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Year 3 - MSK > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

what is juvenile idiopathic arthritis?

A

Group of systemic inflammatory disorders affecting children below age of 16 years

The most commonly diagnosed Rheumatic disease in children

An important cause of disability and blindness

a type of arthritis that causes joint inflammation and stiffness for more than six weeks in a child aged 16 or younger.

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2
Q

what is the Etiology and Pathogenesis of JIA?

A
  • nJIA is an auto-immune disease.
  • Etiopathogenesis is multi-factorial and different from that of adult RA.
  • Strong subset-specific genetic markers may affect immune response.
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3
Q

what is the criteria for diagnosis?

A

Age of onset = < 16 years

Duration of disease = > 6weeks

Presence of arthritis = Joint swelling or 2 of the following:

  • Painful or limited joint motion
  • Tenderness
  • Warmth
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4
Q

Clinical subtypes of JIA: After 6 months 3 major subtypescan be identified

Which will help to suspect:

  • The natural history
  • Complications
  • Prognosis
  • Decide the strategy of treatment

what are the different subtypes?

A
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5
Q

summary table of the different types:

A
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6
Q

what is and how common is Pauciarticular JIA?

A

4 or less joints

Oligoarticular juvenile idiopathic arthritis (formerly called pauciarthritis or pauciarticular-onset juvenile rheumatoid arthritis) is defined as juvenile idiopathic arthritis (JIA) involving fewer than five joints. It is the most common subgroup, constituting approximately 50 percent of cases of JIA

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7
Q

who does type 1 Pauciarticular JIA occur in?

A
  • Majority of pauci (25%)
  • Age: before 5 years, peak 1-3 years
  • Girls: boys = 8:1
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8
Q

what are the symptoms of type 1 Pauciarticular JIA?

A
  • Presentation: limp rather than pain
  • No constitutional manifestations
  • +ve ANA in 40-75%
  • Chronic uveitis in 20% of cases (95% if female < 2years old)
  • Asymptomatic in 50%
  • Irregular iris due to posterior synechiae
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9
Q

where does type 1 Pauciarticular JIA affect?

A
  • Mainly LL joints
  • Knee> ankle> hand or elbow (hip very rare).
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10
Q

who does type 2 pauciarticular JIA affect?

A
  • 15%
  • Age: after 8-9
  • Girls: boys = 1:7
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11
Q

what are the symptoms and presentation of type 2 pauciarticular JIA?

A
  • Presentation: constitutional rare, limp due to LL affection
  • Mainly LL joints: knee, ankle.
  • Hip can be affected early with rapid damage requiring THR early in life + enthesitis + many have sacroilliac joints and may evolve AS or spondyloarthritis
  • 20% difficult to classify to particular spondyloarthropathy group
  • Acute iridocyclitis in 10-20%
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12
Q

in type 2 pauciarticular JIA, those with HLA-B27 + back involvement will be categorized as what?

A

Juvenile Ankylosing spondylitis

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13
Q

Type 3 pauciarticular JIA affects who?

A
  • 15%
  • Age: any age during childhood
  • Girls: boys = 4:1
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14
Q

what are the symptoms and presentation of type 3 pauciarticular JIA?

A

Presentation:

  • constitutional rare
  • asymmetric UL and LL arthritis
  • dactylitis (severe inflammation of the finger and toe joints)
  • Arthritis can be very destructive
  • Family history of psoriasis in 40%
  • +/- nail pitting
  • These patients may develop psoriasis later in life
  • Chronic iridocyclitis in 10-20%.
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15
Q

whatis extended oligoarthritis?

A

30% of these presenting with pauciarticular JIA (less than4 joints involved) can go on to more severe polyarticular course

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16
Q

what is the seocnd most ocmmon type of JIA?

A
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17
Q

what are the 2 different types of Polyarticular JIA?

A

Polyarticular JIA RF negative and RF positive

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18
Q

who does polyarticular JIA RF negative occur in?

A
  • 15% of JIA
  • Age: any age, often early
  • Girls: boys = 9:1
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19
Q

what is the presentation of RF negative polyarticular JIA like?

A

Constitutional manifestations (low grade fever, malaise)

Hepato-splenomegaly

Mild anemia

Growth abnormalities

• Iridocyclitis rare

20
Q

where does RF negative polyarticular JIA affect?

A

• Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck

21
Q

who does rF positive polyarticular JIA occur in?

A
  • 10% of JIA
  • Age: late childhood (teens, 12-16 years)
  • Girls: boys = 7:1
22
Q

what is the presentation and symptoms of RF positive polyarticular JIA?

