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Ortho and Rheum > Children Rheum - JIA > Flashcards

Children Rheum - JIA Flashcards

1
Q

Another name for arthrocentesis

A

Joint aspiration

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2
Q

What is Koebners phenomenon?

A

Linear erythema - skin lesions - appearing on line of trauma

eg. following pruritus

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3
Q

What is Juvenile Idiopathic arthritis (JIA)

A

An umbrella term used to describe at least 7 different conditions - each representing a different form of childhood arthritis

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4
Q

Key features of JIA

A 
Joint pain and swelling 
Diurnal variation (stiffness in morning and after periods of rest) 
Other rheumatic patterns of rash, fever, weakness, disease progression despite simple measures
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5
Q

What age of children are most commonly affected by JIA?

A

Preschool or early school years

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6
Q

What is most commonly affected joint in JIA

A

Knee
Can also be ankle or wrist
Rarer = small joint arthritis or axial (shoulder, hip, spine, TMJ)

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7
Q

What improves stiffness in JIA

A

Movement, warm bath or shower

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8
Q

How can knee joint swelling be identified in children? x4

A

Balloting synovial fluid
Palpating a fluid thrill on joint movement
Positive patella tap
Occasionally finding a Baker’s cyst in popliteal fossa

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9
Q

How can ankle joint swelling be demonstrated in children? x2

A

Swelling of ankle will distort contours of medial or lateral malleoli
Also on ankle dorsiflexion may not be able to see extensor tendons (may be difficult in infants or obese children)

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10
Q

Ways of seeing wrist arthritis in children? x2

A

Press palms of hands together in praying position - dorsal bulge and
Reduced range of movement - especially if asymmetrical

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11
Q

Signs of elbow swelling in children? x3

A

Swelling can usually be palpated either side of olecranon
Can also cause flexion deformity of elbow
Elbow swelling obscures posterior dimple created when elbow is fully extended

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12
Q

Signs of finger swelling x6

A

Joint margin tenderness, restricted movement, swelling, purplish discolouration, incomplete fist closure and diminished grip strength

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13
Q

Signs of cervical spine arthritis x2

A

Inability to rotate head laterally and place chin on each shoulder
Inability to extend cervical spine properly

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14
Q

Presentation of TMJ arthritis

A

Often missed

May prevent full and symmetrical opening of the mouth

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15
Q

Signs of sacroiliac joint involvement or enthesitis in JIA x2

A

Schober test - less than 6cm expansion of lumbar spine with forward bending
Tenderness of sacroiliac joints to direct palpation

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16
Q

Where does enthesopathy commonly affect in JIA

A

Achilles tendon

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17
Q

Other signs of systemic arthritis in JIA other than arthritis

A

Pink, macular, truncal rash - may be pruritic with Koebners
Lymphadenopathy
Hepatosplenomegaly
Myalgia
Arthritis might not be prominent initially

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18
Q

Arthritis which fit under umbrella term of JIA

A 
Systemic arthritis
Oligoarthritis 
Polyarthritis 
Psoriatic arthritis 
Enthesitis-related arthritis 
HLA-B27-related arthritis syndromes
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19
Q

Mechanical disorders which are DDX for JIA x3

A

Joint pain secondary to hypermobility
Trauma
NAI

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20
Q

Features of hypermobility joint pain

A

Younger children are generally more flexible, joints are mobile, girls>boys and black>white
May get pain after physical activity and in evenings (opposite to JIA)
Lower limbs and back
Examination shows 10-15degrees extra motion
Treat with orthotics

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21
Q

Infection-related disorders which are DDX for JIA x3

A

Reactive arthritis
Septic arthritis
Osteomyelitis

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22
Q

What is reactive arthritis?

