What is Still’s?
Still's Disease is a rare illness where the body’s immune system starts attacking itself, causing inflammation in various parts of the body. Still’s can be serious and affect many organs.
In 2025 EULAR (The European Alliance of Associations for Rheumatology) officially recognised Adult-Onset Stills Disease (AOSD) and Systemic Juvenile Idiopathic Arthritis (sJIA) as the same condition. Previously they were divided by age and are now collectively known as Still’s Disease. Other forms of JIA (juvenile idiopathic arthritis) exist besides sJIA, though they are not the same condition.
Who does Still’s affect?
- Still’s affects children and often has peak times of occurrence in two age groups: young adults aged 16–25, and again in people aged 36–46.
- It affects women more often than men.
What causes Still’s Disease?
Doctors don’t know exactly what causes Still’s Disease Research is still ongoing to find out if specific genes or environmental factors play a role. AOSD has some symptoms like those seen in sJIA, especially the daily fevers, arthritis, and pink rash.
Still’s Disease has many symptoms, including:
- High fevers: These happen every day and are usually higher in the evening, sometimes reaching 39°C or more.
- Rash: Salmon-pink rash: This rash appears in many cases, usually on the neck, trunk, and limbs. It’s often non-itchy and disappears quickly. AND/OR Hives: Also known as Urticaria. It is a rash that is raised and itchy can occur
- High Inflammatory Markers: Blood tests will show very high levels for C-reactive protein (CRP), Erythrocyte Sedimentation Rate (ESR) and Serum Amyloid A (SAA)
- Joint pain and swelling: The knees and wrists are the most common areas affected. If untreated, it can lead to joint damage.
- Fatigue: Many people with Still’s Disease feel extremely tired, especially when they have a fever or rash.
- Organ issues: Still’s Disease can affect the heart, lungs, liver, and spleen. You may experience chest pain, trouble breathing, abdominal pain, or liver problems.
- Lymph nodes and spleen swelling: Swollen lymph nodes and an enlarged spleen are common.
- Anaemia: Low red blood cell count can cause fatigue and weakness.
How Still’s Disease behaves
Still’s Disease may follow one of these patterns:
- Monophasic: One episode that lasts a few weeks or months.
- Polyphasic: Multiple episodes with periods of no symptoms in between.
- Chronic: Ongoing symptoms over time.
How is Still’s Disease diagnosed?
Diagnosis can be tricky because there’s no single test that can confirm it. Doctors rely on a combination of:
- A detailed history and symptoms
- Excluding other conditions (using tests like blood tests and imaging)
Doctors may use guidelines called the Yamaguchi or Fautrel Criteria to help make the diagnosis.
Some common tests include:
- Blood tests such as
- C-reactive protein (CRP) and Erythrocyte Sedimentation Rate (ESR) and Serum Amyloid A (SAA) to check for inflammation
- Liver Function Test (LFT) to check on the liver
- Ferritin: A test to see if there’s a lot of iron in the blood, which can be high in Still’s Disease
- Full Blood counts (FBC) to check for signs of infection or anaemia
- Albumin: AOSD can cause low albumin (hypoalbuminemia)
- ANA & rheumatoid factor (RF) to rule out other rheumatic diseases
- interleukin IL-1, IL-6, IL-18, TNF Inflammatory cytokines. These tests are routinely available in Australia and New Zealand. Currently these are only done in specialised research laboratories.
- Imaging: Scans to check for changes in bones, joints, and organ size, like the spleen or liver
- Heart tests: An ECG (echocardiogram) to monitor the heart
In Australia and New Zealand some of these blood tests can be difficult to arrange and there is a cost involved. The cost varies between pathology labs and where you live.
Why is early treatment important?
Getting a diagnosis and starting treatment early is important to stop serious complications from happening, such as:
- Amyloidosis: A rare condition where abnormal proteins build up in organs, which can affect how they work.
- Heart problems: Inflammation around the heart can cause chest pain and other heart issues.
- Macrophage Activation Syndrome (MAS): This is a dangerous complication where the immune system goes into overdrive, causing inflammation, fever, organ problems, and low blood counts.
There is no cure for Still’s Disease, but there are treatments that can help manage the symptoms. The EULAR recommendations state that ultimate aim of treatment is drug free remission.
While there’s no cure, The aim of treatment is to completely control the symptoms to prevent long term damage to the body. Long term treatment is often needed. The length of treatment depends on progress of symptoms and assessment to check if the condition remains active.
- NSAIDs (Non-steroidal Anti-inflammatory Drugs): These help reduce pain, inflammation, and fever. Examples include Ibuprofen, Naproxen and Aspirin.
- Steroids: Prednisolone is a common treatment, but it’s used for short periods due to side effects.
- Biologics: The new EULAR recommendations suggest using biologic as first line treatment with steroids. They are designed to block specific chemicals that play important roles in immune system overactivation. Examples are: IL-1 inhibitors Anakinra and Canakinumab, IL-6 inhibitor Tocilizumab
Biologics are often expensive and not always easy to get.
- TNF (Tumour Necrosis Factor) inhibitors such as Infliximab, Etanercept, and Adalimumab. These are typically used in the later stages of disease for Chronic inflammatory arthritis issues.
- DMARDs (Disease-Modifying Anti-Rheumatic Drugs): These medications slow down the immune system to prevent further damage. Methotrexate, Leflunomide, Hydroxychloroquine, Sulfasalazine Hydroxychloroquine and Azathioprine are examples.
Other helpful treatments:
- Physiotherapy and occupational therapy may help you cope with the physical effects of Still’s disease and improve mobility.
- Besides medication, lifestyle changes can help people with Still’s disease to manage their condition.
IMPORTANT NOTE:
Currently, not all medications commonly used to treat SAIDs are available in Australia and New Zealand, and those available may be difficult to access.
ANZFAID is committed to continuing to advocate for improved options, and timely and affordable access to treatment.
Still's Disease is a serious condition, but with the right treatment and care, many people manage to live well with it.
Helpful info:
- Article - Adult Onset Still's Disease: A Review on Diagnostic Workup and Treatment Options
- Personal Story - Read Tim's story
- Recommendations for Still's Disease
- The EULAR points to consider regarding the development of criteria for the assessment of the disease activity in adult-onset Still’s disease - EULAR Rheumatology Open
