FCAS - FAMILILA COLD AUTOINFLAMMATORY SYNDROME
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Core tip: Children with non-infectious recurrent fever more often fall into two diagnostic categories. The first and most common is periodic fever, aphthae, pharingitis, adenitis (PFAPA), the second, far more rare, are hereditary periodic fevers. Very recently a third category has been increasingly recognized, and is that of undifferentiated periodic fevers or undifferentiated periodic fever (UPF). UPF include patients who do not meet the diagnostic criteria for PFAFA or for a monogenic disease. The clinical presentation and management of patients with UPF are poorly defined. In this study, the authors describe a cohort of patients with UPF showing that: (1) The clinical manifestations are on a half way of clinical spectrum between PFAPA and hereditary periodic fever; (2) PRINTO-Eurofever score is not useful to guide treatment choices and does not predict disease course; and (3) Both steroids and colchicine are useful to control symptoms in most cases.
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PATAGONIA
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