Still’s Disease

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rash in stills disease 4

What is Still’s Disease?

Still’s disease, also known as systemic juvenile idiopathic arthritis, is a form of arthritis named after Sir George F. Still, an English physician [1].

It occurs more commonly in children, hence the name systemic ‘juvenile’  idiopathic arthritis.

Adults can also be affected, although much rarely than in children. In addition, women are more at risk of developing the disease than men. According to statistics, less than 1 out of 100,000 people are affected by Still’s disease each year [2].

The cause of this disease is still unknown. There are theories, however, that say Still’s disease may be caused by a microbial infection. Another theory proposes that Still’s disease is an autoimmune disorder [3].

 

Adult Still’s Disease

Adult Still’s disease has similar symptoms as in systemic juvenile idiopathic arthritis, except that it occurs during adulthood. It occurs at any age before 45 [3], more commonly around the age of 20 to 35 [4].

 

Rash in Still’s Disease

A common feature of Still’s disease is salmon-colored rash that does not usually itch. It occurs in almost all cases of Still’s disease.

Flat spots or raised bumps, or a combination of the two, may appear. It can be seen on the face, torso, or on the limbs, usually on the upper arms or legs.

These rashes, however, are not permanent, but may reappear throughout the course of the disease. [3]

 

Pictures: Rash in Still’s Disease

rash in stills disease 1

Picture 1 : Flat salmon-colored rash in Still’s disease

Source: dermnetnz.org

 rash in stills disease 2

Picture 2 : Raised salmon-colored bumps in Still’s disease

Source: static.wikidoc.org

 rash in stills disease 3

 

Picture 3 : Salmon-colored rash on upper arms

Source: nras.org.uk

 rash in stills disease 4

 

 Picture 4 : Salmon-colored rash on torso

Source: ars.els-cdn.com

Still’s Disease Symptoms

Aside from salmon-colored rashes, other characteristic symptoms of Still’s disease are:

  • High swinging fever that reaches up to 102° F. It occurs quickly once per day, usually during the afternoon or evening.
  • Joint pain and inflammation (arthritis), primarily in the wrists and knees. Ankles, shoulders, elbows and finger joints may also be affected. A few joints are affected at first, then several joints become affected once the disease worsens. Joints also usually stiffen in the morning.[2,3]

 

Other less common symptoms include:

  • Swollen lymph nodes
  • Muscle pain
  • Abdominal pain and inflammation
  • Severe and burning sore throat
  • Weight loss[2,3]

 

Occasional symptoms, which usually happen in severe cases of Still’s disease, include:

  • Spleen inflammation
  • Liver inflammation
  • Lung inflammation (pleuritis) which leads to difficulty in breathing
  • Inflammation of the pericardium or the fibrous sac surrounding the heart (pericarditis)[2,3]

Both systemic juvenile idiopathic arthritis and adult Still’s disease manifest these symptoms. These symptoms may come and go. The relapse may happen several times a year. In some rare cases, the symptoms can permanently disappear.

 

Diagnosis of Still’s Disease

The symptoms of Still’s disease can be confused with other diseases, namely Lyme disease and Crohn’s disease. These diseases should be ruled out first to confirm a case of Still’s disease. Diagnosis includes blood tests and imaging tests. [5]

Blood tests

  • Complete blood count (CBC)- high count of white blood cells and low count of red blood cells
  • High C-reactive protein indicates inflammation
  • High erythrocyte sedimentation rateindicates inflammation
  • High ferritin level
  • High fibrinogen level
  • High liver enzymes (AST, ALT)
  • Negative rheumatoid factor and ANA

Imaging tests

  • Abdominal ultrasound
  • CT scan of abdomen
  • X-ray

Imaging tests check the following:

  • Joint inflammation
  • Enlarged spleen, liver and lymph nodes
  • Inflammation of the pericardium
  • Accumulation of fluid between the linings of the lungs (pleural effusion)

Treatment for Still’s Disease

In the present, there is still no cure for Still’s disease. There is also no way to prevent the disease since the cause is still unknown.

Management of the disease only focuses on treating the symptoms of arthritis. Medications include:

  • Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, naproxen or aspirin, alleviate pain and inflammation. These can be given even before the diagnosis of Still’s disease.
  • Corticosteroids, such as prednisone, address inflammation in severe cases of the disease. However, it cannot be used for long term because of some serious side effects.
  • lmmunosuppresantsisfor chronic cases. Combination of these immunosuppresants can be administered. Examples include:
    • Methotrexate
    • Hydroxychloroquine
    • Sulfasalazine
    • Azathioprine
    • Cyclophosphamide
    • Cyclosporine[3,6]

 

Aside from medications, lifestyle modification is essential in managing the disease.

  • Carrying heavy objects should be avoided to prevent excessively stressing the joints. If carrying heavy objects cannot be avoided, it must be ensured that the objects are carried in a safe way, such as carrying the load close to the body. Carrying aids are also recommended.
  • Exercise should also be regularly done to reduce pain and joint damage. If a swollen joint is not functional, it will cause the muscles around it to become weak.
  • There are three types of exercise that can be done: range of motion exercises, strengthening exercises, and endurance exercises.
  • Range-of-motion exercises reduce stiffness of joints. An example of this exercise is rotating the arms in large circles, which can reduce stiffness of shoulder joints.
  • Strengthening exercises are performed to increase muscle strength.
  • Endurance exercises, such as swimming, walking and cycling strengthen the heart, and give energy to the body.
  • Lastly, the body should be given enough rest after a strenuous activity. Relaxing the muscles around a joint reduces the pain. Daily activities should be well-planned to give way for rest in between these activities.

Research in Still’s Disease

Current researches in the treatment of Still’s disease include the use of intravenous immunoglobulin therapy. The study conducted by Vignes and Wechsler utilized immunoglobulins administered at 2g/kg for 2 to 5 days, repeated 4-6 times every month. Results showed that 50% of the patients obtained long-term remission. However, the mechanism of action is still unknown. [7]

Another treatment under study is tocilizumab, an anti-interleukin (IL)-6 receptor antibody [8]. Research shows that the pathogenic cause of Still’s disease is the uncontrolled activation and hypersecretion of IL-1 and IL-6 [9]. Tocilizumab is targeted against IL-6 receptor, and the studies conducted showed rapid alleviation of arthritic and systemic symptoms.Anakinra, on the other hand, is targeted against IL-1 receptor [2].

Tumor necrosis factor (TNF) inhibitors such as etanercept and infliximab are also promising medications for Still’s disease, and is used when other medications already failed [1].

 

References:

  1. http://www.medicinenet.com/stills_disease/article.htm
  2. http://www.nlm.nih.gov/medlineplus/ency/article/000450.htm
  3. http://arthritis.webmd.com/adult-onset-stills-disease
  4. http://www.iaamovement.org/uploads/Juvenile_Still.pdf
  5. http://health.nytimes.com/health/guides/disease/adult-stills-disease/
  6. http://www.arthritis.ca/page.aspx?pid=1003
  7. http://www.ncbi.nlm.nih.gov/pubmed/10522316
  8. http://www.medpagetoday.com/Rheumatology/GeneralRheumatology/24172
  9. http://www.stillsdisease.org/wp-content/uploads/2011/03/Is-Stills-Disease-An-Autoinflammatory-Syndrome1.pdf

1 COMMENT

  1. This is interesting. It looks and almost sounds like hives but it isn’t. Is this something that is similar to hives in any way? I seem to see a pattern where many diseases over-lap the other and most have to do with an inflammation response problem or stress induced malnutrition.

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