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PATHOLOGY > immune related connective tissue disorders > Flashcards

immune related connective tissue disorders Flashcards

1
Q

eg of organ specific autoimmune disease with organ specific ag

A

pernicious anaemia

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2
Q

eg og organ specific autoimmune disease w/o organ specific ag

A

primary biliary cirrhosis

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3
Q

eg of multisystem autoimmune disease`

A

rheumatoid arthritis
sjorgren’s syndrome
SLE

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4
Q

features of pernicious anaemia

A

Low Hb, high MCV

Autoab against parietal cell
that make intrinsic factor - needed for B12 absorption

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5
Q

features of primary biliary cirrhosis

A

Jaundice
Puritis
Raised LFT - high alk phos

No specific antigen, but it is a specific disorder

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6
Q

features of SLE

A

chronic condition

effects small vessels -> raynauds and gangrene

skin
* malar rash
* discoid rash/lupus
* photosensitivity

kidney:
* proteinuria
* haematuria
* kidney failure
* glomerulonephritis

oral ulcer
pancytopenia
neuropsych
* psychosis
* depression

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7
Q

what is a titre

A

The highest dilutation that you see the positive test is the titre. The more diluted = the more ANA

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8
Q

what are the autoab in SLE

A

anti-dsDNA
anti-smith (against ribonucleoproteins)
antihistone (drug related eg hydralazine ie drgs lead to SLE as a complication)

anti-smith is MOST SPECIFIC - if +ve = definitely SLE

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9
Q

2 methods of measuring dsDNA

A
  1. incubate serum with protazoa (crithidia luciliae) this parasite has large mt with a lot of dsDNA
  2. so if patient has anti-dsDNA then it will bidn
  3. the big mitochondrial DNA is called a Kinetoplast

the other method
1. ELISA
2. have dsDNA - add serum
3. add other Ab which is conjugated to enzyme
4. add substrate -> vchnage in colourb

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10
Q

what is the LE cell in SLE

A

dont use this test anymore

Pink blob is denatured nuclei - engulfed by neutrophils
Used to be called LE cell

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11
Q

histopathology of skin in SLE

A

lymphocytic infiltration of the ypper dermis
basal dermis has undergone vacuolisation - ie disolution of cells
ie damage to basal dermis

extravasation of RBC in upper dermis -> rash

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12
Q

skin under IF in SLE

A

Immune complex deposition in the demis-epidermis junction
Ig-complement = immune complex

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13
Q

what is this

A

normal glomerulus
thin wall of capillaries

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14
Q

histopath of glomerulus in SLE

A

thickening of the capillaries wall - wire loop capillaries
because have immune complex deposition in the basement membrane

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15
Q

immunoflurences and electron microscope of SLE

A

deposition of immune complexes

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16
Q

what is Libman sacks in SLE

A

non-infective endocarditis

presents with
* emboli
* stroke
* HF
* murmur

lymphocytes, neutrophils, eosinophils and fibrin strands

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17
Q

what is scleroderma (systemic sclerosis)

A

fibrosis and extra collagen
localised form called - morphoea in the skin
there is a diffuse and limited form
nucleolar pattern of immunoflurences

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18
Q

ab in diffuse form of SLE

A

DNA topoisomerase (scl70)

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19
Q

ab and features of limited form of scleroderma

A

ab: anticentromere Ab

features: CREST:
* calcinosis
* raynauds
* eosophageal dysmotility
* sclerdactyly
* telangiectasia

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20
Q

area of skin involved in limited vs diffuse sscleroderma

A
  • In trunkal skin is involved = diffuse
    If no trunkal involved = limited
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21
Q
A

calcinosis - on tip of finger = painfu

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22
Q
A

raynauds

goes whitre -> blue -> red

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23
Q
A

sclerodactyly

tightness over skin of fingers

24
Q
A

telangiectasia and microstomia

cant open mouth because of tight skin

25
Q
A

nail fold capillary dilatation in scleroderma

26
Q

immunoflurences of scleroderma

A

In initial ANA test
Not very specific
Add pt serum to cell - staining in nucleus
If nucleolar pattern - suggestive of scleroderma

