germany)
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