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Mechanism of Disease
Not an actual patient. Individual results will vary.
IL-1β is a critical driver of autoinflammatory disease1-4
Autoinflammatory diseases have a different pathogenic profile than autoimmune diseases1-6
| Autoinflammatory Diseases | Autoimmune Diseases |
| Key drivers: IL-1 β, IL-6, IL-18, and TNF | Key drivers: 1FN-y and IL-17 |
IL-1β is primarily induced and actively produced by immune cells under disease conditions7,8
In certain autoinflammatory diseases, there is an excessive release of activated 1L-1β.2,4,9,10
This overabundance can1,2,4,8-10:
- Cause an inflammatory cascade
- Trigger a feedback loop, inducing the production of even more IL-1β
The inflammatory cascade in PFS can drive fever and systemic inflammation1,4,11-12
References: 1. Jesus AA, Oliveira JB, Hilário MO, et al. Pediatric hereditary autoinflammatory syndromes. J Pediatr (Rio J). 2010;86(5):353 366. doi:10.2223/JPED.2015 2. Church LD, Cook GP, McDermott MF. Primer: inflammasomes and interleukin 1β in inflammatory disorders. Nat Clin Pract Rheumatol. 2008;4(1):34 42. doi:10.1038/ncprheum0681 3. Lin Y T, Wang C T, Gershwin ME, Chiang B L. The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis. Autoimmun Rev. 2011;10(8):482 489. doi:10.1016/j.autrev.2011.02.001 4. Lachmann HJ, Quartier P, So A, Hawkins PN. The emerging role of interleukin 1β in autoinflammatory diseases. Arthritis Rheum. 2011;63(2):314 324. doi:10.1002/art.30105 5. Mellins ED, Macaubas C, Grom AA. Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions. Nat Rev Rheumatol. 2011;7(7):416 426. doi:10.1038/nrrheum.2011.68 6. Warrington R, Watson W, Kim HL, Antonetti FR. An introduction to immunology and immunopathology. Allergy Asthma Clin Immunol. 2011;7(suppl 1):S1. doi:10.1186/1710 1492 7 S1 S1 7. Dinarello CA, Simon A, van der Meer JWM. Treating inflammation by blocking interleukin 1 in a broad spectrum of diseases. Nat Rev Drug Discov. 2012;11(8):633-652. doi:10.1038/nrd3800 8. Dinarello CA. Overview of the IL 1 family in innate inflammation and acquired immunity. Immunol Rev. 2018;281(1):8 27. doi:10.1111/imr.12621 9. Dinarello CA. Blocking interleukin 1β in acute and chronic autoinflammatory diseases. J Intern Med. 2011;269(1):16 28. doi:10.1111/j.1365 2796.2010.02313.x 10. Ostrov BE. Immunotherapeutic biologic agents to treat autoinflammatory diseases. In: Metodiev K, ed. Immunotherapy–Myths, Reality, Ideas, Future. InTech; 2017:chap 12. Accessed March 29, 2021. doi:10.5772/66547 11. Lopalco G, Cantarini L, Vitale A, et al. Interleukin 1 as a common denominator from autoinflammatory to autoimmune disorders: premises, perils, and perspectives. Mediators Inflamm. 2015;2015:194864. doi:10.1155/2015/194864 12. Lopez Castejon G, Brough D. Understanding the mechanism of IL 1β secretion. Cytokine Growth Factor Rev. 2011;22(4):189 195. doi:10.1016/j.cytogfr.2011.10.001