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RACP Paediatric Exam - Medstudy edition > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What cytokines are believed to mediate JIA

A

TNF alpha, IL1, IL6

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2
Q

Describe fever in sJIA

A

1-2 fever spikes on a daily basis, quotindian fever

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3
Q

2 poor prognostic indicators fir sJIA

A

Active disease >1year after diagnosis

Diagnosis before 4yo

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4
Q

What is macrophage activation syndrome?

A 
Acquired haemophagocytic syndrome
Persistant fever, hepatosplenomegaly
High ferritin
Cytopenias
Liver dysfunction
Neurologic dysfunction

Tx: steroids, IL-1 and Il6 inhibitors

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5
Q

Which joint is frequently involved in oJIA but often silent, requiring diagnosis by MRI

A

TMJ involvement

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6
Q

In which eye chamber is uveitis due to JIA found?

A

Anterior uveitis

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7
Q

The presents of what antibody in oJIA increases the risk of anterior uveitis?

A

ANA

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8
Q

In poJIA, what does the presence of RF and anti-CCP antibodies indicate?

A

Poor prognostic finding

antiCCP - erosive arthritis

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9
Q

What criteria is needed to make diagnosis of psoriatic JIA

A 
Arthritis and psoriasis 
OR
Arthritis and at least 2 of:
- dactylitis
- nail findings
- FHx of psoriasis in at least one 1st degree relative
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10
Q

In psJIA, how long can the arthritis precede the psoriasis?

A

Years

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11
Q

Arthritis of which joints is unique to psJIA?

A

DIP

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12
Q

Which biologics can be used for JIA?

A

IL-1 blockers - anakira
IL-6 - tocilizumab
TNF alpha inhibitors - infliximab

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13
Q

What percentage of patients with IBD have arthritis

A

25%

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14
Q

In arthritis with IBD, how do the peripheral vs axial arthritis forms differ?

A

Peripheral

  • M=F
  • Not associated with HLA-B27
  • Arthritis flares WITH gut flares

Axial
- M»F
- associated with HLA-B27
Not dependent on gut flares

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15
Q

What are the systemic symptoms of arthritis with IBD

A

Fatigue, Iron def anaemia, low albumin, elevated inflammatory markers (persistent), weight loss, growth delay, fever, oral ulcers, abdo pain, diarrhoea, erythema nodosum, pyoderma gangrenosum, clubbing

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16
Q

What are the differing characteristics between bacterial and viral aetiologies of infectious arthritis?

A

Bacteria- single, large joints

Viral - rash and symmetric involvement of smaller joints

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17
Q

Infections of which 2 systems typically occurs prior to reactive arthritis?

A 
GI infection
GU infection (chlamydia)
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18
Q

Triad of reactive arthritis

A

Urethritis, conjunctivitis, arthritis

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19
Q

Can urethritis occur in reactive arthritis even if the organism is of GI organism

A

Yes, urinalysis may show sterile pyuria

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20
Q

Most common vasculitis of childhood?

A

Henoch Schonlein Purpura

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21
Q

What antibody mediates HSP?

A

IgA

Deposition in vessel walls (along with small amounts of IgG and C3)

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22
Q

What is a predisposing factor in 50% of HSP cases?

A

Viral infection - URTI

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23
Q

What do you do in a child with HSP who has severe, persistent abdominal pain? What do you suspect as an aetiology?

A

Ultrasound

Intussusception - 2-14% of patients

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24
Q

What system are you concerned about up to 6 months in HSP?

