Figure 3. The autoinflammatory penumbra.

Major families of autoinflammatory disorders include the inflammasome/IL-1 diseases, the interferonopathies, the NFκB/TNF disorders, and autoinflammation mediated by other mechanisms. Many other human diseases exhibit clinical and/or mechanistic overlap with these disease families without perhaps being best conceptualized as primarily autoinflammatory; some of these are depicted in a shaded zone (penumbra) surrounding the monogenic autoinflammatory diseases. PFAPA, Periodic Fevers with Aphthous stomatitis, Pharyngitis and Adenopathy; SURF, Syndrome of Undifferentiated Recurrent Fever.