A

Most common form of arthritis in children

Self-limited, acute and painful joint swelling following extra-articular infection

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23
Q

Features of septic arthritis

A

Almost exclusively monoarticular
Associated with “pseudoparalysis” of affected limb
Systemically ill child (high fever)

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24
Q

What is pseudoparalysis

A

Extreme pain with affected joint held rigidly in the position of maximum comfort

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25
Q

Treatment of septic arthritis

A

Joint aspiration for bacterial culture before treatment started - can also help reduce intraarticular pressure
Then antibiotics

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26
Q

Features of osteomyelitis

A

Children present with fever, bone pain and signs of toxicity

Extreme pain at sign of infection and cannot ambulate

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27
Q

Chronic rheumatic conditions as DDX for JIA x3

A

SLE
Juvenile dermatomyositis
Localised scleroderma syndromes

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28
Q

Typical presentation of SLE x4 features

A

In adolescent girl with malaise, fever and bone/joint pain

It is rare in prepubescent children

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29
Q

What sort of rash can be present in SLE?

A

Acneiform facial rash may be present along with typical malar rash (not a uniform finding)
Rash does not affect nasolabial folds

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30
Q

Other features of SLE found in most children x6

A 
Hair loss
Mouth sores
Lymphadenopathy 
Organomegaly 
Other rashes
Swollen joints
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31
Q

Antibody tests for SLE x5

A

ANAs are almost always positive
Double-stranded DNA and Sm antibodies - more specific for SLE
Antibodies to SSA (Ro) and SSB (La) and anti-cardiolipin are positive in less than 50% of paediatric SLE but associated with complications

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32
Q

Other haematological signs of SLE x3

A

Lymphopenia
Thrombocytopenia
Coombs positive anaemia

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33
Q

What can show on urinalysis in SLE

A

Proteinuria and casts - reflecting renal disease (more common in child-onset SLE than adult)

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34
Q

How does juvenile dermatomyositis present?

A

Insidiously with malaise, progressive muscle weakness and muscle pain/discomfort

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35
Q

What % of juvenile dermatomyositis have arthritis

A

20%

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36
Q

Dermatological features of dermatomyositis x4

A

Heliotrope rash (purplish discoloration and oedema of eyelids) Malar rash travelling down to nasolabial folds
Gottrons papules over MCP, elbows and knees
Cuticle hyperaemia

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37
Q

Serum signs of dermatomyositis

A

Elevated serum muscle enzymes, CPK aldolase, AST, ALT and LDH

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38
Q

Features of localised scleroderma syndromes

A

Localised disorder characterised by areas of oval or linear lesions that traverse over joints, face and trunk
Can also have frank arthritis

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39
Q

2 acute inflammatory DDX of JIA

A

HSP

Kawasaki disease

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40
Q

Features of HSP x5

A 
Purpuric rash over lower legs and buttocks
Cramping abdominal pain
Bloody stools 
Haematuria 
Arthritis of ankles and knees
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41
Q

Malignant DDX of JIA x4

A

Acute lymphoblastic leukaemia
Neuroblastoma
Lymphoma
Primary bone malignancies (rare)

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42
Q

ALL presentation similar to JIA

A

Can present with bone pain in children and even frank arthritis
Pain over affected bone

43
Q

Idiopathic pain syndromes as DDX for JIA x2

A

Complex regional pain syndrome

Diffuse MSK pain syndromes

44
Q

Why do you do investigations in JIA

A

JIA is a label for children who fulfil criteria based on clinical features alone - therefore investigations to exclude other DDX

45
Q

Why do you do FBC in JIA suspicion?

A

To look for leucopenia, Coomb’s positive anaemia and thrombocytopenia of SLE and MTCD
Also elevated WBC count and anaemic of chronic inflammation with systemic arthritis

46
Q

Why do you do ESR in JIA suspicion?

A

ESR elevated in systemic arthritis and SLE

Normal in up to 1/2 patients with JIA and in most with dermatomyositis

47
Q

Why do you do urinary catecholamines in JIA suspicion?

A

To exclude neuroblastoma

48
Q

Why do you do Liver function tests in JIA suspicion?

