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Year 3 > Multisystem Autoimmune Disease > Flashcards

Multisystem Autoimmune Disease Flashcards

1
Q

name some autoimmune connective tissue diseases

A 
systemic lupus erythematosus
scleroderma
Sjogren's syndrome
auto-immune myositis
mixed connective tissue disease
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2
Q

name some autoimmune systemic vasculitis diseases

A

giant cell arteritis
granulomatosis polyangiitis (Wegener’s)
microscopic polyangiitis
eosinophilic granulomatosis polyangiitis (Churg-Strauss)

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3
Q

diagnosis of autoimmune diseases

A
  • Cardinal clinical features: history and exam
  • Immunology
  • Imaging
  • Tissue
  • Exclusion of differential diagnosis
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4
Q

mimics of autoimmune diseases

A 
• Drugs – cocaine, minocycline, PTU
• Infection – HIV, endocarditis,
hepatitis, TB
• Malignancy – lymphoma
• Cardiac myxoma
• Cholesterol emboli
• Scurvy
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5
Q

SLE: UK prevalence

A

28/100,000

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6
Q

SLE: UK incidence

A

4/100,000

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7
Q

SLE: M:F

A

1:9

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8
Q

SLE: ethnicity

A

afro-caribbean>asain>caucasian

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9
Q

SLE: age of onset

A

15-50

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10
Q

SLE: classification criteria

A

any 4:
o Malar rash
o Discoid rash – raised, scarring, permanent marks, alopecia
o Photosensitivity
o Oral ulcers
o Arthritis (at least 2 joints)
o Serositis (pleurisy or pericarditis)
o Renal – significant proteinuria or cellular casts in urine
o Neurological – unexplained seizures or psychosis
o Haematological – low WCC, platelets, lymphocytes, haemolytic anaemia
o Immunological – anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement
o ANA

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11
Q

scleroderma: UK prevalence

A

24/100,000

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12
Q

scleroderma: UK incidence

A

10/1,000,000

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13
Q

scleroderma: onset

A

30-50

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14
Q

scleroderma: M:F

A

1:3

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15
Q

scleroderma: complications

A

pulmonary hypertension
pulmonary fibrosis
renal crisis
smal bowel bacterial overgrowth

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16
Q

Sjogren’s syndrome: UK prevalence

A

1/100

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17
Q

Sjogren’s syndrome: UK incidence

A

4/100,000

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18
Q

Sjogren’s syndrome: onset

A

40-50

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19
Q

Sjogren’s syndrome: M”F

A

1:(

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20
Q

Sjogren’s syndrome: signs and symptoms

A 
dry eyes and mouth
parotid gland enlargement
fatigue
fever 
myalgia
arthalgia
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21
Q

Sjogren’s syndrome: complications

A 
lymphoma
neuropathy
purpura
interstitial lung disease
renal tubular acidosis
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22
Q

auto-immune myositis: prevalence

A

6/1,000,000

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23
Q

auto-immune myositis: symptoms

A 
muscle weakness - symmetrical, diffuse, proximal
polymyositis
dermatomyositis
Gottron's papules
heliotrope rash
24
Q

auto-immune myositis: complicatons

A

cancer

interstitial lung disease

25
Q

auto-immune myositis: oveerlap syndromes (mixed connective tissue disease)

A

soft tissue swellings
Raynaud’s
myositis
arthralgia

26
Q

name large vessel vasculitis

A

takayasu arteritis

giant cell arteritis

27
Q

name medium vessel vasculitis

A

polyarteritis nodosa

kawasaki disease

28
Q

name small vessel vasculitis: ANCA associated vasculitis

A

microscopic polyangiitis
granulomatosis with polyangitis
eosinophilic granulomatosis with polyangitis

29
Q

name small vessel vasculitis: immune complex SVV

A

anti-GBM disease
cryoglobulinemic vasculitis
IgA vasculitis (Henoch-Schönlein)
Hypocomplementemic urticarial vasculitis (Anti-C1q vasculitis)

30
Q

giant cell arteritis: classification criteria

A 
3 of:
age of onset >=50
new headache
temporal artery tenderness/reduced pulsation
EST >= 50
abnormal temporal biopsy
31
Q

ANCA associated vasculitis: overall incidence

A

15/million

32
Q

ANCA associated vasculitis: overall prevalence

A

150/million

33
Q

Granulomatosis with Polyangiitis (Wegener’s): features

A
  • Necrotising granulomatous inflammation
  • Usually involving the upper and lower respiratory tract
  • Affecting predominantly small to medium vessels
  • Necrotising glomerulonephritis is common
34
Q

Microscopic Polyangiitis: features

A
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
  • Necrotising arteritis involving small and medium arteries may be present
  • Necrotising glomerulonephritis is very common
  • Pulmonary capillaritis often occurs
  • Granulomatous inflammation is absent
35
Q

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): features

A

• Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory
tract
• Necrotising vasculitis predominantly affecting small to medium vessels
• Associated with asthma and eosinophilia
• ANCA is more frequent when glomerulonephritis is present

36
Q

ANA are found in what % of SLE?

A

99

37
Q

ANA are found in what % of systemic sclerosis?

A

97

38
Q

ANA are found in what % of poly/dermatomyositis?

A

40-80

39
Q

ANA are found in what % of Sjogren’s syndrome?

A

48-96

40
Q

ANA are found in what % of MCTD

A

100

41
Q

ANA are found in what % of drug induced lupus

A

100

42
Q

what conditions make ANA +ve unhelpful?

A

normal 30%
RA 30-40%
MS 25%
infection

43
Q

disease specific ANA profile: SLE

A

dsDNA
Ro
Sm

44
Q

disease specific ANA profile: scleroderma

A

Scl-70

centromere

45
Q

disease specific ANA profile: polymyositis

A

Jo-1

46
Q

disease specific ANA profile: Sjogren’s syndrome

A

Ro

La

47
Q

lupus nephritis class I

A

minimal mesangial

48
Q

lupus nephritis class II

A

mesangial proliferative

49
Q

lupus nephritis class III

A

focal

50
Q

lupus nephritis class IV

A

diffuse

51
Q

lupus nephritis class V

A

membranous

52
Q

lupus nephritis class VI

A

advanced sclerosing

53
Q

treatment of mild autoimmune disease

A

hydroxychloroquine

54
Q

treatment of moderate autoimmune disease

A

azathioprine
methotrexate
mycophenolate

55
Q

treatment of severeautoimmune disease

A

cyclophosphamide

rituximab

Year 3 (164 decks)

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