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Periodic Fever Syndrome are autoinflammatory diseases which cause episodic illness and do not have an infectious cause. Periodic Fever Syndrome are genetic and are also present during childhood. It is defined by the various genetic defects testing, by the clinical features of the syndrome, the affected part of the body, age of the patient and the area of the world from where the patient belongs. Treatment is based on the understanding of the gene defect.
Despite similarities in symptoms, periodic fever syndrome has different patterns of inheritance, causes, length and frequency of ‘attacks.’ Some of the chronic condition associated with Periodic Fever Syndrome includes Cryopyrin-associated periodic syndrome (CAPS), hyperimmunoglobulinemia D syndrome (HIDS), TNF receptor-associated periodic syndrome (TRAPS) and Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA).
Periodic Fever Syndrome affects many organs and tissues and has its own set of symptoms along with high temperature during attacks. Elevated serum levels of acute-phase reactants such as fibrinogen and serum amyloid A, leukocytosis and high level of erythrocyte sedimentation rate (ESR) are associated with periodic fever syndrome.
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Diagnostic tools and genetic test are available for patients with periodic fever. For some conditions such as Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA), no genetic test are available yet. Hereditary periodic fever syndromes (HPFSs) are rare and is characterized by short and recurrent attacks. These attacks undergo remission without the use of antibiotic, anti-inflammatory, or immunosuppressive treatment. The patient feels healthy and is back to its daily routine until the next episode of periodic fever occurs.
Previously, there was no approved medicine for the treatment of TNF Receptor-Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulinemia D Syndrome (HIDS)/ Mevalonate Kinase Deficiency (MKD).
In September 2016, the US Food and Drug Administration (FDA) has granted Novartis’ ilaris (canakinumab) expanded use for the treatment of these three rare and distinct types of Periodic Fever Syndromes.Treatment with oral anti-inflammatory drugs, e.g. corticosteroids and non-steroidal anti-inflammatory drugs (NSAIDs), helps to control the symptoms but...
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