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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
Treatment of septic arthritis
Joint aspiration for bacterial culture before treatment started - can also help reduce intraarticular pressure Then antibiotics
26
Features of osteomyelitis
Children present with fever, bone pain and signs of toxicity | Extreme pain at sign of infection and cannot ambulate
27
Chronic rheumatic conditions as DDX for JIA x3
SLE Juvenile dermatomyositis Localised scleroderma syndromes
28
Typical presentation of SLE x4 features
In adolescent girl with malaise, fever and bone/joint pain | It is rare in prepubescent children
29
What sort of rash can be present in SLE?
Acneiform facial rash may be present along with typical malar rash (not a uniform finding) Rash does not affect nasolabial folds
30
Other features of SLE found in most children x6
``` Hair loss Mouth sores Lymphadenopathy Organomegaly Other rashes Swollen joints ```
31
Antibody tests for SLE x5
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
32
Other haematological signs of SLE x3
Lymphopenia Thrombocytopenia Coombs positive anaemia
33
What can show on urinalysis in SLE
Proteinuria and casts - reflecting renal disease (more common in child-onset SLE than adult)
34
How does juvenile dermatomyositis present?
Insidiously with malaise, progressive muscle weakness and muscle pain/discomfort
35
What % of juvenile dermatomyositis have arthritis
20%
36
Dermatological features of dermatomyositis x4
Heliotrope rash (purplish discoloration and oedema of eyelids) Malar rash travelling down to nasolabial folds Gottrons papules over MCP, elbows and knees Cuticle hyperaemia
37
Serum signs of dermatomyositis
Elevated serum muscle enzymes, CPK aldolase, AST, ALT and LDH
38
Features of localised scleroderma syndromes
Localised disorder characterised by areas of oval or linear lesions that traverse over joints, face and trunk Can also have frank arthritis
39
2 acute inflammatory DDX of JIA
HSP | Kawasaki disease
40
Features of HSP x5
``` Purpuric rash over lower legs and buttocks Cramping abdominal pain Bloody stools Haematuria Arthritis of ankles and knees ```
41
Malignant DDX of JIA x4
Acute lymphoblastic leukaemia Neuroblastoma Lymphoma Primary bone malignancies (rare)
42
ALL presentation similar to JIA
Can present with bone pain in children and even frank arthritis Pain over affected bone
43
Idiopathic pain syndromes as DDX for JIA x2
Complex regional pain syndrome | Diffuse MSK pain syndromes
44
Why do you do investigations in JIA
JIA is a label for children who fulfil criteria based on clinical features alone - therefore investigations to exclude other DDX
45
Why do you do FBC in JIA suspicion?
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
Why do you do ESR in JIA suspicion?
ESR elevated in systemic arthritis and SLE | Normal in up to 1/2 patients with JIA and in most with dermatomyositis
47
Why do you do urinary catecholamines in JIA suspicion?
To exclude neuroblastoma
48
Why do you do Liver function tests in JIA suspicion?
To rule out dermatomyositis
49
Why do you do Antinuclear antibodies in JIA suspicion?
High ANAs seen in virtually all children with SLE and MCTD | Also positive ANA in oligoarthritis
50
Why do you do IgG in JIA suspicion?
IgG highly elevated in SLE
51
Why do you do Borrelia burgdorferi serology in JIA suspicion?
To rule out Lyme disease if there is a history of travel in endemic area
52
Why do you do synovial fluid analysis in JIA suspicion?
Mandatory if suspected septic arthritis
53
Why do you do radiology in JIA suspicion?
To rule out fractures, malignancy, look for joint effusions, bone dysplasia/neoplasia, avascular necrosis
54
Features of systemic arthritis
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
Presentation age of systemic arthritis
Usually begins in early childhood - although it can occur at any age through to adulthood
56
Laboratory studies in systemic arthritis
High WBC count Severe anaemia, thrombocytosis High ESR, CRP and ferritin
57
Duration of systemic features in systemic arthritis?
They usually resolve after a few months but they may last indefinitely
58
Prognosis of systemic arthritis
Very poor, erosions of joint and loss of motion | Also severe growth delay due to treatment with steroids
59
What can occur with systemic arthritis?
Macrophage activation syndrome - carries a 10-15% mortality rate - IV steroids and cyclosporine may reverse rapid deterioration and DIC
60
What does oligo- mean?
Sparse or few
61
What % of JIA does oligoarthritis account for?
