introduction, prevalence, symptoms, diagnosis, treatment goals and complications

1. STILL ‘S DISEASE
 Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other
signs of systemic inflammation.
 Historically, sJIA was named Still's diseaseafter George Frederic Still, who first
reported patients.
 Individuals who manifestafter the 16th birthday arediagnosed with adult onset Still's
disease(AOSD).
 The pathophysiology of sJIA and AOSD areincompletely understood.
 Increased activation of inflammasomes and the expression of proinflammatory
cytokines play a central role.
 S100 proteins, which can activate Toll-like receptors, thus, maintaining positive
feedback loops, have also been detected at increased levels in sera fromsJIA patients.
 Reduced expression of the immune-modulatory cytokineIL-10 may further contribute
to immune cell activation and the production of proinflammatory molecules.
INTRODUCTION AND DEFINITION:
 Still’s diseaseis a type of inflammatory arthritis that causes fevers, rashes, and
inflammation of joints and, sometimes, internal organs.
 Still’s is also called systemic-onsetjuvenileidiopathic arthritis (SJIA), as itwas first
diagnosed in children.
 SJIA is a type of juvenile idiopathic arthritis (JIA), a group of arthritis conditions affecting
children.
 Still’s disease can also occur in adults and is called adult-onsetStill’s disease(AOSD).
While there is no curefor this debilitating condition, medication and a healthy lifestyle can
help with reducing inflammation and managing symptoms

2. STILL ‘S DISEASE
Prevalence
 JIA affects one in 1,000 children and it is estimated that around 300,000children in the
United States havebeen diagnosed with JIA.
 SJIA accounts for 10 percent of children with JIA.1
 Adult-onsetStill’s disease is quite rare.
 Itis believed to affect fever than one in every 100,000 people.
 Itaffects men and women equally, and mostdiagnoses are in young adults ages 16 to
35. The true number of people living with AOSD is unknown becausethe condition is
easily mistaken for other types of inflammatory arthritis due to its rarity and variable
symptomology
Symptoms
Still’s diseasecauses systemic (whole body) symptoms in both adults and children. The
most common symptoms of this condition include:
Fever:
Still’s causes daily fevers of at least 102 degrees Fahrenheit lasting a week or more at a
time. Fever spikes tend to occur in the afternoon and early evening and at least once or
twice daily, with temperatures returning to normalbetween fevers.

3. STILL ‘S DISEASE
Rash:
A salmon color rash may accompany fevers. The rash will appear on the trunk, arms,
and/or legs. These rashes only last for the length of the fever and will present on
different parts of the body with incidences of fever.
Sore throat:
In adults, this is one of the earliest signs of Still’s disease. Both adults and children will
experience tender and swollen lymph nodes in the neck.
Sore joints:
Joints can become inflamed and feel painful and stiff. The knees and wrists are the
most commonly affected, but other joints can become inflamed as well.
Muscle pain:
Muscle pain comes with fevers. Muscle pain can be severeand disruptthe simplest
activities of daily living.
 Children may also develop arthritis in the spine, jaws, and hip joints.
These are areas where SJIA can causethe most destruction and requirejoint
replacement surgeries. Themore joints that are affected, the more severeSJIA will be.
About a quarter of the children with SJIA will develop chronic polyarthritis (arthritis
affecting five or more joints) continuing into adulthood.
GrowthIssues inChildren:
 SJIA can cause growth issues in children. When joints are severely affected,
growth may be slowed, especially in the arms and legs. Sometimes, this is the
diseaseitself and, other times, this is related to medications for treating the
condition.
Rarer Symptoms:
 Enlarged liver or spleen
 Inflammation in the heart lining, a condition called pericarditis
 Inflammation of the lungs, called pleuritis
 Abdominal pain and swelling

4. STILL ‘S DISEASE
 Pain with breathing
 Weight loss without any effort
Causes
 Unknown.
 Some research suggests Still’s may be triggered by ongoing infections, including
microbes. Microbes are anything small, such as bacteria, fungi, and yeasts.
 Other research suggests Still’s disease is an autoimmune disease. Autoimmune
diseases are conditions where the immune systemattacks its own healthy
tissues.
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.
 many patients with Still's diseasedevelop
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.

5. STILL ‘S DISEASE
Criteriafor Diagnosis
Yamaguchi criteria for classification of Still's disease
There should be presenceof 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
Sorethroat
Lymphadenopathy
Hepatomegaly and/or Splenomegaly
Liver dysfunction (high AST/ALT)
Negative Anti Nuclear Antibodies and Rheumatoid Factor
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

6. STILL ‘S DISEASE
Treatment:
Goals:
 To control symptoms and slow down the disease in order to preventor
lessen complications.
Early treatment usually involves prevention of inflammation.
 nonsteroidalanti-inflammatory drugs (NSAIDs), including ibuprofen
and naproxen.
 Corticosteroids, such as prednisone,
 If symptoms persist, immunosuppressantdrugs area second-line
therapy.such as methotrexate and hydroxychloroquine.
 More recently, Still’s disease has been found to involve substances
Complications
 Potential complications of Still’s disease include:6
 Joint destruction due to chronic inflammation
 Inflammation of the heart
 Excess fluid around lungs
 Macrophageactivation syndrome(MAS). This diseasecomplication is
rare, but could be fatal. MAS may causelow blood cell counts,
extremely high triglyceride(type of fat found in the blood) levels, and
abnormalliver function. That causes inflammation, including a
cytokine protein, interleukin.
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