Adult onset Still’s disease
Adult-onset Still's disease is an inflammatory condition characterized by high fevers, rash, sore throat, and joint pain. As it progresses, adult-onset Still's disease may lead to chronic arthritis and other complications. Still's disease was named after an English doctor named George Still, who described the condition in children in 1896. Still's disease which occurs in children (those under the age of 16) is now known as systemic onset juvenile rheumatoid arthritis (JRA). In 1971, the term "adult Still's disease" was used to describe adults who had a condition similar to systemic onset JRA. The cause of adult-onset Still's disease is unknown. No risk factors for the disease have been identified. There's no cure for adult-onset Still's disease; however, treatment may offer symptom relief and help prevent complications.
Common symptoms of AOSD include those on the following list. It is important to remember that there is no definite pattern that AOSD symptoms follow. In some cases, only one or two symptoms occur immediately; others may follow later.
- Aside from fever and joint pain, which are almost always among the first signs of AOSD, some of the symptoms on this list may not appear for weeks, or even at all. Fever The first sign that someone may be coming down with AOSD is a high spiking fever of 103 degrees or above. The fever usually rises rapidly in the evening and then subsides to or near normal by the next morning. Occasionally, an unusual pattern of two fever spikes in a day is seen in one-fifth of AOSD sufferers.
- Rash The typical Still's rash is salmon pink in color and comes and goes unpredictably, although it sometimes occurs at the same time as high fever. It is most common on the chest and thigh, although it may also appear on the face, hands and feet. In some cases, the rash becomes itchy. Joint and Muscle Pain At some point, every AOSD sufferer experiences intense joint pain, often in the hand. This can be accompanied by severe muscle pain as well. Both may become worse during a fever spike.
- Enlargement of the liver and spleen
- Swollen lymph nodes
- Increased white blood cell count in the blood
- Tests for rheumatoid factor and anti-nuclear antibodies are usually negative and serum ferritin is elevated
- Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph nodes, and less commonly fluid accumulation in the lungs and heart
- In rare cases, AOSD can cause aseptic meningitis and sensorineural hearing loss
The cause of adult-onset Still's disease is unknown. Some hypothesize that the condition results from or is triggered by a virus or other infectious agent. Others believe that it is a hypersensitive or autoimmune disorder. To date, no conclusive evidence has been found to prove or disprove either theory.
While there is no simple test for AOSD, most doctors will make the diagnosis if a person has five or more of the criteria below, including two or more from the first (major criteria) list:
Major AOSD Criteria:
- High fever lasting one week or longer
- Joint pain lasting two weeks or longer
- Abnormal white blood cell count and other blood problems
Minor AOSD Criteria:
- Sore throat
- Swelling of the lymph nodes or spleen
- Liver problems
- Absence of rheumatoid arthritis
The most widely used treatment for AOSD symptoms is non-steroidal anti-inflammatory drugs (NSAIDs). A good early indication of how well an AOSD sufferer will do in the long term is their initial response to these drugs. Statistically, 20-25% of AOSD sufferers improve with NSAIDs. Many of these find that their symptoms clear up quickly, sometimes for good. NSAIDs are normally taken until 1-3 months after the symptoms are gone.