Adult Onset Still’s Disease

Abstract

The adult onset Still's disease is a rare affection characterised by occurrence of fever, arthralgia or arthritis and evanescent cutaneous eruption. Multiple other systemic lesions make the diagnosis more difficult. Several diagnostic criteria were formulated to confirm this disease. The physiopathology of the adult onset Still's disease is not well elucidated. However, several studies based on new facts in physiopathology, improved the therapy of refractory forms for which the biotherapy was an interesting alternative. The TNF alpha receptor antagonists are efficient on systemic and articular manifestations of this disease and allow a corticosteroid's saving. Tocilizumab (interleukin 6 receptor antagonist), and Anakinra (interleukin 1 receptor antagonist) are also new promising treatments for resistant forms.

Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis

“…Presence of pericarditis, pleural effusions, and transient pulmonary infiltrates has been observed in 30-40% of patients diagnosed to have AOSD [5]. Pleural effusions are usually small and described as exudative and sterile [8]. Rarely, AOSD may progress to life-threatening adult respiratory distress syndrome, especially if associated with macrophage-activating syndrome [9].…”