Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

RACP Paediatric Exam - Medstudy edition > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What cytokines are believed to mediate JIA

A

TNF alpha, IL1, IL6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Describe fever in sJIA

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

2 poor prognostic indicators fir sJIA

A

Active disease >1year after diagnosis

Diagnosis before 4yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A

TMJ involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

In which eye chamber is uveitis due to JIA found?

A

Anterior uveitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

ANA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A

Poor prognostic finding

antiCCP - erosive arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

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

A

Years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Arthritis of which joints is unique to psJIA?

A

DIP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Which biologics can be used for JIA?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What percentage of patients with IBD have arthritis

A

25%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A 
GI infection
GU infection (chlamydia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Triad of reactive arthritis

A

Urethritis, conjunctivitis, arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

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

A

Yes, urinalysis may show sterile pyuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Most common vasculitis of childhood?

A

Henoch Schonlein Purpura

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What antibody mediates HSP?

A

IgA

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

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

A

Viral infection - URTI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

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

A

Renal involvement - hence recurrent dipstick / BP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Are most cases of HSP self limited?

A

Yes - last 4 weeks in 65% of children

Recurs in 40% of patients from 6wks to 2 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is the nasal deformity seen in granulomatosis with polyangiitis?

A

Saddle nose

Subglottic stenosis also seen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What laboratory findings seen in granulomatosis with polyangiitis?

A

cANCA for PR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the main cause of death in Kawasaki disease?

A

Myocardial infarction - 1st year from onset of disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Diagnostic criteria for Kawasaki?

A 
Fever >5 days
Large unilateral LN 1.5cm
Mucocutaenous findings - strawberry tongue
Desquamation
Rash 
Bilateral non exudative conjunctivitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Gallbladder abnormality in Kawasaki?

A

Hydrops of gallbladder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Cardiac manifestations of Kawasaki?

A

Pericardial effusions, myocarditis

Coronary artery abnormalities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Treatment of Kawasaki

A

IVIG

Aspirin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What are the potential therapies available for Kawasaki refractive to IVIG?

A

Infliximab

Cyclosporine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is polyarteritis nodosa?

A

Medium sized artery inflammation - focal segmental necrotizing vasculitis

  • constitutional sy,ptoms, MSK and renal
  • orchidis (concurrent Hep B)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What organ system is spared in PAN

A

Lung is spared

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What is takayasu arteritis?

A

Rare, large vessel granulomatous vasculitis.

Arteritis of aorta and major vessels, resulting in weak or absent pulses in upper extremities

37
Q

What is the classic triad of Behcet disease?

A 
Oral ulcers (painful, recurrent)
Genital ulcers (painful, recurrent)
Inflammatory eye disease
38
Q

Skin lesions commonly seen in Behcet disease?

A

Erythema nodosum

Necrotic folliculitis

39
Q

Which ethnic groups have higher rates of SLE?

A

Females, African, Asian and Hispanic

40
Q

What antibodies increase the risk for neonatal lupus?

A

Anti Ro and Anti La

41
Q

What cardiac complication is seen in neonatal lupus?

A

Congenital complete heart block

42
Q

Is a positive ANA common in paediatric SLE?

A

Positive ANA occurs in almost all paediatric patients

43
Q

With what are antiphospholipid antibodies associated?

A

Affect pathways of coagulation and increase the risk of miscarriage, thrombocytopenia, livedo reticularis and blood clots

44
Q

What antibody is a marker of disease activity in SLE?

A

anti ds-DNA (C3 and C4 decline with active disease)

45
Q

What antibody is most specific for SLE?

A

Anti smith

46
Q

What are the 2 most common causes of chorea?

A

SLE is the most common cause in the US

47
Q

Which hair finding is common in SLE?

A

Alopecia

48
Q

Where are the painless ulcerations typically located in patients with lupus?

A

Oral lesions - hard palate or nares

49
Q

Which joint abnormality, common in SLE, is due to the disease itself, antiphospholipid antibody and/or high dose steroids?

A

Avascular necrosis

  • presents as nighttime pain, non inflammatory effusion, can be asymptomatic
  • Hips, knees and shoulders
50
Q

Whic type of endocarditis is associated with SLE and antiphospholipid antibodies?

A

Libman-Sacks endocarditis (non bacterial endocarditis with verrucous vegetations)

51
Q

Which drug class reduces lupus mortality and improves prognosis?

A

Antimalarial drugs - hydroxychloroquine

52
Q

What are the clinical side effects of long term corticosteroid use in children with SLE?

A 
AVN
Osteoperosis with fracture / vertebral collapse
Growth failure
Gluacoma and cataracts
Diabetes mellitus
HTN
Accelerated atherosclerosis 
Infection
53
Q

What is the antibody affiliated with mixed connective tissue disease?

A

anti-U1 RNP

- presents like scleroderma

54
Q

What is the main cause of mortality in MCTD?

