C - Immune related multisystem disorders (E- Amir Sam)

Question 1

Sx of SLE

Answer 1

skin - malar rash / discoid
oral ulcers
joints
neuroloigcal - psychosis / depression
serositis
renal - glomerularnephritis
haematological - pancytopaenia
immunological

Question 2

AB for SLE

Answer 2

anti dsDNA
anti smith
anti histone

Question 3

which SLE pts gets anti histone ABs

Answer 3

drug induced SLE

Question 4

what % of SLE pts have anti smith ABs

Answer 4

30% (but its very specific for SLE)

Question 5

what protazoa is used to help diagnosis of SLE and how

Answer 5

crithidia luciliae
- has double stranded DNA so the SLE pt’s ABs bind to it

Question 6

why do SLE pts get a malar rash

Answer 6

lymphocytic infiltration of basal epidermis
immune complex deposition at epidermis-dermis junction

Question 7

kidney histology in SLE

Answer 7

Y loop capillaries - thickening of glomerular capillary walls
immune complex deposition in BM of capillary wall

Question 8

what type of endocarditis can you get in SLE

Answer 8

Libman - Sacks (non infective endocarditis caused by immune complex deposition)

Question 9

what is scleroderma (systemic sclerosis)

Answer 9

fibrosis and excess collagen deposition in skin

Question 10

2 forms of scleroderma

Answer 10

diffuse
limited

Question 11

ABs in diffuse scleroderma

Answer 11

DNA topoisomerase (Scl70)

Question 12

ABs in limited scleroderma

Answer 12

anti centromere

Question 13

Sx of limited scleroderma

Answer 13

CREST
calcinosis
raynauds
eosophageal dysmotility
sclerodactyly
telangiectasia

Question 14

difference between diffuse and limited scleroderma clinically

Answer 14

involvement of trunk
- tight skin over trunk = diffuse
- no tight skin over trunk = limited

Question 15

pattern of immunoflurescence in scleroderma

Answer 15

nucleolar

Question 16

histology of artery in scleroderma

Answer 16

onion skinning - intimal proliferation (narrowed lumen)

Question 17

mixed connective tissue disease has features of which other disorders

Answer 17

SLE
scleroderma
polymyositis
dermatomyositis

Question 18

immunoflurescence pattern of mixed connective tissue disease

Answer 18

speckled pattern

Question 19

finger sign of dermatomyositis

Answer 19

Gottron’s papules
- erythema over distal IPJ

Question 20

Sx of sarcoidosis

Answer 20

joints - arthralgia
skin - nodules and papules, Lupus pernio, erythema nodosum
lung - fibrosis, BHL
lymphadenopathy
heart - pericarditis, valvular lesions
eyes - uveitis, conjunctivitis
neuro - meningitis, CN
liver - hepatitis, cirrhosis
bilateral parotid enlargement

Question 21

pathological hallmark of sarcoidosis

Answer 21

non caseating granulomas

Question 22

what is lupus pernio

Answer 22

erythematous lesion involving nose

Question 23

causes of erythema nodosum

Answer 23

infection
inflammation - IBD, Behcets, sarcoidosis

Question 24

what is a granuloma

Answer 24

ball of lymphocytes

Question 25

what type of cell is seen in granuloma that is a fusion of mutlinucleated lymphocytes

Answer 25

giant cells of langhan

Question 26

what does non caseating mean

Answer 26

no necrosis

Question 27

3 metabolic imbalances in sarcoidosis

Answer 27

high Ca high ACE hypergammaglobulinaemia

Question 28

why do you get high calcium in sarcoid

Answer 28

granulomas in lung hydroxylate (activate) Vit D

Question 29

2 types of large vessel vasculitis

Answer 29

takayasau's giant cell arteritis

Question 30

2 types of medium vessel vasculitis

Answer 30

polyarteritis nodosa Kawasaki's

Question 31

vessels in which parts of the body are affected by polyarteritis nodosa

Answer 31

gut kidney

Question 32

which infection is polyarteritis nodosa associated with

Answer 32

hep B

Question 33

3 types of ANCA associated small vessel vasculitis

Answer 33

microscopic polyangitis granulomatosis with polyangitis (Wegners) eosinophilic granulomatosis with polyangiitis (Churg Strauss)

Question 34

what clinical sign is characteristic of vasculitis

Answer 34

palpable purpuric rash

Question 35

Tx for GCA

Answer 35

steroids

Question 36

Ix for GCA

Answer 36

ESR

Question 37

Sx of Kawasaki

Answer 37

fever erythema / desquamation of soles / palms conjunctivitis lymphadenopathy coronary arteries may be affected (MI/aneurysms)

Question 38

what is polyarteritis nodosa

Answer 38

necrotising arteritis

Question 39

what cells are high in polyarteritis nodosa

Answer 39

polymorphs, lymphocytes, eosinophils

Question 40

what does the angiography of polyarteritis nodosa look like

Answer 40

nodular apperance - due to small aneurysms

Question 41

triad of hallmark organs that are affected in granulomatosis with polyangiitis

Answer 41

ENT lungs kidneys

Question 42

AB in granulomatotsis with polyangiitis

Answer 42

c-ANCA (against proteinase 3)

Question 43

what is c-ANCA

Answer 43

cytoplasmic ANCA

Question 44

triad of hallmark signs in eosinophilic grnaulomatosis with polyangiitis

Answer 44

asthma eopsinophillia vsaculitis

Question 45

AB in eosinophilic granulomatosis with polyangiitis

Answer 45

p-ANCA (directed against myeloperoxidase)

Question 46

what is p-ANCA

Answer 46

perinuclear ANCA