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Year 3 MSK (JK) > Multisystem autoimmune diseases > Flashcards

Multisystem autoimmune diseases Flashcards

1
Q

List some connective tissue diseases

A 
SLE 
scleroderma 
sjogren's 
autoimmune myositis 
MCTD
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2
Q

List some types of system vasculitis

A

giant cell arteritis
granulomatosis polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis polyangiitis

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3
Q

Mimics of multisystem autoimmune disorders

A

drugs eg cocaine
infection eg HIV, TB
cardiac myxoma
scurvey

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4
Q

SLE epidemiology

A

disease of the young and females (9:1 with males)

15-50 years and afrocaribbean and Asian affected more than Caucasian

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5
Q

Talk through body systems and the symptoms of SLE

A

face - butterfly rash and photosensitivity
CNS - fits, ataxia and hemiplegia
alopecia
lungs - pleural effusion, pleurisy and fibrosis
heart - pericarditis, endocarditis and aortic valve lesion
general - fever and depression
skin - vasculitis and urticaria, raynauds
blood - anaemia, thrombocytopenia
myopathy, joint arthritis

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6
Q

How many of the classification criteria are required for an SLE diagnosis?

A

4

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7
Q

List the 11 classification criteria possibilities for SLE

A 
malar rash 
discoid rash 
photosensitivity 
oral ulcers 
arthritis 
renal - proteinuria or cellular casts 
neurological - seizures 
serositis 
haematological eg low WCC
immunological eg anti-dsDNA, sm la, Low complement
ANA
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8
Q

Epidemiology of scleroderma

A

female 3: male 1

30-50 years

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9
Q

Name the 3 types of scleroderma

A

morphea
limited
diffuse

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10
Q

morphea

A

skin affected - localised

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11
Q

limited scleroderma

A 
does not pass elbows 
Calcium
Raynauds
Eosophgeal dysmotility
Sausage fingers
Telengectasia
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12
Q

complications of limited scleroderma

A

pulmonary hypertension

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13
Q

Complications of diffuse scleroderma

A

pulmonary fibrosis
renal crisis - high bp
small bowel bacteria overgrowth

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14
Q

Sjogren’s epidemiology

A

40-50 years

female 9: male 1

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15
Q

Sjogren’s symptoms

A

CNS - fits, hemiplegia, cranial nerve lesions
GI - dysphagia and abnormal motility
resp - ILD
joints - arthralgia
kidney - renal tubular acidosis
skin - palpable purpura and reynauds
PNS - sensory neuropathy, mononeuritis simplex
DRY EYES AND MOUTH, parotid enlargement and 1/3 have systemic upset

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16
Q

5 complications of Sjogren’s

A 
lymphoma
renal tubular acidosis 
purpura 
neuropathy 
ILD
17
Q

Incidence of auto-immune myositis

A

Rare - 6/million

18
Q

Symptoms of myositis

A

muscle weakness - symmetrical, proximal, diffuse

19
Q

2 types of myositis

A

polymyositis

dermatomyositis

20
Q

2 skin changes with dermatomyositis

A

gottrons papules - 80%

heliotrope rash - 30-60%

21
Q

complications of myositis

A

cancer and ILD

22
Q

overlap syndromes (MCTD)

A

raynauds
soft tissue swelling
myositis
arthralgia

23
Q

Example of large vessel vasculitides

A

GCA

24
Q

example of small vessel vascultides

A

ANCA associated

25
Q

giant cell arteritis - 3 of the following…

A 
age of onset >50
new headache 
temporal artery tender/reduced pulsation 
ESR>50
abnormal temporal artery biopsy
26
Q

3 types of ANCA associated vasculitis

A
  1. granulomatosis with polyangiitis
  2. microscopic polyangiitis
  3. eosinophilic granulomatosis with polyangiitis
27
Q

GPA

A

necrotising granulomatous inflammation
URT and LRT
small and medium vessels
necrotising glomerulonephritis common

28
Q

MPA

A

pulmonary capillaritis
necrotising glomerulonephritis, arteritis and vasculitis
few or no immune deposits

29
Q

EGPA

A

eosinophilic rich and necrotising granulomatous inflammation - resp tract
necrotising vasculitis and ANCA
associated with asthma and eosinophilia

30
Q

5 year damage with AAV

A

nerve damage, hearing loss, malignancy, lung and renal damage

31
Q

condition and %ANA

A

MCTD and drug induced lupus - 100%
SLE - 99%
systemic sclerosis - 97%
sjogrens and myositis rarer

32
Q

Conditions where positive ANA is not helpful

A

normal, infection, RA and MS

33
Q

SLE ANA profiles

A

dsDNA, Ro, Sm

34
Q

scleroderma ANA profiles

A

SCI-70, centromere

35
Q

polymyositis ANA

A

jo-1

36
Q

sjogrens ANA

A

Ro, La

37
Q

6 classes of lupus nephritis and briefly describe

A 
1- minimal mesangial
2- mesangial proliferation
3- focal
4- diffuse
5- membranous 
6- advanced sclerosing
38
Q

organ threat and treatment used

A 
mild = hydroxychloroquine 
moderate = methotrexate,mycophenolate, azathioprine
severe = cyclophosphamide, Rituximab

Year 3 MSK (JK) (30 decks)

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