Flare of a Cryopyrin-associated Periodic Syndrome Following Vaccination with Neisseria Meningitidis Polysaccharides

We report the case of a 6-year-old boy with the clinical diagnosis of cryopyrin-associated periodic syndrome (CAPS)1. The patient’s symptoms had started at 3 years of age and consisted of recurrent cold-induced urticarial rashes in association with arthralgia, myalgia, malaise, abdominal pain, headaches, and fever. The symptoms were classified as a mild to moderate CAPS phenotype (in between Muckle Wells-Syndrome and familial cold autoinflammatory syndrome). The family history was negative for autoinflammatory diseases. In 2016, the boy developed a severe systemic inflammatory episode, shortly after the injection of a meningococcal polysaccharide conjugate vaccine (Nimenrix). Prior vaccinations against influenza, tetanus, diphtheria, polio, rotavirus, and hepatitis A had been uneventful, although pneumococcal vaccinations (Prevenar) in 2008 and 2009 were each associated with high fever, commencing within 24 h and lasting for several days. At the time of meningococcal vaccination, the patient was in full remission for several months and had no CAPS-related symptoms. Prior to the scheduled therapy with an anti-interleukin … Address correspondence to Prof. Dr. U.A. Walker, Unispital Basel, Department of Rheumatology, Basel, Switzerland. E-mail: Ulrich.walker{at}usb.ch