Still’s Disease: SoJIA

Some eponymous diseases are named after a patient (think Lou Gerig’s Disease), while others derive their name from the physician who identified the condition.  Still’s Disease was first described in 1896 by an English pediatrician, Sir George Fredric Still.

I recall the first time I heard someone say that he had Stills Disease.  Nobody present at the time knew what Stills was, and so the person explained that it was adult-onset JRA.  I thought, “Wait!  Adult-onset of Juvenile RA would be RA.”  It’s not.  The out-dated term JRA didn’t simply mean RA diagnosed in children; it’s actually a different disease (technically, at least three different diseases).  Adult-onset Stills is similar to one type of juvenile arthritis, but it is not the same.

“Still’s Disease” is Obsolete

George Still’s doctoral thesis was published in 1897, and described a childhood arthritis characterized by joint pain, fevers, and a salmon-colored rash.  This was later renamed systemic onset juvenile rheumatoid arthritis (so as not to be confused with the other types of JRA).

JRA, however, is considered by many to be misnamed, and the word “rheumatoid” is no longer used.  What was originally considered Still’s Disease and was later known as systemic-onset JRA, is now classified as Systemic-Onset Juvenile Idiopathic Arthritis.

Adult-Onset Still’s Disease

In 1971, a British rheumatologist, Eric George Lapthorne Bywaters, noted that some adults had a disease similar to Still’s (systemic onset JIA), but that their disease didn’t meet the criteria for a diagnosis of rheumatoid arthritis.  The disease is relatively rare and hard enough to recognize that it was featured in Dr. Lisa Sanders’ book, Every Patient Tells a Story.

When discussing Still’s, it’s important to distinguish the age when the patient was first afflicted.  The cut-off is age sixteen.  Older, it’s adult-onset Still’s; younger, it’s systemic-onset JIA.

Adult Stills tends to follow three patterns.  Some people’s disease lasts less than one year (monophasic).  Others experience cycles of flares/remission (intermittent).  Unfortunately, others experience on-going disease activity that can lead to joint destruction (chronic).

Treatment

There is no cure for either the juvenile or adult versions of this disease.  There are treatments, though.

Historically, aspirin or NSAIDs were the only treatment available.  In time it was discovered that corticosteroids (such as prednisone) can be effective in reducing the inflammation of this disease, but should not be used indefinitely since long-term steroid use can be bad.

In 1988, methotrexate was FDA approved as an RA treatment, and over time it has been used to treat other autoimmune diseases, too.  Other DMARDs that have proven effective in treating other forms of autoimmune arthritis can also be effective in treating people with adult Still’s/SoJIA.

Additionally, there are stronger medications (known as biologics) that can be very effective if the older DMARDs don’t provide enough relief.

Further Reading
In case you’d like to know more about this disease than “joint inflammation, fevers, and rash,” following are two of the many articles I read in preparing this post.

Adult Still’s Disease
Juvenile Rheumatoid Arthritis

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My reading in preparation for my daughter’s follow-up appointment with the pediatric rheumatologist has followed a bit of a rabbit trail.  I’ll be doing a few posts on a variety of different autoimmune diseases.