I. Autoinflammatory diseases mediated by activated inflammosomes and IL1β production | |
1. FMF 2. CAS 3. MKD/HIDS 4. PAPA 5. NLRC4-MAS 6. DIRA | |
II. Autoinflammatory diseases mediated by the NF-κB pathway | |
1. Blau syndrome 2. HA20 deficiency | |
III. Autoinflammatory diseases mediated by the TNF pathway | |
1. TRAPS 2. IL-10 Deficiency | |
IV. Autoinflammatory disease mediated by the type I interferon pathway | |
1. AGS | |
V. Autoinflammatory diseases with unique not well established mechanism (to be included in the coming classifications) | |
1. DADA2 2. SIFD |