Fever of Unknown Origin and Adult Onset Still’s Disease (AOSD)

Posted by e-Medical PPT Sunday, July 31, 2011
Adult-onset Still's disease is a rare form of inflammatory arthritis.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.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.
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.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.

Major criteria
Fever of at least 39C for at least one week   
Arthralgias or arthritis for at least two weeks   
Nonpruritic salmon colored rash (usually over trunk or extremities while febrile)   
Leukocytosis ( 10,000/microL or greater), with granulocyte predominance   

Minor criteria
Sore throat
Lymphadenopathy
Hepatomegaly or splenomegaly
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 ect.

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