NLRC4-Associated Macrophage Activation Syndrome-Like Illness

…r Canna in March 2014 at the American College of Rheumatology Pediatric Rheumatology Symposium (PRYSM) was the first time that we saw published research about this newly identified autoinflammatory disease associated with periodic fevers and macrophage activation syndrome (MAS). We had heard about this case, because the family is involved in our patient community, and were eagerly anticipating the debut of this finding in the medical world. Refere…

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Periodic Fever Syndrome in…Dogs? Your Four-Legged Best Friend Could Have a Rare Autoinflammatory Disease

…autoinflammatory disease, mevalonate kinase deficiency (hyper-IgD syndrome [HIDS]). Shar-Peis with FSF also have increased IL-6 levels. FSF has been known to be a genetic autoinflammatory disease for several years, but a genetic test for it just became available in 2016. The gene responsible, the HAS2 gene, is the same one that creates the signature wrinkled look of the Shar-Pei. Every Shar-Pei has a mutation on or near HAS2 that creates more hyal…

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