Adult-onset Still's disease natural history, complications and prognosis

If left untreated, adult-onset Still’s disease (AOSD) follows a relapsing and remitting course. Initial presentation of AOSD may be between 16 to 35 years of age. Symptoms usually evolve over weeks to months. Life-threatening complications known to be associated with AOSD include, macrophage activating syndrome (MAS), disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), diffuse alveolar hemorrhage, pulmonary arterial hypertension (PAH), pericardial tamponade and myocarditis. The prognosis of adult-onset Still’s disease depends upon the clinical course of the disease. The chronic articular form of the disease is associated with worse prognosis. Other indicators of poor prognosis of AOSD include, presence of polyarthritis, interestitial pneumonia, pleuritis, joint erosions, Still’s rash (salmon-colored maculopapular rash) and development of secondary complications.

• If left untreated, adult-onset Still’s disease (AOSD) follows a relapsing and remitting course.
• Initial presentation of AOSD may be between 16 to 35 years of age.
• Symptoms usually can evolve over weeks to months.
• AOSD exhibits a variable clinical course:
  • 20% with long-term remission
  • 30% remit-relapse
  • 50% chronic arthritis
• May progress to develop macrophage activating syndrome (MAS), also known as hemophagocytic syndrome.

Adult-onset Still’s disease may lead to the development of the following life-threatening complications:^[1][2]

• Macrophage activation syndrome (MAS)
• Disseminated intravascular coagulation (DIC)
• Thrombotic thrombocytopenic purpura (TTP)
• Diffuse alveolar hemorrhage
• Pulmonary arterial hypertension (PAH)
• Pericardial tamponade
• Myocarditis

The prognosis of adult-onset Still’s disease depends upon the clinical course of the disease:^[3][4]

• The self-limited form of AOSD is characterized by systemic symptoms for example: fever, rash, serositis, and organomegaly.
• Most patients experience one episode and remit within 1 year of the initial presentation.

• Patients experience pre-dominantly systemic symptoms with or without joint symptoms.
• Patients typically follow a relapsing-remitting course with intermittent flares.
• Most episodes milder than the initial presentation.

• Patients have pre-dominant articular symptoms and joint destruction.
• The articular form is associated with a worse prognosis than the systemic form.
• Severe joint destruction may warrant joint replacement surgery in severely ill patients.

• The following findings may be associated with a poor prognosis in patients suffering from AOSD:
  • Polyarthritis
  • Interstitial pneumonia
  • Pleuritis
  • Joint erosions
  • Still’s rash
  • Development of secondary complications, for example:
    • Macrophage activation syndrome (MAS)
    • Disseminated intravascular coagulopathy (DIC)
    • Thrombotic thrombocytopenic purpura (TTP)
    • Diffuse alveolar hemorrhage
    • Pulmonary arterial hypertension

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