Adult Onset Stills Disease

 

What is Adult Onset Stills Disease? 

Adult onset stills disease (AOSD) is a rare acquired autoinflammatory disease. It is characterised by fever and signs and symptoms of inflammation in the body that are not due to infection or autoimmune causes. They are acquired, meaning they are not passed down in families in genes. The disease affects adults, but a similar presentation occurs in children, this is called systemic juvenile idiopathic arthritis (SJIA).

The course of the disease is variable, some people experience one single episode of inflammation, and others get chronic episodic inflammation. AOSD occurs equally in males and females and usually occurs around the age of 16-40.

 

Causes of Adult Onset Stills Disease

Adult onset still disease is an autoinflammatory disease. The immune system usually produces inflammation in response to trauma and infection. In the case of AOSD, unregulated activation of inflammation occurs which damages the body. The disease is not autoimmune, meaning that there are no proteins called antibodies produced, the immune system reaction that is triggered is more primitive and innate.

The exact cause of the over activation of inflammation is still unknown, although there is a suggestion of infections triggering the disease.

 

Symptoms of Adult Onset Stills Disease

Common symptoms:

 

Rare complications:

 

How do we diagnose Adult Onset Stills Disease? 

A thorough history and examination is carried out by your doctor, AOSD is diagnosed when other causes are excluded. Other investigations are listed below:

 

Treatments

Management is based on the severity of symptoms; the main treatments from first line are listed below:

 

Blood markers for inflammation including ferritin and blood cell counts measure the efficacy of treatment.

 

AOSD usually follows one of 3 patterns:

 

Useful Links

Versus Arthritis:  http://www.versusarthritis.org

NRAS: https://www.nras.org.uk/what-is-adult-onset-stills-disease-aosd-