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Systems: Paediatrics AB > Rheumatology > Flashcards

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?

A

HLA B27

24
Q

What is the criteria fro psoriatic JIA?

A

2 or more:

  • FMH
  • Dactylitis: finger or toe swelling
  • Onycholysis: nail pitting
25
Q

What proportion of JIA does systemic JIA account for?

A

5-15%

26
Q

How does systemic JIA present?

A
  • Child unwell
  • Arthritis
  • Intermittent fever > 2 weeks (spikes once a day)
  • Salmon pink erythematous rash
  • Generalised lymphadenopathy
  • Serositis
  • Hepatomegaly/splenomegaly
  • High inflammatory markers
27
Q

How is JIA diagnosed?

A

History

Physical examination findings
-pGALS

Investigations

  • Labs
  • Plain x-ray
  • US
  • MRI with contrast
28
Q

What does pharmacological treatment of JIA include?

A
  • NSIADs
  • DMARDs
  • Biologic agents
  • Intra-articular and oral steroids
29
Q

What non-pharmacological management is there for JIA?

A
  • Counselling for patients and parents
  • School adjustments (including PE)
  • Nutrition (to address anaemia and osteoporosis)
  • Physiotherapy
  • Occupational therapy
30
Q

How effective are intra-articular steroids?

A
  • Highly efficacious
  • Remission >6 months in 84%
  • Greater success in oligoarthritis JIA
  • Safe and effective
  • No long term side effects
31
Q

What is the DMARD of choice?

A

Methotrexate

32
Q

When are DMARDs used in JIA?

A
  • Usually in poor response to IAS in oligo JIA

- They are commonly used (not many side effects)

33
Q

When should DMARDs be used?

A

Early for good outcome

34
Q

How are DMARDs given?

A

Injectable SC form

35
Q

What is necessary when on DMARDs?

A

Blood monitoring

36
Q

When are biologic agents used?

A

When there is failure to respond to DMARDs

37
Q

What biologics are commonly used?

A

Anti-TNF agents

38
Q

What can uveitis associated with JIA progress to if left untreated?

A
  • Chronic uveitis

- Cataracts, glaucoma and blindness

39
Q

How is uveitis complications prevented in JIA?

A
  • All children diagnosed with JIA undergo regular screening

- Early detection prevents complications

40
Q

When is uveitis more common?

A

In ANA positive oligo JIA

41
Q

How does uveitis present?

A
  • Normally <5 years
  • Rarely symptomatic (incidental finding at screening)
  • Red eyes, headache and reduced vision
42
Q

How are children examined for uveitis?

A

Slit light examination

43
Q

How is uveitis treated in JIA?

A
  • Initially topical steroids to reduce inflammation
  • More severe need systemic steroids
  • Poor response to steroids then DMARD and biologics
44
Q

When are children with JIA screened for uveitis?

A
  • Should be seen within 6 weeks of diagnosis

- High risk children screened more often

45
Q

What are the possible complications of JIA?

A
  • Poor growth
  • Localised growth disturbance
  • Micrognathia
  • Contractures
  • Ocular complications

Systems: Paediatrics AB (31 decks)

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