Multisystem Autoimmune Disease

Question 1

name some autoimmune connective tissue diseases

Answer 1

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

Question 2

name some autoimmune systemic vasculitis diseases

Answer 2

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

Question 3

diagnosis of autoimmune diseases

Answer 3

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

Question 4

mimics of autoimmune diseases

Answer 4

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

Question 5

SLE: UK prevalence

Answer 5

28/100,000

Question 6

SLE: UK incidence

Answer 6

4/100,000

Question 7

SLE: M:F

Answer 7

1:9

Question 8

SLE: ethnicity

Answer 8

afro-caribbean>asain>caucasian

Question 9

SLE: age of onset

Answer 9

15-50

Question 10

SLE: classification criteria

Answer 10

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

Question 11

scleroderma: UK prevalence

Answer 11

24/100,000

Question 12

scleroderma: UK incidence

Answer 12

10/1,000,000

Question 13

scleroderma: onset

Answer 13

30-50

Question 14

scleroderma: M:F

Answer 14

1:3

Question 15

scleroderma: complications

Answer 15

pulmonary hypertension
pulmonary fibrosis
renal crisis
smal bowel bacterial overgrowth

Question 16

Sjogren’s syndrome: UK prevalence

Answer 16

1/100

Question 17

Sjogren’s syndrome: UK incidence

Answer 17

4/100,000

Question 18

Sjogren’s syndrome: onset

Answer 18

40-50

Question 19

Sjogren’s syndrome: M”F

Answer 19

1:(

Question 20

Sjogren’s syndrome: signs and symptoms

Answer 20

dry eyes and mouth
parotid gland enlargement
fatigue
fever 
myalgia
arthalgia

Question 21

Sjogren’s syndrome: complications

Answer 21

lymphoma
neuropathy
purpura
interstitial lung disease
renal tubular acidosis

Question 22

auto-immune myositis: prevalence

Answer 22

6/1,000,000

Question 23

auto-immune myositis: symptoms

Answer 23

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

Question 24

auto-immune myositis: complicatons

Answer 24

cancer

interstitial lung disease

Question 25

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

Answer 25

soft tissue swellings Raynaud's myositis arthralgia

Question 26

name large vessel vasculitis

Answer 26

takayasu arteritis | giant cell arteritis

Question 27

name medium vessel vasculitis

Answer 27

polyarteritis nodosa | kawasaki disease

Question 28

name small vessel vasculitis: ANCA associated vasculitis

Answer 28

microscopic polyangiitis granulomatosis with polyangitis eosinophilic granulomatosis with polyangitis

Question 29

name small vessel vasculitis: immune complex SVV

Answer 29

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

Question 30

giant cell arteritis: classification criteria

Answer 30

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

Question 31

ANCA associated vasculitis: overall incidence

Answer 31

15/million

Question 32

ANCA associated vasculitis: overall prevalence

Answer 32

150/million

Question 33

Granulomatosis with Polyangiitis (Wegener’s): features

Answer 33

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

Question 34

Microscopic Polyangiitis: features

Answer 34

* 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

Question 35

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): features

Answer 35

• 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

Question 36

ANA are found in what % of SLE?

Answer 36

99

Question 37

ANA are found in what % of systemic sclerosis?

Answer 37

97

Question 38

ANA are found in what % of poly/dermatomyositis?

Answer 38

40-80

Question 39

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

Answer 39

48-96

Question 40

ANA are found in what % of MCTD

Answer 40

100

Question 41

ANA are found in what % of drug induced lupus

Answer 41

100

Question 42

what conditions make ANA +ve unhelpful?

Answer 42

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

Question 43

disease specific ANA profile: SLE

Answer 43

dsDNA Ro Sm

Question 44

disease specific ANA profile: scleroderma

Answer 44

Scl-70 | centromere

Question 45

disease specific ANA profile: polymyositis

Answer 45

Jo-1

Question 46

disease specific ANA profile: Sjogren's syndrome

Answer 46

Ro | La

Question 47

lupus nephritis class I

Answer 47

minimal mesangial

Question 48

lupus nephritis class II

Answer 48

mesangial proliferative

Question 49

lupus nephritis class III

Answer 49

focal

Question 50

lupus nephritis class IV

Answer 50

diffuse

Question 51

lupus nephritis class V

Answer 51

membranous

Question 52

lupus nephritis class VI

Answer 52

advanced sclerosing

Question 53

treatment of mild autoimmune disease

Answer 53

hydroxychloroquine

Question 54

treatment of moderate autoimmune disease

Answer 54

azathioprine methotrexate mycophenolate

Question 55

treatment of severeautoimmune disease

Answer 55

cyclophosphamide | rituximab