World Scleroderma Foundation (WSF)
Summer School
Pulmonary Arterial Hypertension (PAH)- in depth course
Thu 23 June- Sun 26 June 2011
Location: Villa Medicea La Ferdinanda & Villaggio di “Artimino”,
Florence, Tuscany, Italy
Background
WSF, World Scleroderma Foundation, has been considering for some time the need for
an “in-depth” course to enable rheumatologists to truly expand their knowledge in the
field of pulmonary vascular disease (PVD), particularly in the pathophysiology and
diagnosis with relation to the scleroderma patients they see.
WSF would appreciate support in the delivery of this meeting and are looking for a
sponsor to support financially (to cover some general costs, development of video
content plus a bank of “train the trainer” materials. These will allow the meeting to be
more broadly replicated/executed).
Objectives
1. General
 Provide a vehicle for expanding expertise ( Scleroderma PAH) in the
rheumatology community
 Developing future experts and excellence in the field of Scleroderma and
PVD
 Encourage cross speciality (Pulmonology, Cardiology, Connective Tissue
Pathology) networking by involving “highest” experts in the field of PAH
 Developing an alumni of experts who can transfer their knowledge and
improve skills at a local country level worldwide
2. Specific
 Content for this meeting focussed on Scleroderma and PVD to allow depth of
learning and discussion in this disease area
 Interactive format to aid networking and learning
 Practical, discussion and plenary to address diversity of learning styles
 Use of patients and patient cases to support practical application of learning
 Production of materials to enable “excellent” replication
3. Long term
 Replicate template and core content to support education and training on
Scleroderma PAH across the global community
 Maintaining and increasing size of alumni
Requirements
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3 day meeting (Thursday noon to Sunday noon)
Rental of Echo machines
Video capability or satellite links to expert centres
Venue to accommodate 80-100 (30 students, 20-30 teachers, technicians,
companies)
 Production of a bank of training materials (meeting presentations, videos and
discussion guides) for use during and post the meeting
World Scleroderma Foundation Steering Committee
A Tyndall, Dan Furst, Marco Matucci Cerinic
Local Organizing Committee
Serena Guiducci, Silvia Bellando Randone, Cinzia Pruneti
Board
1. R Abbate
2. M Aringer
3. Y Allanore
4. J Coghlan
5. M D’Alto*
6. O Distler
7. J Easaw*
8. D Furst
9. N Galiè
10. L Gargani*
11. G F Gensini
12. H Gruenig
13. E Hachulla
14. S Harari
15. D Khanna
16. O Kowal Bielecka
17. M Matucci Cerinic
18. U Muller Ladner
19. R Naeje
20. A Pignone
21. J Seibold
22. JL Vachiery
23. G Valentini
24. P Vitulo
25. D Vizza*
26. F Wigley
*Tutors
Scientific Programme
Thursday :
15h00- 19h00
 Welcome- The World Scleroderma Foundation (WSF)
M Matucci Cerinic & Dan Furst
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Wellcome- The University of Florence
Gian Franco Gensini, Dean, University of Florence
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Pre-meeting MCQ test S Guiducci & S Bellando Randone
Learning objectives of the course D Furst
Eustar data on PAH (database) (15’) U Muller Ladner
Lecture : Pathophysiology of lung circulation R Naeje (30’)
Session
1.
2.
3.
4.
5.
PH-PAH definition / classification in SSc (30’) A Ghofrani
ESR PAH Guidelines (20’) N Galiè
Genetics of PAH and SSc PAH (20’) Y Allanore
The screening of SSc PAH E Hachulla
Outcome measures in SSc PAH D Furst
Friday
MORNING
SSc- CLINICAL ASPECTS
 8.30-9.30 Clinical aspects of SSc PAH : when to suspect ? in depth analysis of the
symptoms (dyspnea, heart failure) J Seibold- C Denton
 9.30-10.30 Heart involvement in SSc : focus on myocardiopathy and diastolic
dysfunction, differential diagnosis of PAH in SSc G Valentini- Y Allanore
10.30-11.00 BREAK
 11-12 Lung involvement: prevalence, patterns, imaging subgroup at risk,
disproportionate PAH, Differential outcomes of PAH vs ILD associated PAH D
Khanna- O Kowal
 12-12.30 Lecture- PAH pathogenesis in SSc O Distler
12.30-14.00 Lunch
AFTERNOON- Practical workshops (movies and practice)
14.00-15.00
 Pulmonary functional tests: useful parameters in PAH-SSc S Harari
o MOVIE- PFTs recorded S Harari
15.00-16.00
 Doppler echocardiography : fundamental parameters, parameters useful for
the diagnosis and prognosis of PAH, pitfalls D Vizza, L Gargani
16.00-16.30 BREAK
16.30-18.30
 Exercise echo , yes or no ? A Pignone
o HANDS ON- Doppler echocardiography on patients:
D Vizza, L Gargani, M D’Alto, J Easaw
Saturday
MORNING
8.30-9.30 Practical workshops (movies and practice)
 RHC how to interpret the results J Coghlan
o MOVIE- Catheterization recorded- J Coghlan
Break
11.00-13.00
o HANDS ON- Doppler echocardiography on patients:
D Vizza, L Gargani, M D’Alto, J Easaw
 13-14.30 lunch
AFTERNOON TREATMENT
14.30-18.00
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Update on PAH- Future molecular targets JL Vachiery
Current view of the treatment of iPAH M Humbert
Lung Transplantation in PAH P Vitulo
Current view of the treatment of SSc-PAH & Overall strategy
C Denton
Prostanoids in PAH F Wigley
ERAs in PAH J Seibold
PD5 in PAH JL Vachiery
Combination therapy M D’Alto
Coagulation, yes or no ? R Abbate
Supportive care H Gruenig
Sunday :
MORNING
 Post meeting test
o Difficult cases of SSc-PAH- presentations from young investigators
1. Pericarditis masking PAH
2. Diastolic dysfunction
3. Young patients with severe form and discussion of transplantation
4. Venous involvement