What is Still’s Disease?

Information About Still’s Disease

What is Still’s disease?

Still’s disease is a rare inflammatory disorder that affects the entire body (systemic disease). The cause of the disorder is unknown (idiopathic) Reference 1. It is characterized by episodes of high, spiking fevers, an evanescent (transient) pink or salmon-colored rash (view pictures of the Still’s rash), fatigue, joint pain, muscle pain, a sore throat and other symptoms associated with systemic inflammatory disease. However, symptoms can vary per individual, which can make detection and diagnosis difficult. Still’s disease was first described in children, but it is now known to occur, much less commonly, in adults (in whom it is referred to as Adult-onset Still’s disease, AOSD).

What causes Still’s disease?

There have been a number of schools of thought. One is that Still’s disease is due to infection with a microbe. Another concept is that Still’s disease is a hypersensitive or autoimmune disorder. In truth, the cause of Still’s disease is still not known.

How does Still’s disease fit in with Juvenile Arthritis?

Still’s disease is one type of Juvenile Arthritis also known as systemic-onset juvenile idiopathic arthritis, SOJIA. By “systemic” it is meant that along with joint inflammation it typically begins with symptoms and signs of systemic (body wide) illness, such as high fevers, gland swelling, and internal organ involvement. Still’s disease is named after the English physician Sir George F. Still (1861-1941).

What are symptoms and signs of Still’s disease?

Patients with Still’s disease usually present with systemic (full body) symptoms. Extreme fatigue can accompany waves of high fevers that rise to 104 degrees F (41 degrees C) or even higher and can rapidly return to normal levels or below. Fevers can happen daily and most often spike later in the day, but can occur at any time and may happen more than one time a day.  A faint salmon-colored skin rash characteristically comes and goes and usually does not itch (picture of the Still’s rash).

There may be swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. Some patients develop inflammation of the lungs (pleuritis) or around the heart (pericarditis) with occasional fluid accumulation around the lungs (pleural effusion) or heart (pericardial effusion). Although the arthritis may initially be overlooked because of the impressive nature of the systemic symptoms, most everyone with Still’s disease eventually develops joint pain and swelling. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still’s disease.

If Still’s disease goes untreated, chronic inflammation of the joints can potentially result in deterioration and destruction of the affected joints.

The former ISDF participated in the Early Symptoms of Autoimmune/Autoinflammatory Arthritis study, conducted in 2014 by IFAA – the International Foundation for Autoimmune & Autoinflammatory Arthritis, which compared and contrasted patient-reported symptoms in early disease (0 < 24 months from onset) in adult onset Still’s Disease, Rheumatoid Arthritis, Psoriatic Arthritis, Lupus, and Sjögren’s Syndrome.  You can view those results here to learn more about the findings.

How is Still’s disease diagnosed?

Still’s disease is diagnosed purely on the basis of the typical clinical features of the illness. Persistent arthritis (arthritis lasting at least 6 weeks) is required to make a firm diagnosis of Still’s disease. Other diseases (especially infections, cancers, and other types of arthritis) are excluded. Many patients with Still’s disease develop markedly elevated white blood cell counts, as if they have a serious infection but none is found. Low red blood counts (anemia) and elevated blood tests for inflammation (such as sedimentation rates) are common. However, the classic blood tests for Rheumatoid Arthritis (rheumatoid factor) and systemic Lupus erythematosus (antinuclear antibodies, ANA) are usually negative.

The cause of adult onset Still’s disease is unknown (idiopathic).  Therefore, some researchers believe that adult onset Still’s disease is an autoinflammatory syndrome (part of a group of disorders characterized by recurrent episodes of inflammation due to an abnormality of the innate immune system).  However, others still consider Still’s disease an autoimmune disorder, in which the adaptive immune system malfunctions and mistakenly attacks healthy tissue.  You can learn more about autoimmune versus autoinflammatory here (Reference 2).

What is the frequency of Still’s disease and its features?

Still’s disease accounts for 10-20% of all cases of sJIA. It affects about 25-50,000 children in the United States. It is rare in adults, a majority of whom are between 20 and 35 years of age at onset of symptoms. Of all patients with Still’s disease, 100% have high intermittent fever; 100% have joint inflammation and pain, muscle pain with fevers, and develop persistent chronic arthritis. Ninety-five percent (95%) have the faint salmon-colored skin rash. Eighty-five percent (85%) have swelling of the lymph glands or enlargement of the spleen and liver; and 85% have a marked increase in the white blood cell count. Sixty percent (60%) have inflammation of the lungs (pleuritis) or around the heart (pericarditis). Forty percent (40%) have severe anemia. And twenty percent (20%) have abdominal pain.

Updated statistics is that while Still’s disease is very rare, population.incidence of the disease in the general population is between 1 in 100,000 to 500,000.

Seventy-Six percent (76%) onset before age Thirty-Five (35) and less than Nine (9%) onset after age Fifty (50). [1]

[1] Dr. John J. Cush, International Still’s Disease Conference, September, 2012

Preparing for your appointment

By Mayo Clinic staff

You’re likely to first seek advice from your family doctor, but he or she might refer you to a specialist in joint diseases (rheumatologist).

