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For further information, see CMDT Part 20-06: Adult Still Disease

Key Features

  • A systemic form of juvenile chronic arthritis; 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

  • 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

  • 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

  • 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

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