Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 20-06: Adult Still Disease + Key Features Download Section PDF Listen +++ ++ A systemic form of juvenile chronic arthritis; this rare syndrome also occurs in adults Most adults are in their 20s or 30s; onset after age 60 is rare High spiking fevers are much more prominent, especially at the outset, than arthritis The course of adult Still disease can be monophasic, intermittent, or chronic Macrophage activation syndrome is a life-threatening complication; it can manifest as Fever Splenomegaly Either thrombocytopenia or neutropenia, or both Hypertriglyceridemia Hypofibrinogenemia Marked elevation of serum ferritin Elevated soluble CD25 Depressed natural killer cell activity Hemophagocytosis in bone marrow, spleen, and lymph nodes + Clinical Findings Download Section PDF Listen +++ ++ Fever Often with daily spikes to 40°C Associated with sweats and chills and then plunging to normal or several degrees below normal in the absence of antipyretics Sore throat is common initial complaint An evanescent salmon-colored nonpruritic rash Characteristic Appears chiefly on the chest and abdomen Can be missed since it often appears only with the fever spike Lymphadenopathy Pericardial effusions Joint symptoms Mild or absent in the beginning However, a destructive arthritis, especially of the wrists, may develop months later Differential diagnosis Familial Mediterranean fever TNF receptor-1 associated periodic syndrome Schnitzler syndrome (intermittent bone pain, urticarial rash, and fever associated with a monoclonal gammopathy) + Diagnosis Download Section PDF Listen +++ ++ Anemia and leukocytosis, with white blood counts sometimes exceeding 40,000/mcL Serum ferritin levels are often strikingly elevated (> 3000 mg/mL or 6741 pmol/L) + Treatment Download Section PDF Listen +++ ++ About half of the patients respond to nonsteroidal anti-inflammatory drugs Half of patients require prednisone, sometimes in doses > 60 mg/day orally For patients with refractory disease, Targeting IL-1 with anakinra or canakinumab or Targeting IL-6 with tocilizumab can be effective