A

To rule out dermatomyositis

49
Q

Why do you do Antinuclear antibodies in JIA suspicion?

A

High ANAs seen in virtually all children with SLE and MCTD

Also positive ANA in oligoarthritis

50
Q

Why do you do IgG in JIA suspicion?

A

IgG highly elevated in SLE

51
Q

Why do you do Borrelia burgdorferi serology in JIA suspicion?

A

To rule out Lyme disease if there is a history of travel in endemic area

52
Q

Why do you do synovial fluid analysis in JIA suspicion?

A

Mandatory if suspected septic arthritis

53
Q

Why do you do radiology in JIA suspicion?

A

To rule out fractures, malignancy, look for joint effusions, bone dysplasia/neoplasia, avascular necrosis

54
Q

Features of systemic arthritis

A

Prominent extra-articular features of high fever, rash, myalgia, arthralgia and irritability
Pattern of arthritis can vary from several swollen joints to widespread polyarticular pattern

55
Q

Presentation age of systemic arthritis

A

Usually begins in early childhood - although it can occur at any age through to adulthood

56
Q

Laboratory studies in systemic arthritis

A

High WBC count
Severe anaemia, thrombocytosis
High ESR, CRP and ferritin

57
Q

Duration of systemic features in systemic arthritis?

A

They usually resolve after a few months but they may last indefinitely

58
Q

Prognosis of systemic arthritis

A

Very poor, erosions of joint and loss of motion

Also severe growth delay due to treatment with steroids

59
Q

What can occur with systemic arthritis?

A

Macrophage activation syndrome - carries a 10-15% mortality rate - IV steroids and cyclosporine may reverse rapid deterioration and DIC

60
Q

What does oligo- mean?

A

Sparse or few

61
Q

What % of JIA does oligoarthritis account for?

A

50%

62
Q

Features of oligoarthritis?

A

Knee most commonly affected joint
Otherwise healthy
20% have asymptomatic anterior uveitis

63
Q

Joints affected in oligoarthritis

A

Most commonly the knee then ankle and wrist. Hip rarely ever affected

64
Q

Sex ratio of oligoarthritis

A

Female:Male 5:1

65
Q

Features of eye problems in oligoarthritis

A

Chronic anterior uveitis - occurs in 20%
Clinically silent and insidiously progressive
Produces visual loss and blindness if not detected by slit lamp and treated eary

66
Q

Laboratory investigations in oligoarthritis

A

Frequently positive ANAs but all other examinations are normal

67
Q

Implications of oligoarthritis

A

Often localised growth disturbances - one leg longer than the other due to increased growth in the affected leg (presumbably due to chronic hyperaemia and increased blood supply to joint)
This leads to knee flexion contracture and atrophy of the muscle above the knee
Toddler with oligoarthritis will stand with affected leg bent

68
Q

What is extended oligoarthritis?

A

1/3 of children who have less than 4 joints affected in the first 6 months go on to continue to develop arthritis in further joints afterwards

69
Q

What do children with extended oligoarthritis often have

A

Anterior uveitis

70
Q

What are the two types of polyarthritis?

A

Rheumatoid-negative and rheumatoid-positive

71
Q

Features of rheumatoid negative polyarthritis

A

Usually affects preschool girls

Predominantly symmetrical arthritis of upper and lower limbs

72
Q

What can happen with rheumatoid negative polyarthritis x2

A

Rare but important - growth restriction and chronic anterior uveitis

73
Q

Prognosis of rheumatoid negative polyarthritis?

A

Illness lasts most of childhood and many go on to adulthood with the disease

74
Q

Laboratory findings with rheumatoid negative polyarthritis

A

May have mild anaemia
Usually positive ANAs
ESR and CRP may be mildly elevated

75
Q

What % of JIA is due to rheumatoid negative polyarthritis?

