Multisystem autoimmune diseases

Question 1

List some connective tissue diseases

Answer 1

SLE 
scleroderma 
sjogren's 
autoimmune myositis 
MCTD

Question 2

List some types of system vasculitis

Answer 2

giant cell arteritis
granulomatosis polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis polyangiitis

Question 3

Mimics of multisystem autoimmune disorders

Answer 3

drugs eg cocaine
infection eg HIV, TB
cardiac myxoma
scurvey

Question 4

SLE epidemiology

Answer 4

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

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

Question 5

Talk through body systems and the symptoms of SLE

Answer 5

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

Question 6

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

Answer 6

4

Question 7

List the 11 classification criteria possibilities for SLE

Answer 7

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

Question 8

Epidemiology of scleroderma

Answer 8

female 3: male 1

30-50 years

Question 9

Name the 3 types of scleroderma

Answer 9

morphea
limited
diffuse

Question 10

morphea

Answer 10

skin affected - localised

Question 11

limited scleroderma

Answer 11

does not pass elbows 
Calcium
Raynauds
Eosophgeal dysmotility
Sausage fingers
Telengectasia

Question 12

complications of limited scleroderma

Answer 12

pulmonary hypertension

Question 13

Complications of diffuse scleroderma

Answer 13

pulmonary fibrosis
renal crisis - high bp
small bowel bacteria overgrowth

Question 14

Sjogren’s epidemiology

Answer 14

40-50 years

female 9: male 1

Question 15

Sjogren’s symptoms

Answer 15

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

Question 16

5 complications of Sjogren’s

Answer 16

lymphoma
renal tubular acidosis 
purpura 
neuropathy 
ILD

Question 17

Incidence of auto-immune myositis

Answer 17

Rare - 6/million

Question 18

Symptoms of myositis

Answer 18

muscle weakness - symmetrical, proximal, diffuse

Question 19

2 types of myositis

Answer 19

polymyositis

dermatomyositis

Question 20

2 skin changes with dermatomyositis

Answer 20

gottrons papules - 80%

heliotrope rash - 30-60%

Question 21

complications of myositis

Answer 21

cancer and ILD

Question 22

overlap syndromes (MCTD)

Answer 22

raynauds
soft tissue swelling
myositis
arthralgia

Question 23

Example of large vessel vasculitides

Answer 23

GCA

Question 24

example of small vessel vascultides

Answer 24

ANCA associated

Question 25

giant cell arteritis - 3 of the following...

Answer 25

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

Question 26

3 types of ANCA associated vasculitis

Answer 26

1. granulomatosis with polyangiitis 2. microscopic polyangiitis 3. eosinophilic granulomatosis with polyangiitis

Question 27

GPA

Answer 27

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

Question 28

MPA

Answer 28

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

Question 29

EGPA

Answer 29

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

Question 30

5 year damage with AAV

Answer 30

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

Question 31

condition and %ANA

Answer 31

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

Question 32

Conditions where positive ANA is not helpful

Answer 32

normal, infection, RA and MS

Question 33

SLE ANA profiles

Answer 33

dsDNA, Ro, Sm

Question 34

scleroderma ANA profiles

Answer 34

SCI-70, centromere

Question 35

polymyositis ANA

Answer 35

jo-1

Question 36

sjogrens ANA

Answer 36

Ro, La

Question 37

6 classes of lupus nephritis and briefly describe

Answer 37

``` 1- minimal mesangial 2- mesangial proliferation 3- focal 4- diffuse 5- membranous 6- advanced sclerosing ```

Question 38

organ threat and treatment used

Answer 38

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