Description

Adult onset Still’s disease (AoSD) is a rare form of inflammatory arthritis that affects the entire body, impacting joints and organs. Typically affecting individuals over the age of 16, patients often endure bouts of elevated fever, joint discomfort, and skin rash. Blood tests commonly indicate elevated white blood cell counts. Despite extensive research, the exact cause of AoSD remains elusive, with uncertainty over whether it stems from autoimmune or autoinflammatory origins. The disease's progression varies widely among patients; while some experience sudden onset followed by swift and complete recovery, others contend with chronic manifestations or symptom flare-ups alternating with periods of remission. AoSD bears a resemblance to systemic juvenile rheumatoid arthritis, differing primarily in the age of onset. Although a definitive cure remains elusive, treatment options exist to manage inflammation and mitigate potential organ damage. There are three main patterns of the clinical course of AoSD namely monophasic, intermittent, and chronic. There is an approximately equal distribution in each category however some studies have shown a chronic articular pattern to be more common.

Prevalence

Incidence: 500 Cases per Year [France: Magadur-Joly 1995].
US cases: 320 to 3200 [Mitrovic 2019]

Notes
1. Still's Disease is very rare

Typical Age of onset

Two peaks of onset between 15-25 and 36-45 years

Symptoms

• Daily fever spikes 

• Joint pain (arthralgia)

• Joint warmth and swelling, especially knees and ankles

• Joint stiffness

• A salmon/pink-colored rash on chest and thighs during fever

• Severe muscle aches, especially during fever

• Sore throat

• Abdominal pain and swelling

• Difficulty breathing

• Swollen glands (lymph nodes)

• Unexplained weight loss

Resources

Link to Clinical Trials

Link to Scientific Publications

References

Genetic and Rare Disease Information Center (GARD)
NIH
WebMD
Healthline

Profile by: Emily Romanello
March 2024 Update by Lauren Young