6.4. Rheumatology - Juvenile Idiopathic Arthritis Flashcards

1
Q

What is Juvenile Idiopathic Arthritis?

A

A group of systemic inflammatory disorders affecting children below the age of 16 years

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2
Q

What is the most common Rheumatic Disease in Children?

A

Juvenile Idiopathic Arthritis

Note - This is an important cause of disability and blindness

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3
Q

What type of disease is Juvenile Idiopathic Arthritis?

A

Autoimmune

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4
Q

What is the Aetiopathogenesis of Juvenile Idiopathic Arthritis?

A
Multifactorial:
1. Genetic
2. Environmental
3. Immunological
Note - This is different from Adult Rheumatoid Arthritis
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5
Q

What is the pathology of Juvenile Idiopathic Arthritis?

A

Environmental Factors act on Strong Subset-Specific Genetic Markers, which may affect the Immune Response

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6
Q

What is the Criteria for Diagnosis of Juvenile Idiopathic Arthritis?

A
  1. Age of Onset (<16 years old)
  2. Duration of the Disease (>6 weeks)
  3. Presence of Arthritis
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7
Q

In the Criteria for Diagnosis of Juvenile Idiopathic Arthritis, what confirms the presence of Arthritis?

A
Joint Swelling
Or 2 of the following:
1. Painful or Limited Joint Motion
2. Tenderness
3. Warmth
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8
Q

How many Clinical Subtypes of Juvenile Idiopathic Arthritis are there?

A

3

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9
Q

What are the 3 Clinical Subtypes of Juvenile Idiopathic Arthritis?

A
  1. Pauciarticular (55%)
  2. Polyarticular (25%)
  3. Systemic Onset (20%)
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10
Q

What can the Clinical Subtype of Juvenile Idiopathic Arthritis help us suspect?

A
  1. The Natural History
  2. Complications
  3. Prognosis
  4. Decide the Strategy of Treatment
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11
Q

What does Pauciarticular Juvenile Idiopathic Arthritis involve?

A

Rheumatoid Arthritis of 4 or less Joints

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12
Q

How many Types of Pauciarticular Juvenile Idiopathic Arthritis are there?

A

3:

  1. Type 1 Pauciarticular Juvenile Idiopathic Arthritis
  2. Type 2 Pauciarticular Juvenile Idiopathic Arthritis
  3. Type 3 Pauciarticular Juvenile Idiopathic Arthritis
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13
Q

What is the percentage breakdown of each of the Main Types of Pauciarticular Juvenile Idiopathic Arthritis?

A
  1. Type 1 - 25%
  2. Type 2 - 15%
  3. Type 3 - 15%
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14
Q

How does Type 1 Pauciarticular Juvenile Idiopathic Arthritis commonly present?

A
  1. With a limp rather than pain
  2. No other Constitutional Manifestations
  3. Chronic Uveitis in 20% of cases
  4. Asymptomatic in 50% of cases
  5. Irregular Iris due to Posterior Synechiae
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15
Q

Which Joints are most commonly affected in Type 1 Pauciarticular Juvenile Idiopathic Arthritis?

A

Mainly Lower Limb Joints (most common down):

  1. Knee
  2. Ankle
  3. Hand (Rare)
  4. Elbow (Rare)
  5. Hip (Very Rare)
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16
Q

Who commonly gets Type 1 Pauciarticular Juvenile Idiopathic Arthritis?

A
  1. Girls (8:1)

2. Younger than 5 years old

17
Q

Who commonly gets Type 2 Pauciarticular Juvenile Idiopathic Arthritis?

A
  1. Boys (7:1)

2. Ages after 8 / 9

18
Q

What condition is seen in 10-20% of Type 2 Pauciarticular Juvenile Idiopathic Arthritis patients?

A

Acute Iridocyclitis (Inflammation of the Iris)

19
Q

How does Type 2 Pauciarticular Juvenile Idiopathic Arthritis commonly present?

A
  1. Limp due to Lower Limb affection

2. Consitutional Symptoms are Rare

20
Q

Which Joints are most commonly affected in Type 2 Pauciarticular Juvenile Idiopathic Arthritis?