A

25-30%

76
Q

What type of disease is rheumatoid positive polyarthritis

A

Similar in features and prognosis to adult RA - therefore can be called juvenile rheumatoid arthritis

77
Q

Presentation age of rheumatoid positive polyarthritis

A

Usually in late childhood or adolescence

78
Q

Gender ratio of rheumatoid positive polyarthritis

A

Primarily affects girls

79
Q

Features of rheumatoid positive polyarthritis

A

Like rheumatoid arthritis
can be rapidly progressive and destructive
Rheumatoid nodules are common
Failure to thrive more frequent than in rh- polyarthritis

80
Q

Laboratory findings in rheumatoid positive polyarthritis

A

ANAs are usually positive

81
Q

Features of psoriatic arthritis in children

A

Arthritis may pre-date any classical skin findings by many years
Often asymmetrical arthritis, affects both small and large joints

82
Q

What establishes the diagnosis of psoriatic arthritis

A

Family history of first degree relative with psoriasis

83
Q

What can also affect children with psoriatic arthritis?

A

Asymptomatic uveitis - high risk of blindness if undetected

84
Q

Features of enthesitis-related arthritis x3

A

Lower limb arthritis initially
Complicated by enthesiitis of plantar fascia, achilles tendon and around patella
Can also get uveitis but usually symptomatic with red eyes, photophobia and pain

85
Q

Age and gender of children with enthesitis-related arthritis

A

Usually after 6 years old and boys>girls

86
Q

What is enthesitis-related arthritis a precursor to?

A

Ankylosing spondylitis

87
Q

What does treatment of arthritis in children usually start with? Details of this therapy

A

Usually starts with NSAIDs - used in higher doses relative to body weight than in adults because children have increased metabolism and renal excretion

88
Q

Adverse effects of NSAIDs treatment in JIA

A

Abdominal pain, change in mood, rarely bronchospasm

89
Q

Success of NSAIDs in JIA?

A

Majority of patients with early JIA do not respond to NSAIDs and need more aggressive treatment

90
Q

What can be used for single joint involvement in JIA?

A

Intra-articular corticosteroids
Usually down under GA in UK
Often used in oligoarthritis

91
Q

What is generally avoided treatment wise for JIA?

A

Systemic oral steroids because of side effects on growth and bones (avascular necrosis), and cushings
If it is needed in large pulses to control systemic disease - can also give GH to counteract growth restriction

92
Q

Eg. of two slow-acting anti-rheumatic drugs used in JIA

A

Methotrexate and sulfasalazine

93
Q

What is methotrexate effective at treating in JIA?

A

Effective in approx 70% of children with polyarthritis

Much less effective in systemic arthritis

94
Q

When is methotrexate considered in JIA?

A

If disease not controlled with NSAIDs or intra-articular steroids after 4-12week trial

95
Q

How long after starting can efficacy of methotrexate be determined?

A

1-3 months

96
Q

Most common side effects of methotrexate

A

Nausea and mouth ulcers (ondansetron and folate)

97
Q

Other side effects of methotrexate

A

Abdominal pain, elevated LFTs

Rarely hair loss and bone-marrow suppression

98
Q

What is a possible substitute for methotrexate in JIA?

A

Leflunomide - same efficacy and less toxicity

99
Q

When is sulfasalazine effective?

A

In oligoarthritis and polyarthritis - especially effective in ERA
Not much value in SA and increased risk of macrophage activated syndrome

100
Q

What have revolutionised JIA treatment? 2 eg

A

Biologics - TNFa-antagonists

Etanercept and Infliximab

101
Q

When can etanercept be used in JIA?

A

Children whose disease is not controlled by methotrexate or are intolerant of it
Very effective treatment

102
Q

Infliximab use in JIA?

A

Not labelled yet for JIA use but is being increasing used as it is highly effective - used if fail etanercept
Used in combination with methotrexate to minimise risk of immune reactions
Or if uveitis

103
Q

Hydroxychloroquine use in JIA?

A

Use in children is not supported - studies not shown to be effective

Ortho and Rheum (13 decks)

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