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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
nail fold capillary dilatation in scleroderma
26
immunoflurences of scleroderma
In initial ANA test Not very specific Add pt serum to cell - staining in nucleus If nucleolar pattern - suggestive of scleroderma
27
histopath of skin in scleroderma
Excess deposit of collagen Increase depth and amount of collagen
28
histopath of stomach if scleroderm a
Trichrome stain of stomach Blue is collagen = excess collagen - fibrosis = difficulty swallowing
29
artery in scleroderma *onion skin* intimal proliferation -> obliteration of the lumen might have thrombi in lumen
30
features of myositis
paroxysmal muscle weakness and tenderness High creatine kinase
31
what is mixed connective tissue disease
features of all of: * sle * scleroderma * polymyositis * dermatiomyositis
32
what does a speckled pattern on ANA test suggest
mixed connective tissue diease *then look for specifc Ab*
33
Gottron's papules in dermatomyositis high CK erythermatous rash over knuckles and dorsal aspect of hands
34
where does sarcoidosis effect
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
hallmark of sarcoidosis
non-caseating granulomas
36
lupus pernio - in sarcoidosis
37
erythema nodosum
38
causes of erythema nodosum
sarcoidosis IBD bechets
39
causes of erythema nodosum
sarcoidosis IBD bechets
40
causes of bilateral parotid enlargement
mumps alcohol sjorgrens sarcoidosis
41
histopath of sarcoidosis
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
ix for sarcoidosis
hypergammaglobinaemia raised ACE hypercalcaemia - Vit D hydroxylation iby activated macrophages
43
how is vasculitis classifiedn
by size of vessels
44
what is, and features of giant cell arteritis
large vessel vasculitis headache jaw claudication - pain when eat scalp tenderness temporal artery has no pulse tender swollen >50 years
45
what is polyarteritis nodosa
inflammation effecting the vessle -> renal/mesenteric ischemia angiogram: beading of vessels because of small aneurysms associated with Hep B
46
purpuric rash - characteristic of vasculitis
47
nail fold infarcts - vasculitis
48
rx for temporal arteritis
Need high dose steroids and needs biopsy for dx Blood test - ESR
49
histology of temporal arteritis
Narrow of lumen Multiple granulomas Multinuclear giant cells Lymphocytic infiltration in the tunica media
50
features of Kawasaki disease
fever erythema of palms and soles, desquamation conjunctivitis lymphadenopathy coronary arteries may be effected self limiting *effects medium sized vessels*
51
features of polyarteritis nodosa
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
histopathology of polyarteritis nodosa
inflammatory cell infiltrates around vessel
53
what is the hallmark for granulomatosis with polyangitis
ENT, lung, kidneys *although connectie tissue disease so can effect everything*
54
ix for granulomatosis with polyangitis
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
alternative name for Churg-Strauss
eosinophilic granulomatosis with poolyangitis
56
features of churg-strauss
asthma eosinophilia vasculitis
57
ix for churg-strauss
p-ANCA (perinuclear ANCA) directed against myeloperoxidase Cytoplasm of neutrophils in perinuclear pattern - around nuclear

PATHOLOGY (94 decks)

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