Still's disease, sometimes referred to as Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash.

1. STILL’S DISEASE

2. Introduction…
Still's disease, sometimes referred to as Adult-Onset Still’s
Disease (AOSD) is a rare systemic inflammatory disease
characterized by the classic triad of
persistent high spiking fevers,
joint pain, and…
a distinctive salmon-colored bumpy rash.
The disease is considered a diagnosis of exclusion and sadly is
idiopathic.
There are no specific known risk factors for the development
of Still's disease as well as no known preventive measures.

3. What causes Still's disease?
There have been a number of schools of thought regarding
the cause (or causes) of Still's disease.
One concept is that Still's disease is due to infection with a
microbe. Another idea is that Still's disease is an
autoimmune disorder.
Some say it is of viral mutation leading back to autoimmune
disorders. In fact, the precise cause of Still's disease is not
yet known but with constant research and promising
advancements, the future looks bright.

4. Pathophysiology
However, as the cause remains unknown, it
presumably involves interleukin-1 (IL-1), since drugs
that block the action of IL-1β are effective treatments.
Interleukin-18 is expressed at high levels.
In regards to that, other anti-IL1β drugs are being
developed

5. How does Still's disease relate to
Juvenile Idiopathic Arthritis?
Still's disease is one type of Juvenile Idiopathic Arthritis (JIA) and is also known as
systemic-onset JIA. Still's disease accounts for 10%-20% of all cases of JIA.
Other types of JIA include
• Oligoarticular JIA
• Polyarticular JIA
Systemic-onset JIA was formerly known as systemic-onset juvenile rheumatoid
arthritis (JRA) and is the same disease.
It occurs mostly in adolescence typically affecting children under 16 years of age
hence ‘juvenile’
There is a bimodal age distribution with one peak incidence between ages 15–25
and a second peak between ages of 36–46 years.

6. Signs and Symptoms
Articular.
Arthritis occurs after rash and fevers have been present for some time.
There is eventual development of joint pain and swollen joints. This
usually involves many joints (poly-articular arthritis). Any joint can be
affected.

8. Extra- Articular
Still's disease usually begins with systemic symptoms as such:
Extreme fatigue.
High fevers that rise daily to 39 degree Celcius or even higher and
rapidly return to normal levels or below. Fever spikes often occur at
approximately the same time every day.
A faint salmon-colored skin rash characteristically comes and goes and
does not itch.
Poor appetite, nausea, and weight loss may occur.
Lymphadenopathy, splenomegaly, hepatomegaly, and sore throat are
also common.
Pericarditis, pleuritis, pericardial effusion and pleural effusion may
occur but is less likely. They present as underlying complications.

10. How is Still's disease diagnosed?
Diagnosis is hardly based upon serology but on the
basis of the typical aforementioned clinical features of
the illness.
However, many patients with Still's disease develop
markedly elevated white blood cell counts.
Typically, low red blood counts indicating anemia is
present.
Elevated ESR, C-reactive protein and ferritin are
common.
However, tests for rheumatoid factor and antinuclear
antibodies are usually negative.

11. Criteria for Diagnosis
Yamaguchi criteria for classification of Still's disease
There should be presence of 5 or more criteria, of which at least 2 are Major.
Major Criteria
Fever of >39°C for >1 week
Leukocytosis >10,000/microL with >80% polymononuclear leukocytes (predominating
granulocytes)
Salmon colored rash (usually over the trunk or extremities, non-pruritic)
Arthralgia and/or Arthritis >2 week
Minor Criteria
Sore throat
Lymphadenopathy
Hepatomegaly and/or Splenomegaly
Liver dysfunction (high AST/ALT)
Negative Anti Nuclear Antibodies and Rheumatoid Factor

12. Cush criteria for classification of Still's disease
Requires all of the following:
Fever > 39 degrees
Arthralgia and/or arthritis
Rheumatoid factor < 1:80
Anti Nuclear Antibodies < 1:100
In addition, any two of the following is sited for:
WBC count > 15,000
Stills rash
Pleuritis or Pericarditis
Hepatomegaly, Splenomegaly, or Lymphadenopathy

13. TREATMENT
Treatment of Still's disease is directed toward the individual
areas of inflammation.
Still's disease is treated with anti-inflammatory drugs.
Steroids such as prednisone are used to treat severe
symptoms.
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 hydroxychloroquine (Plaquenil), penicillamine
(Cuprimine, Depen), azathioprine (Imuran), methotrexate
(Rheumatrex, Trexall), and cyclophosphamide (Cytoxan).

14. Recently, because Still's disease has been found to involve a
specific chemical messenger of inflammation known as
interleukin 1 (IL-1), the injectable biologic medication
Anakinra (Kineret), which blocks IL-1, has been found to be a
very effective treatment for Still's disease.
Still’s disease also involves interleukin 6 (IL-6). Tocilizumab
(Actemra), an intravenous treatment which blocks IL-6, has
been approved to treat systemic JIA in children.

15. Iappreciateyour attention.
Adighibenma S.O.S