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Table 1 Classification of the autoinflammatory diseases of the patients of our cohort according to the immune mechanism responsible for mediating the disease process (with modifications from Manthiram et al. 2017)

From: Monogenic autoinflammatory diseases in children: single center experience with clinical, genetic, and imaging review

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
  1. FMF familial Mediterranean fever; TRAPS tumor necrosis factor receptor-associated periodic syndrome; MKD Mevalonate kinase deficiency; HIDS hyperimmunoglobulinemia D syndrome; NOMID neonatal onset multisystem inflammatory disease; MWS Muckle-Wells syndrome; PAPA pyogenic arthritis, pyoderma gangrenosum and acne; DIRA deficiency of interleukin-1 receptor antagonist; DADA2 deficiency of adenosine deaminase type 2; IL10 def interleukin 10 deficiency; MAS macrophage activation syndrome; HA20 deficiency haploinsufficiency of A20; SIFD sideroblastic anemia with B cell immunodeficiency, periodic fever, and developmental delay