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1. Introduction
2. Objectives
3. What monogenic autoinflammatory diseases are
4. Presentation of autoinflammatory diseases
5. Definition of inflammation
6. Immune dysregulatory conditions
7. Autoinflammatory diseases
8. Monogenic autoinflammatory diseases
9. Genetic cause for autoinflammatory diseases
10. IL-1 mediated autoinflammatory diseases
11. FCAS and MWS
12. NOMID/CINCA
13. The disease spectrum of CAPS
14. Prognosis in CAPS
15. Epidemiology
16. Differential diagnosis and diagnostic clues
17. Mutations identified in CIAS1/NLRP3
18. Pathogenesis
19. The inflammasome
20. Is NOMID an IL-1 disease?
21. Blocking IL-1 in NOMID with Anakinra
22. CNS disease
23. Controlling CNS inflammation
24. Inner ear
25. Eye
26. Musculoskeletal manifestations
27. No response to IL-1 blocking therapy
28. Pathogenesis of bone lesions in NOMID
29. Caspase-1 and PGE inhibition
30. Lessons from clinical studies
31. CAPS/NOMID summary
32. Approved treatments: Rilonacept in CAPS
33. Canakinumab/Ilaris in CAPS
34. Currently approved IL-1 inhibitors
35. Deficiency of the IL-1 receptor antagonist
36. Case report: 7 month old boy from Canada
37. Response to IL-1 blocking therapy
38. Genetic workup using a candidate gene approach
39. Genomic deletion in a Puerto Rican patient
40. Founder mutations in DIRA
41. Laboratory response to treatment with Anakinra
42. Vertebral manifestations: radiography
43. DIRA summary
44. Mice have different disease manifestations
45. Comparison NOMID-DIRA (1)
46. Comparison NOMID-DIRA (2)
47. IL-1 expression in the skin
48. Localization of neutrophilic infiltrate
49. Does IL-1 play a role in polygenic diseases?
50. Evidence of IL-1 in the pathogenesis of SOJIA
51. SOJIA - a polygenic IL-1 disease?
52. Inflammasome is activated by danger signals
53. "Metabolic" autoinflammatory diseases?
54. Gout, a "metabolic" autoinflammatory disease?
55. Type 2 diabetes, an autoinflammatory disease?
56. Or a "metabolic" autoinflammatory disease?
57. Inflammasome activation in obesity
58. IL-1 blockade improves glucose tolerance
59. Organ specificity is multi-factorial
60. Summary
61. Acknowledgements
62. The NIH

Topics Covered

• Understand the concept of autoinflammatory syndromes and typical clinical features
• Understand the role of IL-1 on the phenotype of two IL-1 mediated autoinflammatory syndromes: NOMID and DIRA
• Understand the impact of the discovery of the role of IL-1 in rare diseases
• The understanding of the pathogenesis of common diseases such as gout and Type II diabetes

Links

Series:

• Cytokines

Categories:

• Clinical Practice
• Immunology

Therapeutic Areas:

• Immunology & Inflammation

Talk Citation

Goldbach-Mansky, R. (2011, December 5). IL-1 as a key inflammatory regulator in autoinflammatory diseases [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved November 23, 2024, from https://doi.org/10.69645/TUIB3649.
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Publication History

• Published on December 5, 2011

Financial Disclosures

• Dr. Raphaela Goldbach-Mansky has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.