Polyradiculoneuritis, cryopyrin-associated periodic syndromes, and familial Mediterranean fever.

Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease in the world. It shows a high prevalence in countries bordering the eastern Mediterranean. The course of the disease is marked by short and self-limiting bouts of fever, often with a monthly periodicity; other clinical manifestations include serositis and synovitis. Mutations of the cold-induced autoinflammatory syndrome gene (CIAS1) give rise to the cryopyrin-associated periodic syndromes (CAPS). There are 3 types of CAPS, and they were initially described as distinct clinical entities: familial cold autoinflammatory syndrome (FCAS), Muckle—Wells syndrome, and chronic infantile neurological cutaneous and articular syndrome/neonatal onset multisystem inflammatory disease (CINCA/NOMID). From a clinical viewpoint, these 3 entities have certain traits in common, such as early onset of the disease (normally before the age of 5), presence of generalised rash-like exanthem, and a major acute-phase reaction. We present the case of a 53-year-old white man who was examined due to daily fever of 38◦ to 39 ◦C over the past year. He informed us that in the 8 years prior to that time, he had consulted several times for fever and been admitted to hospital on 3 occasions for observation. Each time, his fever resolved with high doses of prednisone. His personal medical history includes an episode of uveitis at the age of 33. At the age of 37, he was diagnosed with diabetes mellitus and polyradiculoneuritis. The same year, he presented symptoms of fever, predominantly leftsided bilateral peripheral facial palsy, and muscle weakness;