Adult onset Still's disease (AOSD) is a rare inflammatory disorder that affects the entire body (systemic disease). The cause of the disorder is unknown (idiopathic). Affected individuals may develop episodes of high, spiking fevers, a pink or salmon colored rash, joint pain, muscle pain, a sore throat and other symptoms associated with systemic inflammatory disease. The specific symptoms and frequency of episodes vary from one person to another and the progression of the disorder is difficult to predict. In some individuals, the disorder appears suddenly, disappears almost as quickly and may not return. In other people, adult onset Still's disease is a chronic, potentially disabling, condition. Various medications are used to treat individuals with adult onset Still's disease, affected individuals may respond to therapy differently. Adult onset Still's disease does not appear to run in families. Adult onset Still's disease is the adult form of systemic juvenile rheumatoid arthritis (juvenile Still's disease). The disorders are name after a British physician who first described systemic juvenile rheumatoid arthritis in the medical literature in 1896. The term "adult Still's disease" was first used in the medial literature in 1971, but cases that fit the description of the disorder appear in the medical literature as early as the late 1800s.
The symptoms, progression and severity of adult onset Still’s disease are highly variable from one person to another. Some individuals may only develop one random episode that responds to treatment and resolves within one year. In some cases, a new episode does not occur or does not occur until many years later. Other individuals may develop chronic disease, in which episodes come and go, often years apart and with no symptoms in-between episodes. Still other individuals may experience frequent episodes that occur every several weeks or months. Individuals with chronic adult onset Still’s disease may have a form predominantly characterized by fever or a form predominantly characterized by joint disease (chronic arthritis). Chronic adult onset Still’s disease can potentially cause long-term, severe and disabling complications.
Most individuals with adult onset Still’s disease develop some combination of the symptoms normally associated with systemic inflammatory disease. Such symptoms include a spiking fever greater than 102.2 degrees Fahrenheit (39 degrees Celsius), joint pain (arthralgia) and inflammation (arthritis), muscle pain (myalgia), and a skin rash.
In some cases, fevers are a daily occurrence and usually peak or spike in the late afternoon or early evening. In rare cases, some individuals develop two spiking fevers in one day. The rash is pink or salmon colored, and usually develops during a fever episode. The chest and thighs are most often affected by the rash. The arms, legs and face are affected less often. The rash may or may not be itchy and tends to disappear quickly (evanescent).
Affected joints may become swollen, stiff and inflamed and may persist for a couple of weeks. The knees, wrists and ankles are most commonly affected. Muscle and joint pain can be intense and is often worse during a fever episode. If adult onset Still’s disease goes untreated, chronic inflammation of the joints can potentially result in deterioration and destruction of the affected joints.
Additional findings may occur in some cases including a sore throat, enlargement of the spleen (splenomegaly), enlargement of the liver (hepatomegaly), and enlargement of the lymph nodes (lymphadenopathy).
In some cases, the thin, sac-like membrane that surrounds the heart (pericardium) or the heart muscle (myocardium) may become inflamed (pericarditis or myocarditis). The membrane lining the chest cavity may also become inflamed and may cause fluid to accumulate in the lungs (pleural effusion). Heart and lung involvement can cause difficulty breathing and chest pain, but in most cases it is usually not severe enough to be readily apparent and is often only detected during a physical examination by a physician.
Most people with adult Still's disease have a combination of the following signs and symptoms:
- Fever. You might have a daily fever of at least 102 F (38.9 C) for a week or longer. The fever usually peaks in the late afternoon or early evening. You might have two fever spikes daily, with your temperature returning to normal in between.
- Rash. A salmon-pink rash might come and go with the fever. The rash usually appears on your trunk, arms or legs.
- Sore throat. This is one of the first symptoms of adult Still's disease. The lymph nodes in your neck might be swollen and tender.
- Achy and swollen joints. Your joints — especially your knees and wrists — might be stiff, painful and inflamed. Ankles, elbows, hands and shoulders might also ache. The joint discomfort usually lasts at least two weeks.
- Muscle pain. Muscular pain usually ebbs and flows with the fever, but the pain can be severe enough to disrupt your daily activities.
The signs and symptoms of this disorder can mimic those of other conditions, including lupus and a type of cancer called lymphoma.
- General Practitioner
There is no single test that can diagnose adult Still’s disease. Instead, blood tests are used to rule out other diseases with similar symptoms.
- Non-steroidal Anti-Inflammatory drugs (NSAIDs)
- Disease-modifying drugs (DMARDs), such as methotrexate, and biologics, are needed in more severe cases or if the arthritis becomes chronic. It may be necessary to take more than one medication at a time to control symptoms
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