Abstract: Still′s disease is a classic autoinflammatory disease, predominantly manifested by polyarthritis and systemic inflammation, with severe complications including macrophage activation syndrome and pulmonary disease. In 2024, the European Alliance of Associations for Rheumatology and the Paediatric Rheumatology European Society for the first time defined systemic juvenile idiopathic arthritis and adult-onset Still′s disease collectively as a single disease entity, that is, Still′s disease. This review summarizes the unification of disease nomenclature for Still′s disease, advances in pathogenesis research, updates in therapeutic regimens, and management strategies for difficult-to-treat patients, aiming to summarize cuttingedge evidence and provide references for the clinical diagnosis and treatment of this disease.