2008 - The Pulmonary Circulation: Scleroderma-Related Pulmonary Arterial Hypertension

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 scl

2008 - The Pulmonary Circulation: Scleroderma-Related Pulmonary Arterial Hypertension

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Clinical and demographic factors in scleroderma-related PAH

P. Hassoun (Baltimore, Md, United States of America)

Assembly Symposium

Accompanying documents

Re-defining end-points in the assessment of pulmonary hypertension

A. Peacock (Glasgow, United Kingdom)

Slides

Accompanying documents

Right ventricular interaction in pulmonary arterial hypertension

A. Vonk Noordegraaf (Amsterdam, Netherlands)

Assembly Symposium

Accompanying documents

Determinants of survival in scleroderma-related PAH

S. Kawut (New York, Ny, United States of America)

Slides

Accompanying documents

The Role of Inflammation in promoting pulmonary hypertension in idiopatchi and connective tissue disease-associated PAH

M. Humbert (Clamart, France)

Accompanying documents

Auto-immunity in idiopathic and scleroderma-related PAH

L. Mouthon (Paris, France)

Slides

Accompanying documents

Stimulatory autoantibodies to the PDGF receptor in scleroderma

A. Gabrielli (Ancona, Italy)

Slides

Accompanying documents

The genetics of systemic sclerosis

C. Fonseca (London, United Kingdom)

Slides

Accompanying documents

Emerging therapies in PAH and scleroderma-related PAH

G. Simonneau (Clamart, France)

Slides

Role of anti-proliferative therapy in PAH

H. Ghofrani (Giessen, Germany)

Slides

Accompanying documents

2008 - The Pulmonary Circulation: Scleroderma-Related Pulmonary Arterial Hypertension

2008 - The Pulmonary Circulation: Scleroderma-Related Pulmonary Arterial Hypertension

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