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Pathology > C - Immune related multisystem disorders (E- Amir Sam) > Flashcards

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

1
Q

Sx of SLE

A

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

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2
Q

AB for SLE

A

anti dsDNA
anti smith
anti histone

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3
Q

which SLE pts gets anti histone ABs

A

drug induced SLE

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4
Q

what % of SLE pts have anti smith ABs

A

30% (but its very specific for SLE)

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5
Q

what protazoa is used to help diagnosis of SLE and how

A

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

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6
Q

why do SLE pts get a malar rash

A

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

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7
Q

kidney histology in SLE

A

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

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8
Q

what type of endocarditis can you get in SLE

A

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

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9
Q

what is scleroderma (systemic sclerosis)

A

fibrosis and excess collagen deposition in skin

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10
Q

2 forms of scleroderma

A

diffuse
limited

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11
Q

ABs in diffuse scleroderma

A

DNA topoisomerase (Scl70)

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12
Q

ABs in limited scleroderma

A

anti centromere

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13
Q

Sx of limited scleroderma

A

CREST
calcinosis
raynauds
eosophageal dysmotility
sclerodactyly
telangiectasia

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14
Q

difference between diffuse and limited scleroderma clinically

A

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

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15
Q

pattern of immunoflurescence in scleroderma

A

nucleolar

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16
Q

histology of artery in scleroderma

A

onion skinning - intimal proliferation (narrowed lumen)

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17
Q

mixed connective tissue disease has features of which other disorders

A

SLE
scleroderma
polymyositis
dermatomyositis

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18
Q

immunoflurescence pattern of mixed connective tissue disease

A

speckled pattern

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19
Q

finger sign of dermatomyositis

A

Gottron’s papules
- erythema over distal IPJ

20
Q

Sx of sarcoidosis

A

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

21
Q

pathological hallmark of sarcoidosis

A

non caseating granulomas

22
Q

what is lupus pernio

A

erythematous lesion involving nose

23
Q

causes of erythema nodosum

A

infection
inflammation - IBD, Behcets, sarcoidosis

24
Q

what is a granuloma

A

ball of lymphocytes

25
Q

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

A

giant cells of langhan

26
Q

what does non caseating mean

A

no necrosis

27
Q

3 metabolic imbalances in sarcoidosis

A

high Ca
high ACE
hypergammaglobulinaemia

28
Q

why do you get high calcium in sarcoid

A

granulomas in lung hydroxylate (activate) Vit D

29
Q

2 types of large vessel vasculitis

A

takayasau’s
giant cell arteritis

30
Q

2 types of medium vessel vasculitis

A

polyarteritis nodosa
Kawasaki’s

31
Q

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

A

gut
kidney

32
Q

which infection is polyarteritis nodosa associated with

A

hep B

33
Q

3 types of ANCA associated small vessel vasculitis

A

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

34
Q

what clinical sign is characteristic of vasculitis

A

palpable purpuric rash

35
Q

Tx for GCA

A

steroids

36
Q

Ix for GCA

A

ESR

37
Q

Sx of Kawasaki

A

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

38
Q

what is polyarteritis nodosa

A

necrotising arteritis

39
Q

what cells are high in polyarteritis nodosa

A

polymorphs, lymphocytes, eosinophils

40
Q

what does the angiography of polyarteritis nodosa look like

A

nodular apperance - due to small aneurysms

41
Q

triad of hallmark organs that are affected in granulomatosis with polyangiitis

A

ENT
lungs
kidneys

42
Q

AB in granulomatotsis with polyangiitis

A

c-ANCA (against proteinase 3)

43
Q

what is c-ANCA

A

cytoplasmic ANCA

44
Q

triad of hallmark signs in eosinophilic grnaulomatosis with polyangiitis

A

asthma
eopsinophillia
vsaculitis

45
Q

AB in eosinophilic granulomatosis with polyangiitis

A

p-ANCA (directed against myeloperoxidase)

46
Q

what is p-ANCA

A

perinuclear ANCA

Pathology (87 decks)

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