Index
Definition:
Still’s disease is a form of arthritis that is characterized by elevated fevers and an evanescent (transient) salmon-colored rash (like the photo above).
It was discovered for the first time in children, but now it is known to occur, much less commonly, in adults. who is known as Still’s disease of onset in adults.
Causes
There have been several schools of thought. One is that Still’s disease is due to infection with a microbe. Another concept is that this disease is a hypersensitive or autoimmune disorder. The truth is that the cause is not yet known.
How does Still’s disease fit with juvenile arthritis?
Still’s disease is a type of juvenile arthritis also known as juvenile idiopathic arthritis of systemic onset.
By “systemic” it is understood that together with inflammation of the joints it usually begins with the symptoms and signs of systemic disease (wide body), such as high fevers, swelling of the glands and involvement of internal organs.
Still’s Disease is named after the English physician Sir George F. Still (1861-1941).
What are the symptoms and signs?
Patients with this disease usually have systemic symptoms (body width).
Extreme fatigue can accompany waves of high fevers that rise to 41 degrees C (104 degrees F) or even higher and return quickly to normal levels or below them.
Also, a weak salmon skin rash typically comes and goes and usually does not itch. There is commonly swelling of the lymph nodes, 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 arthritis may be initially overlooked due to the impressive nature of the systemic symptoms, everyone with Still’s Disease eventually develops joint pain and swelling. This usually involves many joints (polyarticular arthritis).
Any articulation can be affected, although there are preferential patterns of joint participation in Still’s disease.
As a curious fact, in the past years, the International Autoimmune and Autoinflammatory Arthritis Foundation compared and contrasted the symptoms reported by patients in the early disease phase (0 <24 months from the beginning) of this condition, rheumatoid arthritis, psoriatic arthritis, lupus and Sjögren’s syndrome. Your study continues.
How is it diagnosed?
Still’s disease is diagnosed purely on the basis of the typical clinical characteristics of the disease.
Persistent arthritis (arthritis that lasts for at least 6 weeks) is required to make a firm diagnosis of Still’s Disease. Other diseases are excluded (especially infections, cancers and other types of arthritis).
Many patients with Still’s Disease develop a remarkably high white blood cell count, as if they had a serious infection but none is found.
Low red blood counts (anemia) and elevated blood tests for inflammation (such as rates of sedimentation) are common.
However, classical blood tests for rheumatoid arthritis (rheumatoid factor) and systemic lupus erythematosus (antinuclear antibodies, ANA) are usually negative.
What is the frequency of Still’s Disease and its characteristics?
Still’s disease represents 10-20% of all cases of JRA. Affecting around 25-50,000 children in the United States. It is rare in adults, most of whom are between 20 and 35 years old at the onset of symptoms.
Of all patients with Still’s disease, 100% have high intermittent fever; 100% have inflammation and pain in the joints, muscle pain with fever and develop persistent chronic arthritis.
Ninety-five percent (95%) have a faint salmon-colored skin rash.
Also, eighty-five percent (85%) have swollen lymph nodes or enlargement of the spleen and liver; and 85% have a marked increase in the white blood cell count.
Sixty (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.
The updated statistic is that, while Still’s Disease is very rare, the population incidence of the disease in the general population is between 1 in 100,000 and 500,000.
Seventy-six percent (76%) start before the age of thirty-five (35) and less than nine (9%) start after age Fifty (50). [1]
What is the perspective?
Fever and other systemic features tend to run their course within several months for some patients. Arthritis can be a long-term problem.
It usually stays on after the systemic characteristics are gone.
Arthritis can become chronic and persist into adulthood. There are four types of patterns that Still’s Disease can take on any patient.
Treatments
Still’s disease can cause serious damage to the joints, particularly the wrists of the knees and hips. It can also affect the function of the heart and lungs.
Therefore, the treatment of Still’s Disease is directed towards the individual areas of inflammation.
Many symptoms are often controlled with anti-inflammatory drugs, such as aspirin or other nonsteroidal drugs (NSAIDs). Cortisone medications (steroids) are used to treat more serious features of the disease.
For patients with persistent disease, medications are used that affect the inflammatory aspects of the immune system.
The drugs used now 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.
Currently, there is a new class of drugs called biologicals that are very promising in the treatment of Still.
For example, Enbrel, Remicade, Kineret and several others are available and are being used as a first-line therapy in the treatment of the meaning of Still, which dispenses with taking Methotrexate (MTX and not before switching to one of the biological products.
However, it shows that when mtx is given together with enbrel or remicade, the result is much better than with just one.
So, we have that science offers several medications for the treatment of Still’s Disease and that the doctor should know which is the best for the patient.
