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Abstract
Fever of unknown origin is a great challenge for physicians since its approach involves a wide range of differential diagnoses. The main causes are infectious, neoplastic and rheumatological, with infectious causes being the most frequent. Within rheumatological causes, JIA represents the main cause of FUO of rheumatological origin. A rare but highly fatal variant is systemic juvenile idiopathic arthritis.
Pathophysiologically, sJIA is characterized by activation of cells of the innate immune system, with the role of the adaptive immune response being limited in this disease. Within the clinical spectrum fever, arthritis, skin rash, and lymphadenopathy are the most common manifestations at diagnosis. As expected, sJIA is a diagnosis of exclusion and the key finding is the combination of fever with a daily pattern and arthritis; however, these are not highly specific signs and will require an extensive exercise in differential diagnoses.
Regarding treatment, the understanding of the pathophysiology of the disease has allowed the development of targeted therapies that, according to the evidence and current practice, reduce the morbidity and mortality of the disease.
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References
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