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

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Adult-onset Still's disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

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.

Natural History

  • 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.

Complications

Life threatening complications

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

Prognosis

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

Self-limited course

  • 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.

Intermittent systemic course

  • 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.

Chronic articular form

  • 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.

Indicators of poor prognosis

References

  1. Efthimiou P, Kadavath S, Mehta B (March 2014). "Life-threatening complications of adult-onset Still's disease". Clin. Rheumatol. 33 (3): 305–14. doi:10.1007/s10067-014-2487-4. PMID 24435354.
  2. Sachs RN, Talvard O, Lanfranchi J (June 1990). "Myocarditis in adult Still's disease". Int. J. Cardiol. 27 (3): 377–80. PMID 2351497.
  3. Cush JJ (2000). "Adult-onset Still's disease". Bull Rheum Dis. 49 (6): 1–4. PMID 11100625.
  4. Wouters JM, van de Putte LB (November 1986). "Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases". Q. J. Med. 61 (235): 1055–65. PMID 3659248.

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