Rheumatology Flashcards

1
Q

What is the most common chronic rheumatological disease in children?

A

Juvenile idiopathic arthritis

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2
Q

What is the incidence of JIA?

A

2-23 per 100,000

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3
Q

Why does JIA occur?

A
  • Not completely understood
  • Genetic susceptibility
  • Immune response
  • Release of pro-inflammatory markers including TNF, interleukin 1+2
  • Presence of antibodies
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4
Q

How do children with JIA present?

A
  • Arthritis for at least 6 weeks
  • Morning stiffness or gelling
  • Irritability or refusal to walk in toddlers
  • School absence or limited ability to participate in physical activity
  • Rash /fever
  • Fatigue
  • Poor appetite/wt loss
  • Delayed puberty
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5
Q

What is the differential diagnosis for JIA?

A
  • Septic arthritis
  • Osteomyelitis
  • Transient synovitis
  • Malignancies i.e lymphoma, neuroblastoma, bone tumours
  • Recurrent haemarthrosis
  • Vascular abnormalities
  • Trauma
  • Others
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6
Q

What are the signs of JIA?

A
  • Swelling: periarticular soft tissue oedema, intraarticular effusion, hypertrophy of synovial membrane
  • Tenosynovitis (swollen tendons)
  • Joint held in position of maximum comfort
  • Range of motion limited at extremes
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7
Q

What classification system is used for JIA?

A

ILAR classification

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8
Q

What types of JIA are there?

A
  • Polyarticular
  • Oligoarthritic/Pauciarticular
  • Psoriatic
  • Enthesitis-Related
  • Systemic
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9
Q

What is oligoarthritic/pauciarticular JIA?

A
  • 4 or fewer joints

- Large joints and asymmetrical

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10
Q

What are the 2 types of oligoarhr JIA?

A
  • Early childhood onset (more common)

- Late childhood onset

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11
Q

Who does early childhood onset oligoarthritic JIA usually affect?

A

Girls aged 1-5

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12
Q

Who does late childhood onset oligoarthritic JIA usually affect?

A

Boys over 8

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13
Q

What are the features of late childhood onset oligoarthritic JIA?

A
  • Test negative for ANA
  • No extra-articular manifestation
  • Hip involvement
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14
Q

What are the features of early onset oligoarthritic JIA?

A
  • 20-30% develop uveitis
  • Test positive for ANA
  • Joints commonly affected include: knees, ankles, hands, feet and wrists
  • No hip involvement
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15
Q

What is polyarticular JIA?

A
  • 5 or more joints affected

- Few or no systemic manifestations of disease

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16
Q

What are the 2 types of polyarticular JIA?

A
  • Seronegative (20-25%)

- Seropositive (5-10%)

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17
Q

Who is usually affected by seronegative polyarticular JIA?

A

Girls under 5

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18
Q

Who is usually affected by seropositive polyarticular JIA?

A

Girls over 8

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19
Q

What are the features of polyarticular JIA?

A
  • Tempomandibular joint injury is common leading to limited bite and micrognathia
  • Systemic manifestations are rare but can include fever, slight hepatomegaly, lymphadenopathy, pericarditis and chronic uveitis
  • Onset can be acute but mostly insidious
  • Large fast growing joints are mostly affected
20
Q

What is enthesitis-related JIA?

A

Inflammation of the enthesis along with arthritis

21
Q

What is the criteria fro enthsitis-related JIA?

A

2 or more

  • Onset of oligoarthritis/polyarthritis in a boy >8 years
  • HLA B27 positive
  • Acute anterior uveitis
  • Inflammatory spinal pain
  • Sacroiliac joint tenderness
  • FMH
22
Q

What is psoriatic JIA?

A

Chronic arthritis and definite psoriasis is evident

23
Q

What is always positive in psoriatic JIA?

24
Q

What is the criteria fro psoriatic JIA?

A

2 or more:

  • FMH
  • Dactylitis: finger or toe swelling
  • Onycholysis: nail pitting
25
What proportion of JIA does systemic JIA account for?
5-15%
26
How does systemic JIA present?
- Child unwell - Arthritis - Intermittent fever > 2 weeks (spikes once a day) - Salmon pink erythematous rash - Generalised lymphadenopathy - Serositis - Hepatomegaly/splenomegaly - High inflammatory markers
27
How is JIA diagnosed?
History Physical examination findings -pGALS Investigations - Labs - Plain x-ray - US - MRI with contrast
28
What does pharmacological treatment of JIA include?
- NSIADs - DMARDs - Biologic agents - Intra-articular and oral steroids
29
What non-pharmacological management is there for JIA?
- Counselling for patients and parents - School adjustments (including PE) - Nutrition (to address anaemia and osteoporosis) - Physiotherapy - Occupational therapy
30
How effective are intra-articular steroids?
- Highly efficacious - Remission >6 months in 84% - Greater success in oligoarthritis JIA - Safe and effective - No long term side effects
31
What is the DMARD of choice?
Methotrexate
32
When are DMARDs used in JIA?
- Usually in poor response to IAS in oligo JIA | - They are commonly used (not many side effects)
33
When should DMARDs be used?
Early for good outcome
34
How are DMARDs given?
Injectable SC form
35
What is necessary when on DMARDs?
Blood monitoring
36
When are biologic agents used?
When there is failure to respond to DMARDs
37
What biologics are commonly used?
Anti-TNF agents
38
What can uveitis associated with JIA progress to if left untreated?
- Chronic uveitis | - Cataracts, glaucoma and blindness
39
How is uveitis complications prevented in JIA?
- All children diagnosed with JIA undergo regular screening | - Early detection prevents complications
40
When is uveitis more common?
In ANA positive oligo JIA
41
How does uveitis present?
- Normally <5 years - Rarely symptomatic (incidental finding at screening) - Red eyes, headache and reduced vision
42
How are children examined for uveitis?
Slit light examination
43
How is uveitis treated in JIA?
- Initially topical steroids to reduce inflammation - More severe need systemic steroids - Poor response to steroids then DMARD and biologics
44
When are children with JIA screened for uveitis?
- Should be seen within 6 weeks of diagnosis | - High risk children screened more often
45
What are the possible complications of JIA?
- Poor growth - Localised growth disturbance - Micrognathia - Contractures - Ocular complications