mechanism of autoinflammatory Disease
The IL-1 Pathway
The IL-1 pathway is critical in autoinflammatory disease activity1
There are 2 forms of IL-1: IL-1α and IL-1β2,3
- IL-1α and IL-1β have different roles in inflammation:
- IL-1α is constitutively expressed in normal cells* and is the primary driver of local inflammation as part of the host defense
- IL-1β is primarily induced and actively produced by immune cells under disease conditions
IL-1α
IL-1β
Proinflammatory cytokine3
Protein constitutively present in normal cells2,3*
Primary driver of local inflammation as part of host defense2
Primarily induced and actively produced by innate immune cells under disease conditions2,3
Key driver of systemic inflammation that can lead to tissue/organ damage2-4
Triggers downstream activation of proinflammatory cytokines (eg, IL-6, TNF)5-7
Further induces its own production2
*Keratinocytes of the skin, epithelial cells of mucosal membranes throughout the body, and cells of organs such as the liver, lung, and kidney.
No clinical implications can be derived from these data. Prospective, head-to-head studies comparing inhibition of both IL-1α and IL-1β, vs IL-1β alone, have not been done in patients with autoinflammatory diseases.
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Better understand autoinflammatory diseases and the various characteristics found in certain types of diseases
