Adult Onset Still’s Disease

Adult onset Still’s disease is an immune system based condition characterized by recurrent fevers, rash, and joint pain. It may lead to chronic arthritis.

Causes, Incidence, and Risk factors

The cause of Adult onset Still’s disease is unknown. The condition can occur both in pediatric and adult patients, perhaps more often in children. There are no identifiable risk factors for the disease.

Symptoms

Almost all patients will present with recurrent daily fevers, joint pain, sore throat, and a rash. The fever usually comes on quickly once or twice daily, around the same time each day. The rash is typically salmon pink colored, will occur on the abdomen and extremities and comes and goes with the fever.

Other common symptoms are joint pain and inflammation (warmth and swelling of the joint). Usually, several joints are involved at the same time.

Additional symptoms include swollen lymph nodes (glands), pain with a deep breath (pleurisy), abdominal pain and swelling, and weight loss.

Diagnosis

A thorough medical history and physical examination is imperative. There are many conditions with overlapping signs and symptoms and laboratory changes that can be confused with Adult onset Still’s Disease.

Specifically, the physical exam may show the fever, rash, and arthritis. Other signs include enlargement of the lymph nodes, liver, or spleen.

Blood tests that can be helpful in diagnosing Adult Still’s Disease include:

  • Elevation in the ESR (sedimentation rate)
  • Elevation in the White Blood Cell count
  • Decrease in the Red Blood Cell count (anemia)
  • Elevation in liver function tests
  • Very high elevation in the Ferritin level
  • Negative rheumatoid factor and ANA test

Other tests may include:

  • Joint X-rays
  • Chest X-ray that may show signs pericarditis or pleural effusion
  • abdominal X-ray, CT scan or ultrasound for liver and spleen enlargement

Adult Still’s Disease can only be diagnosed after other diseases are excluded. It may require many medical tests before a final diagnosis is made.

Treatment

Nonsteroidal anti-inflammatory medications (NSAIDs) are often sufficient to control the arthritis and milder symptoms of Adult onset Still’s disease.

For more severe cases with persistent fevers and laboratory abnormalities, corticosteroids (ie. prednisone), may be used.

For those unable to control their symptoms on corticosteroids and/or NSAIDs, methotrexate or newer biologic therapies may be utilized. Biologic medications that target specific immune system components that cause information include infliximab (Remicade), etanercept (Enbrel) and anakinra (Kineret) among others.

Prognosis

Research show that about 20% of patients enter remission at one year without recurrence and another 30% will have several disease recurrences. The remaining 50% will develop a chronic phase of Adult onset Still’s disease with a chronic arthritis.