A

Chronic interstitial lung disease or pulmonary HTN

55
Q

Name the diagnostic criteria for Sjogren syndrome?

A

Inflamed and dry eyes
Dryness of mouth
Lymphocytic infiltrate on minor salivary gland biopsy
Lab evidence of RF+, ANA+ or Ro + or LA +

56
Q

How do most paediatric patients with Sjogren syndrome present?

A

Recurrent parotitis and keratoconjunctivitis sicca

57
Q

Which malignancies are patients with Sjogren syndrome at risk for?

A

Lymphoma, especially MALT and non-Hodgkin lymphoma

58
Q

Describe the classic rashes of juvenile dermatomyositis?

A 
Heliotrope rash (faint purple-to-red discolouration of the eyelids with or without oedema)
Gottron papules (red plaques over the extensor surfaces)
59
Q

The muscle weakness of JDM mainly affects which muscle group?

A

Proximal and symmetric

60
Q

What is the primary therapy for JDM?

A

Prednisolone

61
Q

What are the 2 main subtypes of systemic scleroderma?

A

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
Q

Which part of the GI tract is most commonly affected in systemic scleroderma?

A

Distal oesophagus - distal smooth muscle = dysphagia

63
Q

Which class of antihypertensive do you use to reduce the incidence of scleroderma renal crisis?

A

ACE inhibitors

64
Q

Which lab findings useful in the diagnosis of systemic scleroderma?

A

Anti-centromere
SCL70
Speckled ANA

65
Q

Anticentromere antibodies are seen in what?

A

Limited systemic sclerosis (CREST)

Primary biliary cirrhosis

66
Q

Why are corticosteroids not used in systemic sclerosis?

A

Increased risk of renal crisis

67
Q

What is the most common form of localised scleroderma in children? How does it affect soft tissue and bone?

A

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
Q

What are common antiinflammatory treatments used for linear scleroderma?

A

Systemic steroids

Methotrexate

69
Q

List the most common systems affected in sarcoidosis

A

Lungs
Lymph nodes
Eyes
Skin

70
Q

What is the triad of clinical manifestations typically seen in Blau syndrome?

A

Uveitis, rash and boddy arthritis

Blau = familial form of sarcoidosis

71
Q

Describe the skin findings in sarcoidosis.

A

Red brown, maculopapular and <1cm in diameter

Face, neck, back and extremities

72
Q

Is morning pain a common finding in growing pains?

A

No, usually bilateral occurring late in the day or evening, wakes from sleep.

73
Q

Are unilateral findings common in growing pains?

A

No, unilateral pain requires further evaluation

74
Q

Describe the tasks that can be attempted to see if someone has hypermobility syndrome

A

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
Q

When you find hypermobility syndrome, which traits do you look for to determine if a hereditary syndrome might be present?

A

High arched palate, ocular/cardiac lesions, skin hyperelasticity, arachnodactyly, velvety skin texture

76
Q

What is the recommended exercise activity for hypermobility syndrome

A

Swimming and other low / no impact sports

77
Q

What are the clinical findings in patients with amplified MSK pain syndromes

A

One affected limb, constant pain

No articular swelling, loss of motion or muscle weakness

78
Q

What are some diseases that cause amyloid deposition?

A 
Infectious (TB, leprosy, chromic OM)
Familial mediterranian fever
IBD
Behcet disease
SLE
79
Q

What is the most serious manifestation of amyloid deposition?

A

Renal disease

80
Q

What is PFAPA syndrome?

A

Periodic fever, aphthous ulcers, pharyngitis, cervical adenitis
Benign - 6mo - 7yo, no genetic cause

81
Q

Which recurrent fever syndrome does not have a known genetic abnormality?

A

PFAPA

82
Q

Where is the gene responsible for familial Mediterranean fever? What is the product of the gene?

A

MEFV - chromosome 16
Product is pyrin - responsible for regulation of PMN inflammatory response and interacts with TNF, IL-1 and other cytokines

83
Q

How do you treat FMF?

A

Daily colchicine - treats acute attacks, prevents future attacks and prevents development of amyloidosis

84
Q

What is TRAPS?

A

TNF receptor-1-associated periodic syndrome

- autosomal dominant - incomplete penetrance - genetic defect in the gene that encodes the receptor for TNF

85
Q

Define the genetic defect in hyper IgD syndrome

A

MVK gene that encodes mevalonate kinase, which results in excess production of IL-1
Elevated IgD

86
Q

What is CAPS?

A

Crypoyrin associated periodic syndromes

- autoinflammatory diseases, AD inheritance

87
Q

Name 3 periodic fever syndromes due to crypoyrin abnormalities

A

Familial cold autoinflammatory syndrome
Muckle-Wells syndrome
Neonatal onset multisystem inflammatory disease

88
Q

What is the neutrophilic pattern in cyclic neutropenia?

A

ANC <0.5 every 21 days like clockwork, each episode lasting 3-10 days

RACP Paediatric Exam - Medstudy edition (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions