Overview:
Adult Still’s disease or adult-onset Still’s disease, as its name indicates, occurs in adults and is the severe form of juvenile idiopathic arthritis affecting children. While Still’s disease is systemic juvenile idiopathic arthritis, adult Still’s disease is the adult and severe version of the former.
What Is Adult Stills Disease?
Adult Still's disease is an inflammatory condition of autoimmune origin, meaning the body’s own immune system causes the condition by mistake. This condition causes joint pain and stiffness, including inflammation in other bodily tissues as well. The signs and symptoms of adult Still’s disease can either follow periods of exacerbation and remission, persist continuously, or vanish and never occur again for a long period. If flare-ups are present, these can either occur once or multiple times within a short (one month) or long duration (one year).
What Are the Clinical Features of Adult Stills Disease?
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Fever - The most common clinical feature of this condition is fever. This fever lasts for several days and peaks every day at the same time. This peak can occur twice a day in some individuals.
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Rash - Still’s disease also presents with a rash that is similar to hives but not itchy. These rashes are salmon pink in color. These rashes mostly develop on the chest, thighs, arms, legs, and face; however, they disappear quickly.
Other symptoms include -
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Sore throat.
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Joint pain and swelling (arthritis), especially of the knee, wrist, ankle, hands, hips, shoulder, elbows, and jaw joints.
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Unintentional weight loss.
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Abdominal pain.
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Muscle pain.
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Pain while breathing deep (pleurisy).
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Lymph node enlargement.
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Spleen and liver enlargement (rarely).
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Inflammation of the lung and heart (rarely).
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Exhaustion, tiredness, or fatigue that does not get better with adequate rest and sleep.
What Causes Adult-Onset Stills Disease?
The disease occurs due to the body’s immune system attacking its own tissues mistaking them to be foreign bodies. But the exact reason why this autoimmunity occurs is still not known. Researchers believe some bacterial or viral infection could be the probable trigger. A combination of environmental exposures, genetic factors, and abnormal reactions to infections contribute to Adult Still’s disease.
Also, specialized proteins called cytokines that mediate the inflammatory reactions are known to play some role in the development of adult-onset Still’s disease. Such cytokines are interleukin-1 (IL-1), interleukin-6 (IL-6), interleukin-18 (IL-18), and tumor necrosis factor-alpha (TNF-alpha).
How Common Is Adult-Onset Stills Disease?
The condition is very rare, with an incidence of less than 1 out of every 100,000 people every year. Though males and females have equal incidence rates, women have a slightly higher predilection than men. People aged between 15 to 25 years and 36 to 46 years are more likely to develop the condition. The disorder is not hereditary, meaning it does not run in families.
Is Adult Stills Disease Permanent?
It is said that the disease follows any one of the three stages in different people. In one-third of the people with the condition, the signs and symptoms constantly prevail for a year before they ease. In the next third of the people, exacerbation of the existing symptoms or flare-ups develops before the symptoms ease with treatment. In the final third of the affected people, their symptoms do not ease even after a year, and they continue lifelong with occasional flare-ups. In such conditions, if their joints remain affected persistently, the damage can become permanent and cannot be treated with drugs. There are also some people who live a normal life with the help of drugs.
What Are the Complications of Adult Stills Disease?
- Fluid Build-up Around the Lungs - Inflammation around the lung tissues can cause excessive fluid to accumulate around the lungs, making it difficult to breathe. It can also cause pain during deep breathing.
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Heart Inflammation - The inflammation can involve the layer that covers the heart (pericardium) and cause pericarditis or involves the heart muscle (myocardium) and causes myocarditis.
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Macrophage Activation Syndrome - This possible but rare complication is potentially fatal. This is an abnormal and overactive response of the immune system. Its clinical features are abnormal liver function, high triglyceride levels, and low blood cell count.
How Is Adult Stills Disease Diagnosed?
Diagnosis of this condition involves ruling out other conditions with similar clinical features (diagnosis of exclusion). A thorough clinical evaluation and detailed patient history, along with the exclusion of other possible disorders, help diagnose the disorder.
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Echocardiogram - Echocardiogram helps identify any inflammation of the pericardium or myocardium.
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Blood Tests - This helps reveal elevated levels of white blood cells (leukocytosis), platelets (thrombocytosis), low levels of red blood cells (anemia), elevated inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), elevated serum ferritin, high blood levels of liver enzymes, namely alanine aminotransferase (ALT), aspartate aminotransferase (AST) and lactate dehydrogenase (LDH).
What Does Yamaguchi Criteria Mean?
This criterion includes certain parametric levels for adult-onset Still’s disease to be diagnosed. The fulfillment of 5 criteria, including two major criteria, decides the presence of adult Still’s disease. These parameters are;
Major Criteria -
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Fever of at least 102.2 degrees Fahrenheit or 39 degrees Celsius lasting at least a week.
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Joint pain or arthritis lasting for at least two weeks.
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Presence of salmon-colored rash.
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Elevated white blood cell count.
Minor Criteria -
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Sore throat.
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Lymph node enlargement.
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Liver or spleen enlargement.
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Elevated liver enzymes.
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Test negative for antinuclear antibodies and rheumatoid factor.
How Is Adult Stills Disease Treated?
Either taken alone or in combination, the following medications help subside the symptoms and reduce their severity.
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NSAIDs (Non-Steroidal Anti-Inflammatory Drugs) - Drugs belonging to this group such as Ibuprofen, Naproxen, Indomethacin, and Diclofenac.
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Steroids - Systemic inflammation can be treated with steroids like Prednisone.
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Methotrexate - This is an immunosuppressive drug that, when used in combination with steroids, helps use steroids at a reduced dosage, thereby managing systemic symptoms.
What Lifestyle Changes Should People With Adult-Onset Stills Disease Implement?
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People under steroid treatment for adult Still’s disease should check with their doctor and consume calcium and vitamin D supplements due to the risk of osteoporosis.
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Regular exercising is important. It might cause joint pain, but regular exercise improves joint flexibility and range of motion and is good for people with arthritis.
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Continue your medications regardless of the presence or absence of symptoms.
Conclusion:
Though adult still’s disease is not permanently curable, it is treatable with the help of medications. It is a rare condition, and if at all you know someone with the condition, keep them informed that it is possible to live a near to normal life.
