NLRC4 and macrophage activation syndrome: Other conditions classified as inflammasomopathies, with increased levels of IL-1β, include the pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPA), caused by autosomal dominant mutation in the proline-serine-threonine phosphatase-interacting protein [PSTPIP1, or CD2- binding protein 1 (CD2BP1)] and the NLRC4-related MAS/syndrome of enterocolitis and autoinflammation associated with mutation in NLRC4, the latest also associated to increased levels of IL-18 (102).