| Adult-onset Still's disease | |
|---|---|
| Classification and external resources | |
| ICD-10 | M06.1 |
| ICD-9 | 714.2 |
| DiseasesDB | 34295 |
| MedlinePlus | 000450 |
| MeSH | D016706 |
Adult-onset Still's disease is a rare form of inflammatory arthritis that was characterized by EG Bywaters in 1971.[1] The underlying cause is unknown. It usually presents with high spiking fevers, joint and muscle pains, a salmon colored rash and other symptoms of systemic inflammation.
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Still's disease is named after English physician Sir George Frederic Still (1861–1941).[2][3]
Adult-onset Still's Disease is rare and has been described all over the world. Prevalence is estimated at 1.5 cases per 100,000-1,000,000 population. There is a bimodal age distribution with one peak incidence between ages 15–25 and a second peak between ages of 36–46 years.[4]
The disease typically affects 16–35 year olds and presents with arthralgia, elevated serum ferritin, a 'salmon-pink' rash, pyrexia and lymphadenopathy. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph glands, and less commonly fluid accumulation in the lungs and heart.
The diagnosis is clinical, not based upon serology.[5] At least seven sets of diagnostic criteria have been devised, however the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.[6]
| Major criteria | Minor criteria |
|---|---|
| Fever of at least 39C for at least one week | Sore throat |
| Arthralgias or arthritis for at least two weeks | Lymphadenopathy |
| Nonpruritic salmon colored rash (usually over trunk or extremities while febrile) | Hepatomegaly or splenomegaly |
| Leukocytosis ( 10,000/microL or greater), with granulocyte predominance | Abnormal liver function tests |
| Negative tests for antinuclear antibody and rheumatoid factor |
Treatment for adult-onset Still's disease is with anti-inflammatory drugs. Steroids such as prednisone are used to treat severe symptoms of Still's. Other medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, cyclophosphamide, adalimumab, rituximab, and infliximab.
Adult-onset Still's disease is chronic; and reports of remission is either slim or temporary at best.
The condition "juvenile-onset Still disease" is now usually grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.[7]
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