Still’s Disease: Symptoms, Causes, Treatment

What are the symptoms of Still’s disease?

The symptoms of Adult-Onset Still’s Disease (AOSD) can vary but typically include a combination of systemic and inflammatory signs. The most common symptoms are:

1. Fever:

Daily or bi-daily spiking fever (often above 102°F or 39°C), usually occurring at the same time each day, typically in the afternoon or evening.
Fever spikes often return to normal or subnormal levels between episodes.

2. Salmon-colored Rash:

A faint, salmon-pink rash that often appears during fever spikes.
The rash usually appears on the trunk, arms, or legs and is often non-itchy.
It may be triggered by heat or friction (e.g., rubbing the skin).

3. Joint Pain and Swelling (Arthritis):

Severe joint pain and inflammation, typically affecting the wrists, knees, ankles, and shoulders.
Joint symptoms can lead to chronic arthritis in some cases.

4. Muscle Pain:

Myalgia or generalized muscle pain, often worse during fever spikes.

5. Sore Throat:

A persistent or recurring sore throat is common, especially during the onset of the disease.

6. Fatigue:

Severe fatigue and tiredness are frequent, often worsened by fever and pain.

7. Enlarged Lymph Nodes, Liver, or Spleen:

In some cases, lymphadenopathy (swollen lymph nodes), hepatomegaly (enlarged liver), or splenomegaly (enlarged spleen) may occur.

8. Weight Loss:

Unintentional weight loss due to the systemic nature of the disease.

9. Pleural and Pericardial Inflammation:

Pleuritis (inflammation of the lining of the lungs) can cause chest pain, especially when breathing deeply.
Pericarditis (inflammation of the sac around the heart) can also occur, causing chest discomfort.

10. Elevated Inflammatory Markers:

Blood tests often show high levels of inflammation, including elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and elevated white blood cell count.

The symptoms can come and go, with periods of remission and flare-ups. In some cases, AOSD can lead to complications such as chronic arthritis, lung or heart inflammation, and macrophage activation syndrome (MAS), a potentially life-threatening complication. Treatment typically involves managing inflammation with medications such as NSAIDs, corticosteroids, or immunosuppressants.

What are the causes of Still’s disease?

The exact cause of Adult-Onset Still’s Disease (AOSD) is unknown, but several factors may contribute to its development:

Immune system dysregulation: An abnormal immune response is thought to play a key role, where the body mistakenly attacks its own tissues.
Genetic predisposition: Certain genetic factors may increase the risk of developing the disease.
Infections: Viral or bacterial infections may trigger the disease in individuals with a predisposition. Potential triggers include viruses like Epstein-Barr and cytomegalovirus.
Environmental factors: Exposure to certain environmental factors may influence disease onset in genetically susceptible individuals.

Research continues to explore these contributing factors.

What is the treatment for Still’s disease?

Treatment for Adult-Onset Still’s Disease (AOSD) focuses on reducing inflammation, managing symptoms, and preventing complications. Common treatment options include:

Nonsteroidal anti-inflammatory drugs (NSAIDs): Help reduce pain and inflammation, especially during flare-ups.
Corticosteroids: Prednisone or other steroids are often used to control inflammation, especially in severe cases.
Disease-modifying antirheumatic drugs (DMARDs): Medications like methotrexate can be prescribed to control the immune system and reduce long-term damage.
Biologic agents: Targeted therapies such as tumor necrosis factor (TNF) inhibitors, interleukin-1 (IL-1) inhibitors, or interleukin-6 (IL-6) inhibitors may be used to control inflammation.
Physical therapy: Helps maintain joint function and mobility, especially in cases with joint damage.
Antibiotics or antivirals (if applicable): To treat infections that may trigger or exacerbate symptoms.

Treatment plans are often individualized based on symptom severity and response to therapy. Regular monitoring by a healthcare provider is essential.