A

Constitutional manifestations (low grade fever, malaise, weight loss)

Anemia

Nodules

  • Can be complicated by sjogren’s, Felty or vasculitis, AR, pulmonary fibrosis, AAS, CTS.
  • Similar to adult RA but in a child
  • Erosions in x ray occur early
  • Iridocyclitis rare
23
Q

what is the leats common type of JIA?

A

Systemic onset JIA

The least common (20%)

but most serious short and long term morbidity and mortality

24
Q

what defines systemic onset JIA?

A

Extra-articular features define the disease

(start early and disappear after 2-5 years)

25
Q

what is another name for systemic onset JIA?

A

stills disease

26
Q

who does systemic onset JIA occur in?

A
  • 20% of JIA
  • Age: throughout childhood (4-6 years)
  • Girls: boys = 1.5:1
27
Q

what fever may occur in systemic onset JIA?

A
  • Rise to 39.5 C daily for at least 2 weeks
  • Late in afternoon or evening and returns to normal or subnormal in the morning
  • Child appears toxic with fever +/- chills but looks normal when fever goes away
28
Q

what rash may occur in systemic onset JIA?

A
  • 90%
  • Evanescent salmon red eruption
  • On trunk and thighs
  • Accompanies fever
  • Can be brought by scratching (+ve Koebner’s phenomenon)
29
Q

what lymph node problems may occur in systemic onset JIA?

A
  • 50-75%
  • Generalized lymphadenopathy
  • Non tender
30
Q

what abdominal problems may occur in systemic onset JIA?

A
  • Hepatosplenomegaly
  • 50-75%
  • Abdominal pain
  • +/- transaminases
31
Q

what serositis (inflammation of a serous membrane) may occur in systemic onset JIA?

A
  • Polyserositis
  • Pericarditis in 36%
  • Tamponade and myocarditis rare
32
Q

what pulmonary problems may occur in systemic onset JIA?

A
  • Rare
  • Pleural effusion
  • Pulmonary fibrosis
33
Q

what arthiritis may occur in systemic onset JIA?

A
  • 75%
  • Within 3-12 months of onset of fever
  • Wrists, knees, ankles, cervical spine, hips and TMJ
34
Q

What are some things to look out for in children with arthritis?

A
  • Sowllen knee
  • Children are bendy – hypermobile so an important clinical sign is a loss of this hypermobility
  • Jaw involvement – children growing so the effect on a growing skeleton is different from an adults skeleton, joint can be effect unilaterally or bilaterally
35
Q

when does uveitis occur in JIA?

A
36
Q

what does uveitis in JIA cause?

A
37
Q

what is 1st line therapy in JIA?

A
  • Simple pain killers
  • NSAIDs:

– Difference between adults and children half life

– Can control disease

– Doses

– Same compounds only

38
Q

what is 2nd line therapy in JIA?

A

If no response to NSAIDs/ joint (steroid) injections

  • Rarely needed in oligoarticular JIA
    (1) Methotrexate (pharmocokinetics is age related)
    (2) Anti-TNF Rx. (all 3): In methotrexate failure
    (3) IL-1 R-antagonist (Anakinra) in refractory systemic arthritis
    (4) IL-6 antagonist (Tocilizumab) for refractory systemic disease
39
Q

how are systemic steroid used in JIA?

A
  • Limited indications due to serious side effects
  • Used in:
  • Systemic JIA (control pain and fever)
  • Serious disease complications with any subtype e.g. periocordial effusion, tamponade, vasculitis, severe auto-immune anemia, severe aye disease
  • As a bridge between DMARDs
  • Children undergoing surgery
40
Q

whata re the risks of used systemic steroid in JIA?

A

•Risk of osteoporosis, infections, growth abnormalities

41
Q

another kind of manage is rehabilitation and that is done by who?

A
  • Physiotherapy
  • Occupational therapy
42
Q

when would local steroids be used?

A
  • Intra-articular mainly in Oligo-articular JIA
  • Eye disease (ANA +ve oligo-articular disease)
43
Q

what surgery may be done? (Try avoid this in young people)

A
  • Synovectomy
  • Reconstructive / joint replacement surgery
44
Q

what growth failure may occur in JIA?

A

Micrognathia is a condition in which the lower jaw is undersized

45
Q

Summary:

  • JIA is not ____
  • Subtypes guide management and prognosis
  • Eye screening for all patients _______ (more common in the ______ subtype
  • ANA is not _________
A

rare

important

oligos

diagnostic

Year 3 - MSK (45 decks)

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