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

	Adult-onset Still's disease
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Adult-onset Still’s Disease from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Template:Adult-onset Still's disease On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Template:Adult-onset Still's disease All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Adult-onset Still's disease natural history, complications and prognosis
CDC on Template:Adult-onset Still's disease
Template:Adult-onset Still's disease in the news
Blogs on Template:Adult-onset Still's disease
Risk calculators and risk factors for Template:Adult-onset Still's disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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.
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]

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.

↑ 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.
↑ Sachs RN, Talvard O, Lanfranchi J (June 1990). "Myocarditis in adult Still's disease". Int. J. Cardiol. 27 (3): 377–80. PMID 2351497.
↑ Cush JJ (2000). "Adult-onset Still's disease". Bull Rheum Dis. 49 (6): 1–4. PMID 11100625.
↑ 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.