A

Mainly Lower Limb:

  1. Knee
  2. Ankle
  3. Hip
21
Q

How are those with HLA-B27 Positive Back Involvement categorised as?

A

Juvenile Ankylosing Spondylitis

22
Q

How does Type 3 Pauciarticular Juvenile Idiopathic Arthritis present?

A
  1. Asymmetric Upper Limb and Lower Limb Arthritis
  2. Dactylitis (Inflammation of the Toes / Fingers)
  3. Constitutional Symptoms are Rare
23
Q

Who commonly gets Type 3 Pauciarticular Juvenile Idiopathic Arthritis?

A
  1. Girls (4:1)

2. This develops at Any Age (Before 16)

24
Q

What conditions are associated with Type 3 Pauciarticular Juvenile Idiopathic Arthritis?

A
  1. Chronic Iridocyclitis in 10-20% of patients (Inflammation of the Iris)
  2. Psoriasis (Later in life)
    Note - this condition is more likely with a Family History of Psoriasis
25
Q

What percentage of Pauciarticular Juvenile Idiopathic Arthritis go on to develop Polyarticular Juvenile Idiopathic Arthritis?

A

30%

26
Q

How many Joints are involved in Polyarticular Juvenile Idiopathic Arthritis?

A

5 or more

27
Q

What are the 2 Sub-types of Polyarticular Juvenile Idiopathic Arthritis?

A
  1. Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis
  2. Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis
28
Q

How does Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis present?

A
  1. Symmetric Large and Small Joints affection (Knee, Wrist, Ankle, MCP, PIP, Neck)
  2. Consitutional Manifestations (Fever, Malaise…)
  3. Hepato-Splenomegaly
  4. Mild Anaemia
  5. Growth Abnormalities
  6. Iridocyclitis (Inflammation of the Iris) (Rare)
29
Q

Who commonly gets Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis?

A
  1. Girls (9:1)
  2. This can present at any age (below 16)
    Note - This accounts for 15% of all Juvenile Idiopathic Arthritis
30
Q

How does Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis present?

A

Similar to Adult Rheumatoid Arthritis:

  1. Constitutional Manifestations (Fever, Malaise…)
  2. Anaemia
  3. Nodules
  4. Iridocyclitis (Inflammation of the Iris) (Rare)
  5. Erosions in X-Ray occur early
31
Q

Who commonly gets Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis?

A
  1. Girls (7:1)
  2. In late Childhood (12-16 years)
    Note - This accounts for 10% of all Juvenile Idiopathic Arthritis
32
Q

What is the most Serious type of Juvenile Idiopathic Arthritis?

A

Systemic Onset of Juvenile Idiopathic Arthritis

Note - This has short and long term morbidity and mortality

33
Q

Who commonly gets Systemic Onset of Juvenile Idiopathic Arthritis?

A
  1. Girls (3:2)

2. At any age throughout childhood but mainly at 4-6 years olf

34
Q

What Joints does Systemic Onset of Juvenile Idiopathic Arthritis commonly affect?

A
  1. Wrists
  2. Knees
  3. Ankles
  4. Cervical Spine
  5. Hips
  6. TMJ
35
Q

How does Systemic Onset of Juvenile Idiopathic Arthritis present?

A
  1. Arthritis
  2. Fever (within 3-12 months of onset)
  3. Salmon Red Eruption on the Trunk / Thighs
  4. Polyseositis (Pleural Effusion / Pulmonary Fibrosis / Pericarditis)
  5. Lymphadenopathy
  6. Abdominal Pain (+/- Transaminases)
36
Q

What is the Therapy for Juvenile Idiopathic Arthritis?

A
  1. Simple Pain Killers (NSAID’s)
  2. Joint (Steroid) Injections
  3. Methotrexate
  4. Biologics (Anti-TNF, IL-1 Antagonist, IL-6 Antagonist)
  5. Local / Systemic Steroids
  6. Rehabilitation / Occupational Therapy
  7. Surgical (Synovectomy / Reconstructive)