Still's Disease - Mayo Clinic



Still's Disease, Juvenile Arthritis, and Rheumatoid Arthritis: What's the Difference?

Systemic juvenile idiopathic arthritis and adult-onset Still's disease are similar conditions — the only major difference is the age at which symptoms begin.

By Tula Karras

Medically Reviewed by Alexa Meara, MD

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Like Still's disease, systemic juvenile idiopathic arthritis is an inflammatory arthritic condition.
Like Still's disease, systemic juvenile idiopathic arthritis is an inflammatory arthritic condition.
Rafal Rodzoch/Alamy

Systemic juvenile idiopathic arthritis (SJIA) is one of seven types of juvenile (childhood) idiopathic arthritis, or JIA (idiopathic means “of unknown origin”). SJIA may sometimes be referred to as pediatric Still's disease, which is named after the doctor who first reported it in children in the late 1800s.

SJIA accounts for about 10 percent of JIA cases, says , clinical director of pediatric rheumatology at The Children's Hospital of Philadelphia, and assistant professor of clinical pediatrics at the University of Pennsylvania's Perelman School of Medicine. JIA, the most common type of juvenile arthritis, affects between 1 in 1000 and 1 in 2500 children, or 30,000 to 75,000 children, in the United States, according to 2015 census data estimates.

When the disease develops after age 16, it's called adult-onset Still's disease (AOSD). “Up to 10 percent of rheumatoid arthritis cases in U.S. adults may be AOSD,” says Brian Golden, MD, clinical associate professor of medicine in the division of rheumatology at NYU Langone Medical Center in New York City.

AOSD usually strikes young adults from ages 16 to 35, but it can develop at any age. “The immune systems of children and young adults share some commonalities, which may be why we see it more often in younger adults,” says Petros Efthimiou, MD, associate chief of medicine and rheumatology at New York Methodist Hospital in Brooklyn, New York, and associate professor of clinical medicine at Weill Cornell Medicine in New York City.

What Causes SJIA and AOSD?

The disease is due to an overactive innate immune system, making it different from most other types of chronic arthritis, which involve the adaptive immune system. (The adaptive immune system makes specific antibodies to specific substances, whereas the innate system launches a more general response). SJIA and AOSD are systemic — meaning they affect the entire body — and are also idiopathic, meaning the cause is unknown. Although SJIA and AOSD are diseases of the immune system, they are not considered autoimmune, as are most types of rheumatoid arthritis, lupus, or Sjögren’s syndrome; instead, they're considered autoinflammatory diseases.

No one knows exactly why the innate immune system goes awry, though it may be a response to an infection or have a genetic component. “But not everyone with the genetic markers gets the disease, and not everyone with the disease has the genetic markers,” says Dr. Mehta. Symptoms include:

  • A daily high fever of 102 F or higher that lasts for about 4 hours and typically spikes in the afternoon, but can occur anytime (for a SJIA diagnosis, the fever must be present for two or more weeks)
  • A salmon-pink rash that can appear anywhere but often shows up on the limbs and trunk, and that comes and goes — often along with a fever

  • Joint pain and swelling in any joint, frequently in the knees, ankles, wrists (in adults), and the cervical spine joints (in children)

  • Generalized muscle pain (myalgia)

  • Lymph node swelling

Joint pain and inflammation may not develop until a few weeks or months after the fever and rash. And once the disease becomes chronic, the rash and fever may not be present anymore, says Dr. Efthimiou.

In children, about one-third of those with SJIA will have a “one and done” episode: They develop initial symptoms, get diagnosed, start on medication that quells symptoms, and can eventually curtail some or all of their meds, says Mehta. Another one-third with SJIA will experience remission with periods of flare-ups, and another third will have constant disease activity with inflammation. “Some children will outgrow SJIA, but we don’t have great data on this since they transition to adult rheumatologists and we often don’t know the outcomes,” says Mehta.

In about 10 percent of SJIA cases (and more rarely in adults), a potentially deadly complication called macrophage activation syndrome (MAS) can develop. In MAS, there is overwhelming systemic inflammation that can affect any organ, including the heart and lungs. Signs of MAS include an unrelenting high fever, enlarged lymph nodes, and an enlarged spleen and liver. Early treatment is vital, as the condition causes death in 8 percent of cases.

