| Abstract | | Periodic fever syndromes are classified as a subset of the autoinflammatory diseases. They are defined as recurrent self-resolving attacks of systemic inflammatory reactions, which are usually accompanied by associated symptoms and signs. The differential diagnosis includes infections, autoimmune diseases and malignancy. In the last seven years the causative genes for eight hereditary autoinflammatory syndromes have been identified. Familial Mediterranean fever is the most frequent entity, while other autoinflammatory syndromes are very rare. The underlying mechanisms of these inflammatory diseases are specif c for eaoh type. Future research into molecular mechanisms will hopefully lead to a better understanding of these diseases and with that, new opportunities for better treatment. Key words: periodic fever syndromes, hereditary autoinflammatory syndromes, familial Mediterranean fever, hyperimmunoglobulinaemia D periodic fever syndrome, tumour necrosis factor receptor associated periodic syndrome, periodic fever accompanied by aphthous stomatitis, pharyngitis, cervical adenitis, chronic infantile neurologic cutaneous articular syndrome.
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