Adult-onset Still’s disease 

Still’s disease is a rare systemic inflammatory autoimmune disorder. Patients with this condition may develop recurring high fevers, joint pain, skin rashes (which may come and go with the fever), sore throat and generalised symptoms such as fatigue and loss of appetite. 
There are 2 forms of the condition, which have similar presentations:
- Adult-onset still’s disease (AOSD) which occurs in adulthood
- Systemic Juvenile Idiopathic Arthritis (sJIA) which begins in childhood

Still’s disease is an autoimmune disase where your own immune system attacks your own tissues in the body damaging various parts of the body. Genetic predispositions and environmental triggers such as infections may play a role in development of the condition. However, the exact cause is unknown. 

Symptoms may vary depending on the organ systems involved and may appear similar to that of an infection. Common symptoms include:
- Characteristic daily high spiking fevers, which lasts a few hours and improves but recurs (quotidian fever)
- Rashes over the body which appears salmon-pink and often appears during the fever (evanescent rash)
- Joint pain, swelling, and early morning stiffness
- Sore throat
- Fatigue
- Loss of weight or loss of appetite
In severe cases, still’s disease may be associated with a life-threatening complication known as macrophage activation syndrome which causes overactivation of the immune system, multi-organ damage and rapid clinical deterioration.

Still’s disease is rare and diagnosis can be challenging. There is no specific test to diagnose this condition, and symptoms may mimic other diseases such as infections and cancers. A comprehensive assessment will usually involve: 
• Detailed history taking and physical examination
• Laboratory tests including blood counts, inflammatory markers which are often raised (such as ferritin, Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)), and liver function tests showing raised liver enzymes. Other tests such as infection screens are usually performed to exclude infections.
• Imaging: CT or PET-CT scans may be performed to look for other causes of fever. Abnormalities found on scans such as swollen lymph nodes may need to be biopsied to exclude cancers and infections which can also cause similar symptoms.
• Additional tests may be done depending on the suspected organ involvement and to exclude other conditions.

Still’s disease can be effectively treated with medications. Prompt treatment should be initiated upon diagnosis to reduce inflammation and avoid long-term complications. Treatment varies depending on the severity of disease, but often involves medications to suppress the inflammation and calm down the immune system. 
- Nonsteroidal anti-inflammatory drugs (NSAIDs) (such as ibuprofen, diclofenac, naproxen, etoricoxib, celecoxib etc.) may be given for mild disease. These medications do not suppress the immune system but are effective in reducing inflammation in mild disease.
- Corticosteroids are usually required in moderate to severe disease, or if symptoms do not respond to NSAIDs. These are fast-acting medications that effectively suppress the immune system. The dosing differs depending on the severity of the disease. In mild-moderate disease, oral steroids are given. In severe or life-threatening disease, steroids are usually started at high doses given as intravenous injections through the veins, then reduced over time.
- Disease-modifying anti-rheumatic drugs (DMARDs) and immunosuppressive medications may be given in patients to aid in reducing steroid doses and are often continued long-term to suppress inflammation. Examples include methotrexate, azathioprine, ciclosporin and leflunomide. 
- Biologic medications are more targeted injection medications given subcutaneously (under the skin in the abdomen or thighs) or intravenously through the veins. These may be given for patients with more severe inflammation or for those who do not respond adequately to the above treatment. Examples include infliximab, adalimumab, tocilizumab and anakinra.
As multiple organ systems may be affected in still’s disease, patients are often cared for by a multidisciplinary team of medical professionals, including a primary rheumatologist and other relevant subspecialty teams and allied health professionals. Your treatment plan will be carefully tailored to your symptoms, preferences, and goals of care. 

Additionally, to prevent infections when on high doses of immune suppression medications, prophylactic antibiotics and certain vaccinations are encouraged (e.g. influenza, pneumococcal, COVID-19, respiratory syncytial virus and shingles). Other co-existing medical conditions such as osteoporosis, high cholesterol, diabetes mellitus and high blood pressure will also be addressed as part of holistic care.

Patients with still’s disease are able to live active lives with the right treatment and support. Some recommended lifestyle changes include:
• Eat a balanced diet. Avoid raw and partially cooked food to reduce risks of infections, especially if you are on medications that suppress the immune system (e.g. steroids).
• Avoid smoking.
• Stay active and exercise in moderation. Ensure sufficient rest and sleep well.
• Attend regular medical check-ups to monitor your health. Participate actively in your care and ask questions during your clinic consultations.
• Take your medications as prescribed, even when you feel well, to avoid disease flares.
• Seek medical attention early if you suspect that you have a disease flare.

Seek a doctor's advice. You will be referred to a specialist (rheumatologist) who can confirm the diagnosis and start you on the appropriate treatment. Once diagnosed, it is important to start treatment early and follow your rheumatologist’s advice. This condition is managed by the Division of Rheumatology and Allergy.