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*Paediatric - MSK > Juvenile idiopathic arthritis > Flashcards

Juvenile idiopathic arthritis Flashcards

1
Q

What is arthritis?

A

An inflammation of a joint which presents as pain and swelling

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2
Q

What is ‘Juvenile idiopathic arthritis’?

A

Arthritis starting before the age of 16 years and lasting > 3 months

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3
Q

What is the prevalance of ‘Juvenile idiopathic arthritis’?

A

1 in 1000 children

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4
Q

What is the pathophysiology of ‘Juvenile idiopathic arthritis’?

A

Autoimmune disease in which the immune system targets the synovial membrane lining the joint

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5
Q

What are the clinical features of Juvenile idiopathic arthritis?

A
  1. Pain, redness and swelling of the joint
  2. Morning stiffness
  3. Joint feels warm
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6
Q

Ix in JIA

A
  1. Antinuclear antibody (ANA) is positive especially in oligoarticular JIA
  2. Rheumatoid factor is usually negative
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7
Q

What are the 3 main classifications of ‘Juvenile idiopathic arthritis’?

A
  1. Systemic onset juvenile idiopathic arthritis 2. Oligoarthritis 3. Polyarthritis 4. Enthesitis related arthritis
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8
Q

What is the definition systemic onset juvenile idiopathic arthritis?

A
  1. Arthritis in which systemic symptoms are present and joint involvement maybe minimal
  2. Also known as ‘Still’s disease’
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9
Q

Who does systemic onset juvenile idiopathic arthritis normally effect?

A
  1. 10 - 15% of JA 2. Typically effects children < 5 years 3. Equal sex distribution before 5 years but mainly effects girls after 5
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10
Q

How does systemic onset juvenile idiopathic arthritis normally present? ( name 4 systemic symptoms )

A
  1. Starts with predominantly systemic features - Swinging fever - Salmon pink macular rash - tiredness - Organomegaly 2. Arthritis occurs secondary to the systemic symptoms
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11
Q

How does systemic onset juvenile idiopathic arthritis effect the joints? (2)

A
  1. Usually symmetrical 2. Mainly effects knees, wrists, hands and feet
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12
Q

What is ‘Macrophage activation syndrome’ ?

A
  1. Life threatening complication of sJIA
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13
Q

What are the features of ‘Macrophage activation syndrome’ ?

A
  1. Anaemia , Leukopenia , Thrombocytopenia
  2. Raised triglycerides
  3. Falling ESR
  4. Raised liver enzymes

5. RAISED FERRITIN ( > 100,00 )

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14
Q

What is the tx for ‘Macrophage activation syndrome’?

A

Treat underlying trigger plus high dose steroids

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15
Q

What is the definition of oligoarthritis?

A

Arthritis in which up to 4 joints are affected

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16
Q

What is the most common type of JIA?

A

Oligoarthritis

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17
Q

Who does oligoathritis most commonly effect?

A
  1. < 6 years
  2. Pre school girls
18
Q

How does oligoarthritis effect the joints?

A
  1. Asymmetrical 2. Knee, ankle and elbow most commonly effected
19
Q

What is the definition of polyarticular arthritis?

A

Arthritis in which 5 or more joints are involved

20
Q

Who does polyarticular arthritis most commonly effect?

A

Children > 8 years

Girls more commonly effected

21
Q

How does polyarticular arthritis effect the joints?

A
  1. Symmetrical
  2. Effects large and small joints
  3. Later involves the temporomandibular joints and cervical spine
22
Q

What is Enthesis related arthritis?

A

Inflammation at the attatchments of tendons or ligaments of the bone to the bone

  • Associated with HLAB27 antigen
23
Q

Name 2 subsects of ‘Enthesis related arthritis’

A
  1. Juvenile anklyosing spondylitis
  2. Psoriatic arthritis
24
Q

Enthesis related arthritis - Juvenile anklyosing spondylitis

(clinical features )

A
  1. Effects Boys > girls
  2. Initially oligoarticular
  3. Sacroilliac involvement develops gradually during adolescence
25
Q

Enthesis related arthritis - Psoriatic arthritis

A
  1. Monoarthritis usually involving the fingers
  2. Nail pitting
  3. Psoriatic rash
26
Q

How can you elicit morning stifness in young children?

A

Ask the parents if the child finds the first nappy change of the day particularly uncomfortable

27
Q

How is JIA diagnosed?

A
  1. Clinical diagnosis
  2. History to exclude septic arthritis or malignancy
  3. Genetic testing to exclude HLA - B27 antigen
28
Q

Name 4 diseases caused by HLA B 27 antigen?

A
  1. Enthesitis related arthritis
  2. Inflammatory bowel disease
  3. Uveitis
29
Q

What should be tested regularly in children with JIA?

A
  1. 1 in 5 children develop chronic anterior uveitis
  2. Untreated this can lead to glaucoma, cataracts and blindness
  3. Often assymtomatic
30
Q

What is the management of JIA?

A
  1. Pain management
  2. Physiotherapy - regular exercise
  3. Medication
31
Q

What kind of medication therapies are used to treat JIA?

(4)

A
  1. Main form of treatment are NSAIDs to ease the pain
  2. Disease modifing anti rheumatic drugs - Methotrexate
  3. Corticosteroid injections to the joint
  4. ‘Biologics’ and immunotherapies - TNF alpha inhibitors
32
Q

What is the prognosis of JIA?

A
  1. Majority of children enter remission
  2. 30% of affected children continue to have active disease into adulthood
  3. Even with remission, considerable joint damage has already ocurred leading to joint replacement surgery in young adults
33
Q

Ix in Paediatric MSK

A
  1. Exclude definable causes of arthritis e.g. septic
  2. sJIA - Leucocytosis / thrombocytosis
  3. ESR
  4. ANA - risk of uveitis
  5. Radiology ( US / Plain films / MRI )
34
Q

What is the risk in Oligoarthrtitis?

A
  1. Risk of silent uveitis
  2. Leg length discrepency
35
Q

What is the likelihood of oligoarthritis progression?

A

Polyarticular course in 30% of cases

36
Q

Blood test in oligoarthritis?

A

ANA positive

37
Q

Which condition can occur in Enthesitis related arthritis?

A

Acute painful iritis

38
Q

MDT approach for Paediatric MSK? (7)

A
  1. Paediatric rheumatology
  2. Nurse specialist
  3. Occupational therapist / physiotherapist
  4. Social worker
  5. Opthalmologist
  6. Podiatrist
  7. Psychoogist
39
Q

Drug Tx options (3 x classes )

A
  1. Anti inflammatory drugs
    - NSAIDS
    - Glucocorticoids
  2. Disease modifying drugs
    - Methotrexate
  3. Biologics
    - Anti TNF alpha
40
Q

What are indicators of poor prognosis?

A
  1. Polyarticular onset and course
  2. Rheumatoid factor positive girls
  3. Delay in starting effective treatment

*Paediatric - MSK (16 decks)

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