Because appointments can be brief and there’s often a lot of ground to cover, it’s a good idea to be well prepared for your appointment. Here’s some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

  • Write down your symptoms, including when they first started and how often they flare up.
  • Write down your key medical information, including any other health conditions with which you’ve been diagnosed.
  • List all the medications you’re currently taking, including prescription and over-the-counter drugs, vitamins, or supplements.
  • Take a family member or friend along, if possible. Someone who accompanies you can help remember information that you missed or forgot.
  • Write down questions to ask your doctor. Creating your list of questions in advance can help you make the most of your time with your doctor.

What to expect from your doctor Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

  • What are your symptoms, and when did they first develop?
  • Do your symptoms come and go, or are they continuous?
  • When are your symptoms most likely to flare up?
  • What treatments or self-care measures have you tried so far?
  • Have any treatments or self-care measures helped?
  • Have you been diagnosed with any other medical conditions?

What is the outlook with Still’s disease?

The fever and other systemic features tend to run their course within several months for some patients. The arthritis can be a long-term problem. It usually stays on after the systemic features have gone. The arthritis can then become chronic and persist into adulthood.

How is Still’s disease treated?

Still’s disease can cause serious damage to the joints, particularly the wrists knees and hips. It can also impair the function of the heart and lungs. Treatment of Still’s disease is directed toward the individual areas of inflammation. Many symptoms are often controlled with anti-inflammatory drugs, such as aspirin or other non-steroid drugs (NSAIDs). Cortisone medications (steroids), such as prednisone, are used to treat more severe features of the illness. For patients with persistent illness, medications that affect the inflammatory aspects of the immune system are used. Medications now being used are analogous to the classic “second-line” therapies used for patients with Rheumatoid Arthritis. These include Gold, Hydroxychloroquine (PLAQUENIL), Penicillamine, Azathioprine (IMURAN), Methotrexate (RHEUMATREX), and Cyclophosphamide. Biologics, such as Enbrel, Remicade, Kineret and several others are available and are being used as a first line therapy in treating Still’s meaning you don’t have to take Methotrexate (MTX and fail before you can move on to one of the biologics). Most of the research however shows that when mtx is given along with enbrel or remicade that the outcome is much better than with either one alone.

Patient and Family Education

Patients and their families should be provided with the necessary information to enable them to have a complete understanding of the disease and its effects on their life. Still’s disease may manifest itself mostly as joint symptoms, especially early in the course of the disease. It is essential that patients with Still’s and their families understand that the disease is systemic and may involve many areas of the body.

Patients and their families should understand that the disease is often cyclic in nature, and that they should expect “good” and “bad” days and even “good and “bad” hours of the day and the night. Further, they should understand that their actions on any given day can cause a “flare” or exacerbation of the disease (that is, a “bad” day). While a patient may never be able to completely stop a bad day, frequently a patient can manage her or his life to reduce the number of bad days.

Central to controlling bad days is planning activities and rest periods. Patients and their families must understand the need for planning virtually every activity of their lives. This is necessary because a patient with this disease can cause a flare by over-working or by increasing physical or emotional stress. Rest is important for the patient with Still’s and cannot be overemphasized.

Planning by the patient with Still’s should be done on a yearly, monthly, weekly, and daily basis. For example, if the patient is considering a vacation, the dates should be marked on a calendar well in advance so there is ample time to pack and otherwise prepare for the trip. Patients who prepare immediately before the trip may be too fatigued and sore to enjoy the trip and may initiate a flare. Similarly, weeks should be planned so that there are rest days interspersed with work days. And even the hours of the day should be planned so that after a period of physical activity, a period of rest follows. For every hour of activity, 20 minutes of rest is recommended when the disease is chronic.

Planning should also incorporate changes in body position. Patients should be encouraged to change their position frequently during the course of the day. Ideally, position changes should occur at least every two hours. The patient with Stills who sits most of the day should periodically get up and walk around. The patient who stands most of the day should find some way to periodically sit and rest. It should be acknowledged by all involved that at some point changes in lifestyle may need to be made.

There is some evidence that emotional highs and lows play a part in exacerbation of Still’s. Clearly, we cannot plan for every stressful or emotional situation, but there are some instances in which we can. For example, if the patient with Still’s gets emotionally involved with sporting events, then he or she should probably avoid watching the event. If driving at night or in bad weather is stressful for the patient, plan to avoid driving at these times. As noted previously, the disease is cyclic in nature and there are good days/hours as well as bad ones.

One problem with good days, especially in the newly diagnosed patient, is that there may be a tendency for the patient to believe he or she has been “cured” or that the physician may have incorrectly diagnosed the problem. This is why educating the patient and family is important. They must understand the cyclic nature of the disease. Patients who do not understand the disease process may quit taking medication or quit other therapy on good days because they mistakenly believe they are free of the disease. Patients who quit managing the disease are more likely to flare, which can result in further joint destruction or other systemic problems.

Finally, patient education should include a discussion about quackery. There are any number of “rip-off” artists preying on ill-informed patients. There are plenty of devices or gizmos on the market that are advertised as cures for the disease. Further, tabloids sell their issues by printing in large, bold letters purported cures that are “hidden” from the public by the medical establishment. Others claim certain diets and/or vitamin supplements are the best methods to become disease free.

Patients and families are particularly susceptible to these claims if remission of the disease occurs immediately following the use of one of the purported cures. It is the responsibility of health care providers to educate patients about quackery, and this topic should be part of any formal educational program. If patients, families, or practitioners need further information on therapeutic interventions or devices, please have them call the Arthritis Foundation.

* Dr. John J. Cush – Reported at Still’s Conference September, 2012