In adults, some research suggests that about one-third of those with AOSD will have one episode of fever, rash, or joint pain symptoms (or a combination of two or more of these) that's short-lived and doesn’t recur. In about one-quarter of patients, the disease recurs periodically, with episodes recurring either years apart — or even months or weeks apart. More than a third will have chronic disease.

Diagnosing SJIA and AOSD

Because there's no hard-and-fast blood test for SJIA or AOSD, doctors must diagnose the disease based on symptoms, by eliminating other diseases, and by assessing certain biological markers. Blood test results that can indicate SJIA or AOSD include:

  • High ferritin levels (a protein that stores iron)

  • A high white blood cell count (a high count can indicate an immune disorder or an inflammatory condition)

  • High interleukin 1 levels (a type of protein produced by immune cells that can indicate inflammation)

  • A high erythrocyte sedimentation rate

  • A high C reactive protein level

(Note that the rheumatoid arthritis factor is usually negative in SJIA and can also be negative for AOSD.)

Musculoskelatal X-rays and ultrasound are also used to assess joint damage for diagnosis and remission.

Certain conditions are often ruled out before doctors consider SJIA or AOSD. And because the disease shares features with other more common conditions, there can be a delay in diagnosis and treatment. This is especially concerning for SJIA, since untreated SJIA can impair musculoskeletal growth (just like JIA).

“Pediatricians will often try antibiotics for what they think are back-to-back viral infections, then they will refer the child to an oncologist, who will rule out cancers, sometimes with a bone marrow biopsy,” says Mehta. It’s often not until the child tests negative for cancers that they are referred to a rheumatologist, causing weeks or months of delay in a diagnosis. “Ideally, a pediatrician will refer a child to a pediatric rheumatologist,” says Mehta. But there are only about 400 pediatric rheumatologists in the country, which makes the referral process difficult. Oftentimes, rheumatologists who treat adults end up diagnosing SJIA in children.

For adults, doctors may first consider a chronic infection or cancer of the immune system, such as lymphoma. Other conditions that a primary care doctor will want to rule out are tuberculosis and bacterial endocarditis, says Dr. Golden. Once these are eliminated, the doctor may refer the patient to a rheumatologist, who will then consider rheumatologic diseases such as AOSD.

Treating AOSD and SJIA

The first course of treatment for AOSD is often anti-inflammatory drugs, including non-steroidal anti-inflammatory drugs (NSAIDS) and corticosteroids, followed by biologics: drugs that are often injected or infused, and which block the activity of inflammatory cytokines. Because there are more medicines approved for SJIA than AOSD, doctors generally treat AOSD with some of the same drugs as SJIA.

“Putting patients on NSAIDs and steroids first to get the disease under control, and then using other drugs such as biologics, yields a good response,” says Golden, adding that some patients can get off of medication altogether. Although some research shows that the traditional RA drug methotrexate (Rasuvo, Trexall) is less effective for SJIA and AOSD, some doctors prescribe it as a way to lower the dosage of steroids needed to control inflammation; long-term use of steroids can increase the risk of osteoporosis, cataracts, weight gain, diabetes, cardiovascular risk, and more, says Golden.

The greatest area of research for both AOSD and SJIA is with the biologic drugs, including medications such as Kineret (anakinra), Actemra (tocilizumab), and Ilaris (canakinumab), which block IL-1 cytokines and IL-6 cytokines, both of which are thought to play a role in the disease. But some research has found that methotrexate is less effective for AOSD and SJIA than it is in other RA conditions.

With SJIA, patients are often started on NSAIDs. Some children with mild to moderate symptoms do well on this treatment alone. Steroids are used more sparingly in children because of the concern over their effect on growth, so biologic drugs are often the next step for children whose symptoms cannot be controlled with NSAIDs.

Because many of the drugs used to treat SJIA, AOSD, and RA suppress the immune system, patients must be monitored carefully for infections. But most experts agree that risks associated with drug side effects are far, far less worrisome than the risks of not treating the condition aggressively. And the outcome is hopeful for those undergoing drug treatment: “Children tend to do really well with treatment, and we can prevent long-term effects of the disease,” says Mehta.

In addition to drug therapy, regular physical activity — especially for children, who are still growing and developing — is key to keeping joints flexible and muscles strong (which is important for supporting joints), and in helping to reduce pain.






Video: Mysterious Symptoms and then...Diagnosis: Still's Disease

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Date: 10.12.2018, 14:17 / Views: 54264