Causes,
incidence, and risk factors
The cause of Adult
Still's disease is unknown. The condition rarely
occurs in adults. It is more common in children,
where it is called Systemic Juvenile Rheumatoid
Arthritis. No risk factors for the disease have
been identified.
Symptoms
Almost all patients
will have fever, joint pain, sore throat, and
a rash. The fever usually comes on quickly once
per day, most commonly in the afternoon or evening.
The rash is typically salmon pink colored and
comes and goes with the fever.
Another common
symptom is joint pain and inflammation (warmth
and swelling of the joint). Usually, several joints
are involved at the same time.
Additional symptoms
include swollen lymph nodes (glands), pain with
a deep breath (pleurisy), abdominal pain and swelling,
and weight loss.
Signs
and tests
The physical exam
may show the fever, rash, and arthritis. Other
signs include enlargement of the lymph nodes,
liver, or spleen. Also, the presence of changes
in the sound of the heart or lungs may indicate
pericarditis or pleurisy.
Blood tests that
can be helpful in diagnosing Adult Still's Disease
include:
- Elevation in the ESR (sedimentation
rate)
- Elevation in the White Blood
Cell count
- Elevation in liver function
tests
- Decrease in the Red Blood
Cell count
- Very high elevation in the
Ferritin level
- Negative rheumatoid
factor and ANA
test
Other tests may
include:
- joint X-rays
- chest X-ray
that may show pericarditis or pleural effusion
- abdominal X-ray, CT scan
or ultrasound for liver and spleen enlargement
Adult Still's Disease
can only be diagnosed after other diseases are
excluded. It may require many medical tests before
a final diagnosis is made.
Treatment
The symptoms of
arthritis
are generally controlled with adequate doses of
salicylates (aspirin)
or nonsteroidal anti-inflammatory
medications (NSAIDs)
such as ibuprofen. Prednisone may be used for
more severe cases. In the disease becomes chronic
immunosuppressive medications might be needed.
These may include methotrexate or new biologic therapies. Biologic agents can
be very helpful in treating Still’s disease.
Particularly TNF agents such as Infliximab
(Remicade) and Entarecept
(Enbrel) and also a
different type on biologic agent called an IL-1
receptor antagonist called Anakinra
(Kineret) can control
patients with difficult disease.
Expectations
(prognosis)
Studies show that
about 20% of patients have all of the symptoms
go away in a year and never come back. About 30%
of patients have all of the symptoms go away, but they come back several times over the next years.
The rest of the patients (about 50%) will develop
a chronic arthritis.
Long
Term Complications can
include:
- arthritis
- liver disease
- spleen enlargement
- pericarditis
- pleural effusion
