“Enfermedad de still del adulto”

Abstract

Adult Still's disease (ESA) is an infrequent pathology, with inflammatory characteristics, with a broad spectrum in its clinical presentation. This pathology occurs in people older than 15 years, since in younger patients is the clinical picture called juvenile idiopathic systemic arthritis or Still's disease. Its diagnosis is based fundamentally on the high clinical and analytical suspicion through the performance of basic complementary tests of infectious, autoimmune and neoformative causes of fever of uncertain origin. The presence of fever in all patients, without existing clinical or pathognomonic laboratory manifestations of any disease, initially makes it difficult to diagnose. Several criteria have been proposed for its detection, among which are those of Yamaguchi itself that helps us to approach an accurate diagnosis. Given its behavior and the lack of diagnostic tests, it is considered a diagnosis of exclusion. The pathophysiology of ESA has not yet been fully understood. Numerous causes have been postulated such as infectious agents, genetic factors, alteration of the regulation of the immune response and apoptosis, but there are still no specific data, although it is said that the immunological pathways of the disease have been attributed as the most significant in the physiopathology. Its treatment is clinical and is based on the use of a combined scheme. Most cases of ESA occur as a mild or moderate disease; however it should be considered that severe manifestations of the disease can occur that present with high mortality, mainly when associated with severe pulmonary manifestations.