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
What percentage of Pauciarticular Juvenile Idiopathic Arthritis go on to develop Polyarticular Juvenile Idiopathic Arthritis?
30%
26
How many Joints are involved in Polyarticular Juvenile Idiopathic Arthritis?
5 or more
27
What are the 2 Sub-types of Polyarticular Juvenile Idiopathic Arthritis?
1. Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis 2. Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis
28
How does Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis present?
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
Who commonly gets Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis?
1. Girls (9:1) 2. This can present at any age (below 16) Note - This accounts for 15% of all Juvenile Idiopathic Arthritis
30
How does Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis present?
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
Who commonly gets Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis?
1. Girls (7:1) 2. In late Childhood (12-16 years) Note - This accounts for 10% of all Juvenile Idiopathic Arthritis
32
What is the most Serious type of Juvenile Idiopathic Arthritis?
Systemic Onset of Juvenile Idiopathic Arthritis | Note - This has short and long term morbidity and mortality
33
Who commonly gets Systemic Onset of Juvenile Idiopathic Arthritis?
1. Girls (3:2) | 2. At any age throughout childhood but mainly at 4-6 years olf
34
What Joints does Systemic Onset of Juvenile Idiopathic Arthritis commonly affect?
1. Wrists 2. Knees 3. Ankles 4. Cervical Spine 5. Hips 6. TMJ
35
How does Systemic Onset of Juvenile Idiopathic Arthritis present?
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
What is the Therapy for Juvenile Idiopathic Arthritis?
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)