50%
62
Features of oligoarthritis?
Knee most commonly affected joint Otherwise healthy 20% have asymptomatic anterior uveitis
63
Joints affected in oligoarthritis
Most commonly the knee then ankle and wrist. Hip rarely ever affected
64
Sex ratio of oligoarthritis
Female:Male 5:1
65
Features of eye problems in oligoarthritis
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
Laboratory investigations in oligoarthritis
Frequently positive ANAs but all other examinations are normal
67
Implications of oligoarthritis
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
What is extended oligoarthritis?
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
What do children with extended oligoarthritis often have
Anterior uveitis
70
What are the two types of polyarthritis?
Rheumatoid-negative and rheumatoid-positive
71
Features of rheumatoid negative polyarthritis
Usually affects preschool girls | Predominantly symmetrical arthritis of upper and lower limbs
72
What can happen with rheumatoid negative polyarthritis x2
Rare but important - growth restriction and chronic anterior uveitis
73
Prognosis of rheumatoid negative polyarthritis?
Illness lasts most of childhood and many go on to adulthood with the disease
74
Laboratory findings with rheumatoid negative polyarthritis
May have mild anaemia Usually positive ANAs ESR and CRP may be mildly elevated
75
What % of JIA is due to rheumatoid negative polyarthritis?
25-30%
76
What type of disease is rheumatoid positive polyarthritis
Similar in features and prognosis to adult RA - therefore can be called juvenile rheumatoid arthritis
77
Presentation age of rheumatoid positive polyarthritis
Usually in late childhood or adolescence
78
Gender ratio of rheumatoid positive polyarthritis
Primarily affects girls
79
Features of rheumatoid positive polyarthritis
Like rheumatoid arthritis can be rapidly progressive and destructive Rheumatoid nodules are common Failure to thrive more frequent than in rh- polyarthritis
80
Laboratory findings in rheumatoid positive polyarthritis
ANAs are usually positive
81
Features of psoriatic arthritis in children
Arthritis may pre-date any classical skin findings by many years Often asymmetrical arthritis, affects both small and large joints
82
What establishes the diagnosis of psoriatic arthritis
Family history of first degree relative with psoriasis
83
What can also affect children with psoriatic arthritis?
Asymptomatic uveitis - high risk of blindness if undetected
84
Features of enthesitis-related arthritis x3
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
Age and gender of children with enthesitis-related arthritis
Usually after 6 years old and boys>girls
86
What is enthesitis-related arthritis a precursor to?
Ankylosing spondylitis
87
What does treatment of arthritis in children usually start with? Details of this therapy
Usually starts with NSAIDs - used in higher doses relative to body weight than in adults because children have increased metabolism and renal excretion
88
Adverse effects of NSAIDs treatment in JIA
Abdominal pain, change in mood, rarely bronchospasm
89
Success of NSAIDs in JIA?
Majority of patients with early JIA do not respond to NSAIDs and need more aggressive treatment
90
What can be used for single joint involvement in JIA?
Intra-articular corticosteroids Usually down under GA in UK Often used in oligoarthritis
91
What is generally avoided treatment wise for JIA?
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
Eg. of two slow-acting anti-rheumatic drugs used in JIA
Methotrexate and sulfasalazine
93
What is methotrexate effective at treating in JIA?
Effective in approx 70% of children with polyarthritis | Much less effective in systemic arthritis
94
When is methotrexate considered in JIA?
If disease not controlled with NSAIDs or intra-articular steroids after 4-12week trial
95
How long after starting can efficacy of methotrexate be determined?
1-3 months
96
Most common side effects of methotrexate
Nausea and mouth ulcers (ondansetron and folate)
97
Other side effects of methotrexate
Abdominal pain, elevated LFTs | Rarely hair loss and bone-marrow suppression
98
What is a possible substitute for methotrexate in JIA?
Leflunomide - same efficacy and less toxicity
99
When is sulfasalazine effective?
In oligoarthritis and polyarthritis - especially effective in ERA Not much value in SA and increased risk of macrophage activated syndrome
100
What have revolutionised JIA treatment? 2 eg
Biologics - TNFa-antagonists | Etanercept and Infliximab
101
When can etanercept be used in JIA?
Children whose disease is not controlled by methotrexate or are intolerant of it Very effective treatment
102
Infliximab use in JIA?
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
Hydroxychloroquine use in JIA?
Use in children is not supported - studies not shown to be effective

Ortho and Rheum (13 decks)

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