Adult-onset Still's Disease

Adult-onset Still's disease (AOSD) is a rare and complex autoimmune disorder that affects adults, typically between the ages of 20 and 40 years old. It is characterized by high fevers, joint pain and swelling, and rash.

Causes and Risk Factors

The exact cause of AOSD is unknown, but it is believed to be related to an abnormal immune response, which leads to inflammation in various parts of the body. Some potential triggers include viral or bacterial infections, genetic predisposition, and environmental factors.

Symptoms

The symptoms of AOSD can vary from person to person, but common features include:

• High fevers, often exceeding 39°C (102°F)
• Joint pain and swelling, particularly in the wrists, knees, and ankles
• Rash, typically a salmon-pink color, which can appear on the trunk, arms, and legs
• Muscle pain and weakness
• Sore throat and swollen lymph nodes
• Weight loss and fatigue

Diagnosis

Diagnosing AOSD can be challenging, as the symptoms are similar to those of other conditions, such as rheumatoid arthritis, lupus, and infections. A combination of physical examination, laboratory tests, and imaging studies is used to confirm the diagnosis.

• Blood tests to check for elevated levels of inflammatory markers, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
• Complete blood count (CBC) to rule out infections or other conditions
• Imaging studies, such as X-rays or magnetic resonance imaging (MRI), to assess joint damage
• Bone marrow biopsy to exclude other conditions, such as lymphoma

Treatment

Treatment for AOSD typically involves a combination of medications to reduce inflammation, manage symptoms, and prevent complications.

• Nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce fever and joint pain
• Corticosteroids to suppress the immune system and reduce inflammation
• Disease-modifying antirheumatic drugs (DMARDs), such as methotrexate or azathioprine, to slow disease progression
• Biologic agents, such as tumor necrosis factor-alpha (TNF-alpha) inhibitors, to target specific components of the immune system

Complications and Prognosis

If left untreated or undertreated, AOSD can lead to serious complications, including:

• Joint damage and deformity
• Organ damage, such as kidney or liver failure
• Infections, particularly in people taking immunosuppressive medications
• Mental health issues, such as depression and anxiety

The prognosis for AOSD varies depending on the severity of symptoms, response to treatment, and presence of complications. With prompt and effective treatment, many people with AOSD can achieve remission or significant improvement in their symptoms.

Frequently Asked Questions (FAQs)

What is Adult-onset Still's disease?
A rare inflammatory disorder characterized by high fevers, joint pain, and rash.

What are the typical symptoms of Adult-onset Still's disease?
High spiking fevers, joint pain and swelling, rash, sore throat, and swollen lymph nodes.

What is the cause of Adult-onset Still's disease?
The exact cause is unknown, but it is thought to be an autoimmune disorder.

How is Adult-onset Still's disease diagnosed?
Diagnosis is based on a combination of clinical presentation, laboratory tests, and exclusion of other conditions.

What laboratory tests are used to diagnose Adult-onset Still's disease?
Tests may include complete blood count, erythrocyte sedimentation rate, C-reactive protein, and ferritin levels.

Is Adult-onset Still's disease contagious?
No, it is not contagious.

Can Adult-onset Still's disease be cured?
There is no known cure, but symptoms can be managed with treatment.

What treatments are available for Adult-onset Still's disease?
Treatments may include nonsteroidal anti-inflammatory drugs, corticosteroids, and immunosuppressive medications.

Can Adult-onset Still's disease affect other parts of the body?
Yes, it can affect organs such as the liver, spleen, and lungs in some cases.

Is Adult-onset Still's disease related to juvenile rheumatoid arthritis?
It is sometimes considered the adult equivalent of systemic juvenile idiopathic arthritis, also known as Still's disease.