27
Q

histopath of skin in scleroderma

A

Excess deposit of collagen
Increase depth and amount of collagen

28
Q

histopath of stomach if scleroderm a

A

Trichrome stain of stomach

Blue is collagen = excess collagen - fibrosis = difficulty swallowing

29
Q
A

artery in scleroderma

onion skin
intimal proliferation -> obliteration of the lumen

might have thrombi in lumen

30
Q

features of myositis

A

paroxysmal muscle weakness and tenderness
High creatine kinase

31
Q

what is mixed connective tissue disease

A

features of all of:
* sle
* scleroderma
* polymyositis
* dermatiomyositis

32
Q

what does a speckled pattern on ANA test suggest

A

mixed connective tissue diease

then look for specifc Ab

33
Q
A

Gottron’s papules in dermatomyositis

high CK

erythermatous rash over knuckles and dorsal aspect of hands

34
Q

where does sarcoidosis effect

A

lymphadenopathy
* hilar

lungs
* dry cough
* breathless

skin
* lesions
* nodules and papules
* lupus pernio - rash over nose

joints
* back ache
* arthralgia

heart
* epicardium = pericarditis
* muscle = HF
* endocardium = valvular lesions

eyes
* uveitis
* conjunctivitis
* anterior uveitis

brain
* meningitis
* effect cranial nerve

liver
* hepatitis
* cirrhosis

35
Q

hallmark of sarcoidosis

A

non-caseating granulomas

36
Q
A

lupus pernio - in sarcoidosis

37
Q
A

erythema nodosum

38
Q

causes of erythema nodosum

A

sarcoidosis
IBD
bechets

39
Q

causes of erythema nodosum

A

sarcoidosis
IBD
bechets

40
Q

causes of bilateral parotid enlargement

A

mumps
alcohol
sjorgrens
sarcoidosis

41
Q

histopath of sarcoidosis

A

Granuloma - ball of macrophages
Histiocytes - activated macrophages
Epithelioid cells
Macrophages fuse to form multinucleate giant cells of langhans - with horseshow appearance

Non-caseating - ie no necrosis

42
Q

ix for sarcoidosis

A

hypergammaglobinaemia
raised ACE
hypercalcaemia - Vit D hydroxylation iby activated macrophages

43
Q

how is vasculitis classifiedn

A

by size of vessels

44
Q

what is, and features of giant cell arteritis

A

large vessel vasculitis

headache
jaw claudication - pain when eat
scalp tenderness
temporal artery has no pulse
tender
swollen
>50 years

45
Q

what is polyarteritis nodosa

A

inflammation effecting the vessle
->
renal/mesenteric ischemia

angiogram: beading of vessels because of small aneurysms

associated with Hep B

46
Q
A

purpuric rash - characteristic of vasculitis

47
Q
A

nail fold infarcts - vasculitis

48
Q

rx for temporal arteritis

A

Need high dose steroids and needs biopsy for dx
Blood test - ESR

49
Q

histology of temporal arteritis

A

Narrow of lumen
Multiple granulomas
Multinuclear giant cells
Lymphocytic infiltration in the tunica media

50
Q

features of Kawasaki disease

A

fever
erythema of palms and soles, desquamation
conjunctivitis
lymphadenopathy
coronary arteries may be effected
self limiting

effects medium sized vessels

51
Q

features of polyarteritis nodosa

A

necrotising arteritis
effects multiple vessels
infiltration around vessles - polymorphs, lymphocytes, eosinophils

arteritis is focal and sharply demarcated

heals by fibrosis

more often renal and mesenteric arteries

52
Q

histopathology of polyarteritis nodosa

A

inflammatory cell infiltrates around vessel

53
Q

what is the hallmark for granulomatosis with polyangitis

A

ENT, lung, kidneys

although connectie tissue disease so can effect everything

54
Q

ix for granulomatosis with polyangitis

A

if have triad (ENT, lung, kidneyy) then request c_ANCA

the Ab binds to the cytoplasm of neutrophils
c-ANCA binds to protease 3

55
Q

alternative name for Churg-Strauss

A

eosinophilic granulomatosis with poolyangitis

56
Q

features of churg-strauss

A

asthma
eosinophilia
vasculitis

57
Q

ix for churg-strauss

A

p-ANCA (perinuclear ANCA) directed against myeloperoxidase

Cytoplasm of neutrophils in perinuclear pattern - around nuclear

PATHOLOGY (94 decks)

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