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1. Introduction
2. Dramatic new developments in past few years
3. Pulmonary hypertension response
4. Pulmonary hypertension (PHT)
5. Epidemiology and pathophysiology
6. Diagnostic and treatment challenges
7. WHO classification of pulmonary hypertension
8. New York Heart Association classification
9. WHO organization functional classification 1973
10. Main difference between the two classifications
11. Pathogenesis of pulmonary hypertension
12. Imbalance of vascular effectors
13. Roles of vascular effectors
14. Production of fibromuscular changes
15. PHT genetic and immunological factors
16. PHT characteristics: summary
17. The various factors involved in PHT pathogenesis
18. Role of anti-phospholipid antibodies in PHT
19. Clue for anti-phospholipid antibodies role in PHT
20. Pulmonary hypertension and systemic sclerosis
21. PHT and anti-phospholipid antibodies: conclusions
22. Role of T cells in PHT pathogenesis
23. Conditions with CD4 cell defects and PAH
24. Depletion of CD4 cells and autoimmune disease
25. Autoimmune aspects of PHT
26. PHT may precede the connective tissue disease
27. Some idiopathic HPT patients are ANF positive
28. Anticentromere antibody
29. Pulmonary arterial hypertension (PAH) and HIV
30. Relationship to AIRE gene
31. APECED is a condition of AIRE loss of function
32. PAH reported following splenectomy
33. Clinical manifestations
34. Signs of pulmonary hypertensions
35. Regular routine examinations and PHT signs
36. X-ray finding of PHT
37. Electrocardiography might alert of PAH presence
38. Laboratory tests
39. Predisposing factors in thromboembolic PAH (1)
40. Predisposing factors in thromboembolic PAH (2)
41. Diagnosis and severity assessment of PAH
42. Chronic thromboembolic pulmonary hypertension
43. Embolism travels up to heart and lungs
44. Histology seen in the lungs
45. Histological lesions
46. Examples of vascular pathology
47. Intimal proliferation in pulmonary hypertension
48. Medial hypertrophy of the pulmonary arterial
49. Pulmonary artery lumen and lymphocyte infiltration
50. Poor prognostic factors
51. Causes of death
52. Prognosis of PAH
53. Survival of pulmonary hypertension patients
54. PHT in connective tissue disorders
55. Form of PHT in connective tissue disorders
56. PHT type variation in connective tissue diseases
57. PAH in the different connective tissue disorders
58. Pulmonary hypertension in SLE
59. Types of pulmonary hypertension in SLE
60. PHT in SLE and it's associations
61. PHT and systemic sclerosis characteristics
62. Frequency of PAH and fibrosis in SSc patients
63. PAH from heart involvement
64. Echocardiogram and diastolic dysfunction
65. Onset of PAH is often delayed
66. Screening of patients with SSc for PAH
67. Use of DLCO
68. DLCO in patients who developed PAH vs. controls
69. The ratio FVC% / DLCO% is very helpful
70. FVC/DLCO ratio between 1.6 - 1.8
71. Cardiopulmonary findings in limited SSc with PAH
72. Autoantibodies in PAH cases and controls
73. Frequency of auto-antibodies in all SSc patients
74. Echocardiography
75. Echo may indicate PAH but does not diagnose it
76. Risk factors for PAH in systemic sclerosis
77. Making diagnosis of PAH
78. Right heart catheterization as PAH measurement
79. Dyspnoea/significant mortality and PAH in SSc
80. Natural history of elevated PASP in SSc
81. Incidence of HLA DRw52 gene/DR3/DR6 antigens
82. Anti-phospholipid antibodies
83. Rheumatoid arthritis and PHT
84. Elevations of pulmonary arterial pressures
85. Sjogren's syndrome and PHT
86. Treatment
87. Improved survival for patients with IPAH
88. Approved therapy for SSc PHT
89. Treprostinal or inhaled iloprost treatment
90. Continuous i.v. therapy for SSc PHT
91. Other therapies for SSc PHT
92. Comments on PAH therapy
93. New compounds being currently tested
94. Supportive therapy
95. Treatment is dependent on type of lung disease
96. PHT in diffuse SSc
97. Summary of PAH in SSc
98. Acknowledgments

Topics Covered

• Diagnosis of pulmonary arterial hypertension (PAH)
• Classification of the types of PAH
• Pathogenesis
• Role of endothelin 1
• Role of prostacyclin/thromboxane
• Role of antiphospholipid antibodies
• CD4 cells and PAH in HIV infection and autoimmune diseases
• PAH in SLE, systemic sclerosis and CREST syndrome
• Rheumatoid arthritis, Sjogren's syndrome
• Autoimmune aspects of "primary" idiopathic PAH
• Clinical manifestations of PAH
• Thromboembolic PAH
• New therapies with endothelin 1 antagonists (non-selective and selective), prostacyclin analogues (inhaled and IV) and phosphodiesterase inhibitors (e.g. sildenafil)

Links

Series:

• Autoimmunity

Categories:

• Clinical Practice
• Immunology

Therapeutic Areas:

• Immunology & Inflammation

Talk Citation

Asherson, R. (2007, October 1). Pulmonary hypertension in the connective tissue disease pathogenesis, clinical features and therapeutic options in 2006 [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 19, 2024, from https://hstalks.com/bs/202/.
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Publication History

• Published on October 1, 2007
• Archived on May 31, 2018

Financial Disclosures

• Prof. Ronald Asherson has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.