A

Renal involvement - hence recurrent dipstick / BP

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25
Are most cases of HSP self limited?
Yes - last 4 weeks in 65% of children | Recurs in 40% of patients from 6wks to 2 years
26
What is the nasal deformity seen in granulomatosis with polyangiitis?
Saddle nose | Subglottic stenosis also seen
27
What laboratory findings seen in granulomatosis with polyangiitis?
cANCA for PR3
28
What is the main cause of death in Kawasaki disease?
Myocardial infarction - 1st year from onset of disease
29
Diagnostic criteria for Kawasaki?
``` Fever >5 days Large unilateral LN 1.5cm Mucocutaenous findings - strawberry tongue Desquamation Rash Bilateral non exudative conjunctivitis ```
30
Gallbladder abnormality in Kawasaki?
Hydrops of gallbladder
31
Cardiac manifestations of Kawasaki?
Pericardial effusions, myocarditis | Coronary artery abnormalities
32
Treatment of Kawasaki
IVIG | Aspirin
33
What are the potential therapies available for Kawasaki refractive to IVIG?
Infliximab | Cyclosporine
34
What is polyarteritis nodosa?
Medium sized artery inflammation - focal segmental necrotizing vasculitis - constitutional sy,ptoms, MSK and renal - orchidis (concurrent Hep B)
35
What organ system is spared in PAN
Lung is spared
36
What is takayasu arteritis?
Rare, large vessel granulomatous vasculitis. | Arteritis of aorta and major vessels, resulting in weak or absent pulses in upper extremities
37
What is the classic triad of Behcet disease?
``` Oral ulcers (painful, recurrent) Genital ulcers (painful, recurrent) Inflammatory eye disease ```
38
Skin lesions commonly seen in Behcet disease?
Erythema nodosum | Necrotic folliculitis
39
Which ethnic groups have higher rates of SLE?
Females, African, Asian and Hispanic
40
What antibodies increase the risk for neonatal lupus?
Anti Ro and Anti La
41
What cardiac complication is seen in neonatal lupus?
Congenital complete heart block
42
Is a positive ANA common in paediatric SLE?
Positive ANA occurs in almost all paediatric patients
43
With what are antiphospholipid antibodies associated?
Affect pathways of coagulation and increase the risk of miscarriage, thrombocytopenia, livedo reticularis and blood clots
44
What antibody is a marker of disease activity in SLE?
anti ds-DNA (C3 and C4 decline with active disease)
45
What antibody is most specific for SLE?
Anti smith
46
What are the 2 most common causes of chorea?
SLE is the most common cause in the US
47
Which hair finding is common in SLE?
Alopecia
48
Where are the painless ulcerations typically located in patients with lupus?
Oral lesions - hard palate or nares
49
Which joint abnormality, common in SLE, is due to the disease itself, antiphospholipid antibody and/or high dose steroids?
Avascular necrosis - presents as nighttime pain, non inflammatory effusion, can be asymptomatic - Hips, knees and shoulders
50
Whic type of endocarditis is associated with SLE and antiphospholipid antibodies?
Libman-Sacks endocarditis (non bacterial endocarditis with verrucous vegetations)
51
Which drug class reduces lupus mortality and improves prognosis?
Antimalarial drugs - hydroxychloroquine
52
What are the clinical side effects of long term corticosteroid use in children with SLE?
``` AVN Osteoperosis with fracture / vertebral collapse Growth failure Gluacoma and cataracts Diabetes mellitus HTN Accelerated atherosclerosis Infection ```
53
What is the antibody affiliated with mixed connective tissue disease?
anti-U1 RNP | - presents like scleroderma
54
What is the main cause of mortality in MCTD?
Chronic interstitial lung disease or pulmonary HTN
55
Name the diagnostic criteria for Sjogren syndrome?
Inflamed and dry eyes Dryness of mouth Lymphocytic infiltrate on minor salivary gland biopsy Lab evidence of RF+, ANA+ or Ro + or LA +
56
How do most paediatric patients with Sjogren syndrome present?
Recurrent parotitis and keratoconjunctivitis sicca
57
Which malignancies are patients with Sjogren syndrome at risk for?
Lymphoma, especially MALT and non-Hodgkin lymphoma
58
Describe the classic rashes of juvenile dermatomyositis?
``` Heliotrope rash (faint purple-to-red discolouration of the eyelids with or without oedema) Gottron papules (red plaques over the extensor surfaces) ```
59
The muscle weakness of JDM mainly affects which muscle group?
Proximal and symmetric
60
What is the primary therapy for JDM?
Prednisolone
61
What are the 2 main subtypes of systemic scleroderma?
