7 Surprising Facts About Still's Disease

Doctor William C Lloyd Healthgrades Medical Reviewer
Medically Reviewed By William C. Lloyd III, MD, FACS
Written By Elizabeth Hanes, RN on June 22, 2021
  • Woman using computer
    Key Facts to Know About Still’s Disease
    If you’re living with diagnosed or suspected Still’s disease, you may wonder how this disorder will affect your general health and life expectancy going forward. Because this autoinflammatory disease is rare and not well studied, researchers lack the hard data to project with accuracy how the disease may progress in any individual. However, the best available statistics show that about two-thirds of people with Still’s disease will achieve remission, while one-third develop a chronic condition and fewer still experience life-threatening complications. Learn more Still’s disease facts to help you manage this condition.
  • woman standing along in pedestrian mall while blurred out people walk by
    1. Still’s disease in adults is very rare.
    The best available data show that adult-onset Still’s disease affects about 1 in 100,000 people in the United States each year, making it a very rare disease. By comparison, breast cancer affects around 1 in 8 women annually. Most estimates indicate Still’s disease affects more women than men, but the exact rates in each sex remain unclear. Because of the disease’s rarity, diagnosing Still’s disease can take a long time as doctors rule out more-common probable explanations for symptoms.
  • woman with rash on neck and chest
    2. Still’s disease is hard to diagnose.
    Part of the difficulty in diagnosing Still’s disease lies in the fact that its symptoms are largely nonspecific, which means they could indicate a wide variety of conditions. It’s true the condition often manifests with the Still’s disease rash—an area of salmon-pink inflammation around the trunk, arms or legs—but the other common Still’s disease symptoms of fever, sore throat, joint pain, and muscle pain all could signal some other condition, like an infection or even cancer. This non-specificity of symptoms makes Still’s disease hard to diagnose, since doctors must rule out other causes first.
  • man holding wrist in pain due to injury or arthritis
    3. Still’s disease is an autoinflammatory disorder, not an autoimmune disease.
    Researchers think Still’s disease may be triggered by an infection, but the root cause remains unknown. The disease is considered an arthritis, but as a systemic disease, it affects the entire body. Still’s disease is considered an “autoinflammatory” disorder, which means it occurs when the immune system erroneously stimulates inflammation throughout the body. This is different from an autoimmune disorder, in which the immune system mistakenly attacks healthy tissues. The question of why the body’s immune system triggers inflammation in people with Still’s disease remains a puzzle for researchers.
  • man pointing to picture of liver held up to his front torso
    4. Still’s disease affects internal organs.
    Although Still’s disease is classified as a type of arthritis, the condition also affects most of the internal organs, including the heart, lungs, liver and spleen. The excessive inflammation generated by Still’s disease can cause these organs to become enlarged, resulting in organ malfunction. In the case of the liver, Still’s disease often causes certain liver enzymes to become elevated— a data point used to achieve a diagnosis. Still’s disease treatment focuses on reducing systemic inflammation to help protect the organs as well as the joints.
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    5. Still’s disease complications can be life threatening.
    For approximately two-thirds of people who develop Still’s disease, the condition goes into remission after one episode or several cyclical episodes over the course of a few years. But the remaining one-third of people diagnosed with Still’s develop a chronic form of the disorder and can face a life-threatening complication called macrophage activation syndrome (MAS). MAS causes an extreme proliferation of macrophages (a type of large immune system cell) that overrun the body and can result in death. For most people, life expectancy in Still’s disease is normal, but for a few people the condition can be life-limiting.
  • Hispanic man examining prescription bottle in kitchen
    6. Still’s disease treatment focuses on inflammation.
    For most people diagnosed with Still’s disease, anti-inflammatory agents of all kinds form the cornerstone of the treatment plan. Treating Still’s disease may involve taking over-the-counter nonsteroidal anti-inflammatory (NSAID) medications, oral corticosteroids like prednisone, or antirheumatic drugs commonly used to treat autoimmune disorders like rheumatoid arthritis. Newer biologic medications may be helpful in treating severe or resistant cases of Still’s disease. All of these medications help keep inflammation levels down to relieve symptoms and prevent organ or joint damage.
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    7. You can live well with Still’s disease.
    Even if Still’s disease turns chronic, you can live a healthy, active lifestyle. In fact, engaging in healthy habits like quitting smoking; exercising regularly; and adopting an eating pattern that emphasizes fresh vegetables and fruits, lean proteins, and whole grains can help keep your heart and other organs in good shape despite the inflammation of Still’s disease. Taking a holistic approach to your wellness may help you achieve remission from Still’s disease and go on to live a long, healthy life.
Still's Disease Facts | 7 Surprising Facts About Still's Disease

About The Author

As “the nurse who knows content,” Elizabeth Hanes, RN, works with national and regional healthcare systems, brands, agencies and publishers to produce all types of consumer-facing content. Formerly a perioperative and cosmetic surgery nurse, Elizabeth today uses her nursing knowledge to inform her writing on a wide variety of medical, health and wellness topics.
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  3. Juvenile Arthritis. Medline Plus, U.S. National Library of Medicine. https://medlineplus.gov/juvenilearthritis.html
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  5. Adult Onset Still’s Disease. U.S. National Organization for Rare Disorders. https://rarediseases.org/rare-diseases/adult-onset-stills-disease/
  6. Ruscitti P, Cipriani P, et al. Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Med. 2016;14:194. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5131497/
  7. U.S. Breast Cancer Statistics. BreastCancer.org. https://www.breastcancer.org/symptoms/understand_bc/statistics
  8. Rosario C, Zandman-Goddard G, et al. The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med. 2013;11:185. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3751883/

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Last Review Date: 2021 Jun 22
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