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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
Enthesis related arthritis - Psoriatic arthritis
1. Monoarthritis usually involving the fingers 2. Nail pitting 3. Psoriatic rash
26
How can you elicit morning stifness in young children?
Ask the parents if the child finds the first nappy change of the day particularly uncomfortable
27
How is JIA diagnosed?
1. Clinical diagnosis 2. History to exclude septic arthritis or malignancy 3. Genetic testing to exclude HLA - B27 antigen
28
Name 4 diseases caused by HLA B 27 antigen?
1. Enthesitis related arthritis 2. Inflammatory bowel disease 3. Uveitis
29
What should be tested regularly in children with JIA?
1. 1 in 5 children develop chronic anterior uveitis 2. Untreated this can lead to glaucoma, cataracts and blindness 3. Often assymtomatic
30
What is the management of JIA?
1. Pain management 2. Physiotherapy - regular exercise 3. Medication
31
What kind of medication therapies are used to treat JIA? (4)
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
What is the prognosis of JIA?
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
Ix in Paediatric MSK
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
What is the risk in Oligoarthrtitis?
1. Risk of silent uveitis 2. Leg length discrepency
35
What is the likelihood of oligoarthritis progression?
Polyarticular course in 30% of cases
36
Blood test in oligoarthritis?
ANA positive
37
Which condition can occur in Enthesitis related arthritis?
Acute painful iritis
38
MDT approach for Paediatric MSK? (7)
1. Paediatric rheumatology 2. Nurse specialist 3. Occupational therapist / physiotherapist 4. Social worker 5. Opthalmologist 6. Podiatrist 7. Psychoogist
39
Drug Tx options (3 x classes )
1. Anti inflammatory drugs - NSAIDS - Glucocorticoids 2. Disease modifying drugs - Methotrexate 3. Biologics - Anti TNF alpha
40
What are indicators of poor prognosis?
1. Polyarticular onset and course 2. Rheumatoid factor positive girls 3. Delay in starting effective treatment

*Paediatric - MSK (16 decks)

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