Diffuse cutaneous scleroderma - prox and distal skin involvement with internal organ dysfunction of the GI tract, lung, heart and kidney Limited cutaneous slceroderma (CREST) - calcinosis, Raynaud, eosophageal dysmotility, sclerodactyly, telangiectasias
62
Which part of the GI tract is most commonly affected in systemic scleroderma?
Distal oesophagus - distal smooth muscle = dysphagia
63
Which class of antihypertensive do you use to reduce the incidence of scleroderma renal crisis?
ACE inhibitors
64
Which lab findings useful in the diagnosis of systemic scleroderma?
Anti-centromere SCL70 Speckled ANA
65
Anticentromere antibodies are seen in what?
Limited systemic sclerosis (CREST) | Primary biliary cirrhosis
66
Why are corticosteroids not used in systemic sclerosis?
Increased risk of renal crisis
67
What is the most common form of localised scleroderma in children? How does it affect soft tissue and bone?
Linear scleroderma - linear streaks that are dermatomal - if crosses joint causes contractures - become more indurated and extend deeper into muscle and bone Streaks on face are known as en coupe de sabre
68
What are common antiinflammatory treatments used for linear scleroderma?
Systemic steroids | Methotrexate
69
List the most common systems affected in sarcoidosis
Lungs Lymph nodes Eyes Skin
70
What is the triad of clinical manifestations typically seen in Blau syndrome?
Uveitis, rash and boddy arthritis | Blau = familial form of sarcoidosis
71
Describe the skin findings in sarcoidosis.
Red brown, maculopapular and <1cm in diameter | Face, neck, back and extremities
72
Is morning pain a common finding in growing pains?
No, usually bilateral occurring late in the day or evening, wakes from sleep.
73
Are unilateral findings common in growing pains?
No, unilateral pain requires further evaluation
74
Describe the tasks that can be attempted to see if someone has hypermobility syndrome
5 tasks: 1. Extend wrist and MCP joints so that the fingers are parallel to the dorsum of the forearm 2. Passively oppose the thumb to the flexor aspect of the forearm 3. Hyperextend the elbows 10 degrees or more 4. Hyperextend the knees 10 degrees or more 5. Flex the trunk with the knees filly extended so the palms rest on the floor
75
When you find hypermobility syndrome, which traits do you look for to determine if a hereditary syndrome might be present?
High arched palate, ocular/cardiac lesions, skin hyperelasticity, arachnodactyly, velvety skin texture
76
What is the recommended exercise activity for hypermobility syndrome
Swimming and other low / no impact sports
77
What are the clinical findings in patients with amplified MSK pain syndromes
One affected limb, constant pain | No articular swelling, loss of motion or muscle weakness
78
What are some diseases that cause amyloid deposition?
``` Infectious (TB, leprosy, chromic OM) Familial mediterranian fever IBD Behcet disease SLE ```
79
What is the most serious manifestation of amyloid deposition?
Renal disease
80
What is PFAPA syndrome?
Periodic fever, aphthous ulcers, pharyngitis, cervical adenitis Benign - 6mo - 7yo, no genetic cause
81
Which recurrent fever syndrome does not have a known genetic abnormality?
PFAPA
82
Where is the gene responsible for familial Mediterranean fever? What is the product of the gene?
MEFV - chromosome 16 Product is pyrin - responsible for regulation of PMN inflammatory response and interacts with TNF, IL-1 and other cytokines
83
How do you treat FMF?
Daily colchicine - treats acute attacks, prevents future attacks and prevents development of amyloidosis
84
What is TRAPS?
TNF receptor-1-associated periodic syndrome | - autosomal dominant - incomplete penetrance - genetic defect in the gene that encodes the receptor for TNF
85
Define the genetic defect in hyper IgD syndrome
MVK gene that encodes mevalonate kinase, which results in excess production of IL-1 Elevated IgD
86
What is CAPS?
Crypoyrin associated periodic syndromes | - autoinflammatory diseases, AD inheritance
87
Name 3 periodic fever syndromes due to crypoyrin abnormalities
Familial cold autoinflammatory syndrome Muckle-Wells syndrome Neonatal onset multisystem inflammatory disease
88
What is the neutrophilic pattern in cyclic neutropenia?
ANC <0.5 every 21 days like clockwork, each episode lasting 3-10 days

RACP Paediatric Exam - Medstudy edition (9 decks)

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