To classify the autoinflamamtory<unk>  is quite complicated and it is a work in process that will need to be refined as more information on the pathogenesis of these<unk>  becomes available. Clinical classification 1. Episodic and multisystemic AIDs (NLRP12-associated<unk>  Mevalonate kinase deficiency, PFAPA (Periodic fever syndrome, aphthous stomatitis, pharyngitis, and cervical adenitis) or TRAPS (tumor necrosis factor (TNF) receptor–associated periodic fever syndrome)) 2. Episodic, affecting the joints (Gout) 3. Episodic, affecting bone (Chronic recurrent multifocal osteomyelitis (CRMO)) 4. Persistent and multisystemic (Schnitzler syndrome, Crohn's<unk> or DIRA) 5. Persistent, affecting the skin (Interleukin-36-receptor antagonist deficiency (DITRA), Sweet syndrome or Neutrophilic panniculitis) Molecular mechanism of the origin 1. Inflammasome activation (Mevalonate kinase deficiency or Muckle–Wells syndrome) 2. NFκB activation (NLRP12-associated<unk>  Crohn's<unk> or Blau syndrome) 3. IL‑1β pathway dysregulation (PFAPA, Schnitzler syndrome, DIRA or DITRA) 4. Impaired efficacy of cytotoxic T lymphocytes with compensatory macrophage activation (Familial hemophagocytic lymphohistiocytosis (HLH)) 5. Inactivation of IL‑10 signalling (Early-onset enterocolitis) 6. Multiple (TRAPS) and Uncharacterized (CRMO or Behçet<unk>  Simplified classification by the predominant cytokine or pathway 1. IL-1 mediated 2. IFN-mediated 3. Mediated by increased NF-κB activation
Autoinflammatory diseases
Immune-mediated inflammatory diseases
Central nervous system disease
Risk factors for tuberculosis
Skin manifestations of sarcoidosis
Cranial nerve disease
Oral manifestations of systemic disease
Autoimmune disease