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Adult Still’s disease

Adult Still's Disease (ASD) is a rare type of inflammatory arthritis that affects adults, although it is more commonly diagnosed in children (as Systemic Juvenile Idiopathic Arthritis or Still's Disease). It is characterized by systemic inflammation that can affect multiple organs, and it often presents with fever, skin rashes, and joint pain. The exact cause of the disease is not fully understood, but it is thought to be related to an abnormal immune response that leads to inflammation throughout the body.

Adult Still's Disease (ASD) is a rare type of inflammatory arthritis that affects adults, although it is more commonly diagnosed in children (as Systemic Juvenile Idiopathic Arthritis or Still's Disease). It is characterized by systemic inflammation that can affect multiple organs, and it often presents with fever, skin rashes, and joint pain. The exact cause of the disease is not fully understood, but it is thought to be related to an abnormal immune response that leads to inflammation throughout the body.

Key Features of Adult Still's Disease:

  1. Fever: A hallmark symptom of ASD is a daily high spiking fever, typically occurring in the late afternoon or evening. The fever often peaks at around 39°C (102°F) or higher.
  2. Rash: A salmon-colored, bumpy, or maculopapular (flat or raised) rash may appear during fever episodes, often on the trunk, arms, and legs. The rash tends to appear and disappear with the fever spikes.
  3. Joint Pain (Arthritis): Many individuals with ASD experience joint inflammation, particularly in the wrists, knees, and ankles. The arthritis can be quite painful and debilitating.
  4. Systemic Involvement: In addition to joint inflammation, ASD may affect other organs, including the liver, spleen, and lymph nodes. In severe cases, it can also affect the heart, lungs, and kidneys.
  5. Myalgias: Muscle pain (myalgia) is another common symptom of ASD.

Symptoms and Course:

  • Fever: The fever typically occurs in a pattern, often spiking at the same time every day.
  • Rash: The rash can appear during the fever peaks and usually fades as the fever subsides.
  • Arthritis: Joint pain and swelling, especially in the large joints, are common and may be transient (appearing and disappearing) or persist over time.
  • Fatigue: Patients may experience extreme tiredness and general discomfort.

In some cases, ASD may involve complications like liver dysfunction, respiratory problems, and inflammation of the heart muscle (myocarditis). This disease can also cause long-term joint damage and disability if not treated appropriately.

Causes and Risk Factors:

The exact cause of Adult Still’s disease is not fully understood, but it is believed to involve:

  1. Immune System Dysfunction: Still’s disease is considered an autoimmune disease, where the immune system attacks the body's own tissues, causing inflammation.
  2. Genetic Factors: There may be a genetic predisposition that increases the risk of developing the disease.
  3. Infections: Some researchers believe infections or viral agents may trigger the onset of the disease in genetically predisposed individuals, although this connection is not yet fully proven.

Diagnosis:

There is no single test to diagnose Adult Still’s disease. Diagnosis is typically made based on clinical signs and symptoms and by excluding other conditions. Diagnostic steps may include:

  1. Blood Tests: Patients often have elevated levels of inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Additionally, high levels of ferritin (a protein involved in iron storage) are often seen in ASD and can help confirm the diagnosis.
  2. Rheumatoid Factor (RF) and Antinuclear Antibodies (ANA) tests: These may be negative, helping to distinguish Still's disease from other autoimmune conditions like rheumatoid arthritis or lupus.
  3. Imaging: Imaging tests such as X-rays or MRI may be used to check for joint damage and inflammation.

Treatment:

The treatment for Adult Still's disease focuses on controlling inflammation and symptoms. The goal is to manage the disease and prevent organ damage and disability. Treatment options include:

  1. Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): These are used to reduce inflammation and relieve pain, particularly during fever spikes.
  2. Corticosteroids: Prednisone or other corticosteroids are commonly prescribed to reduce systemic inflammation, especially during acute flare-ups.
  3. Disease-Modifying Antirheumatic Drugs (DMARDs): Medications like methotrexate or sulfasalazine may be used to control the disease and prevent long-term damage to joints.
  4. Biologic Drugs: In cases where other treatments are ineffective, biologic agents like anakinra (which targets interleukin-1, a key player in inflammation) or tocilizumab (which targets interleukin-6) may be prescribed to help control inflammation.
  5. Other Immunosuppressive Drugs: In severe cases, immunosuppressive medications like cyclophosphamide or azathioprine may be used.

Prognosis:

The prognosis of Adult Still’s disease varies widely from person to person. Some people may experience only one or two episodes, while others may have persistent symptoms and chronic inflammation. With early and appropriate treatment, the symptoms of ASD can be managed effectively, and most people can lead a relatively normal life. However, if left untreated, the disease can lead to significant joint damage, organ complications, and long-term disability.

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