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Year 5 😊✌🏼 > Path 6 > Flashcards

Path 6 Flashcards

1
Q

Features of SLE

A

can affect any organ!!

fever, fatigue, weight loss - flare ups

skin - e.g. malar rash
oral ulcers
joints (arthritis)
neurological (e.g. seizures, psychosis)
serositis (e.g. pericarditis, pleuritis; recurrent abdo pain)
renal (glomerulonephritis, protein +)
haematological (pancytopenia)
immunological (autoantibodies)

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2
Q

40yo female with pancytopenia and proteinuria - dx?

A

SLE

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3
Q

Antoantibodies in SLE

A

ANA - used for screening, does not show which antibody specifically is involved

Anti-dsDNA

Anti-Smith (ribonucleoprotiens) - most SPECIFIC (if +ve, v likely to have SLE) but not very sensitive (30% people with SLE have it)

Anti-histone (seen in drug induced lupus e.g. hydrazalazine

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4
Q

What medications can cause drug induced lupus?
Which antibody is positive?

A

hydralazine
procainamide

anti histone antibody

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5
Q

if a number is stated along with ANA, what does it mean?

A

the dilution of the serum at which the test is +ve, e.g. with 1/1000 dilution -> significantly high ANA

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6
Q

What is the underlying skin histology in a malar rash/SLE skin?

A

lymphocytic infiltration of the upper dermis

basal epidermis has undergone vacuolisation

red cells extraposition in the dermis

Immunofluorescence: immune complex deposition at epidermis-dermis junction

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7
Q

SLE skin - where are immune complexes deposited?

How can you see them (method)?

A

epidermis dermis junction

seen with immunofluorescence (Immunoglobulin binding)

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8
Q

What histological changes can you see lupus nephritis?

A
  • thickening of glomerular capillaries (thick wall, wire loop pathology appearance) - due to immune complex deposition in BM
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9
Q

What causes wire loop pathology in lupus nephritis?

A

deposition of immune complexes in the BM leading to thickening of glomerular wall

can be seen on immunofluorescence or electron microscopy

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10
Q

What is Libman sacks?

A

non-infective endocarditis seen in SLE

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11
Q

How does Libman sacks endocarditis present?

A

emboli
stroke
murmurs

the vegetations on the valves are lymphocytes, neutrophils, eosinophils, fibrin strands etc.

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12
Q

What is the problem in Scleroderma?

A

fibrosis and excess collagen in the skin

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13
Q

what is the localised form of scleroderma called?

A

morphoea in the skin (patch of tight skin)

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14
Q

What are the 2 types of systemic sclerosis/scleroderma?

A

diffuse

limited

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15
Q

are scleroderma and systemic sclerosis synonymous?

A

yes

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16
Q

What is the difference between diffuse and limited scleroderma/systemic sclerosis?

A

diffuse - trunk is involved

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17
Q

What is more common, diffuse or limited scleroderma?

A

limited is more common

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18
Q

What autoantibodies are diffuse and limited scleroderma associated with?

A

Diffuse: anti-scl70 (antibodies to DNA topoisomerase)

Limited: anticentromere

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19
Q

What is CREST syndrome?

A

Term no longer used!!

describes limited scelroderma

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20
Q

CREST - what does it stand for?

A

Calcinosis
Raynauds
Oesophageal dysmotility
Sclerodactyly
Telegiectasia

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21
Q

oder of colour change in raynauds?

A

white
blue
crimson

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22
Q

What is sclerodactyly?

A

thinkening of the skin of the fingers+hand causing them to curl inwards causing a claw deformity and limitation in movement

seen in scleroderma

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23
Q

What pattern can be seen on IF in scleroderma?

A

nucleolar pattern

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24
Q

How common is raynauds in the general population (females)?

A

1 in 10

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25
Q

feautures of systemic sclerosis

A

calcinosis
raynauds
oesophageal dysmotility
sclerodactyly
telangiecstasia

nail fold capillary dilatation

microstomia (difficulty opening small mouth)

onion skin - intimal proliferation of arterioles +/- obliteration of the lumen. may have thrombi.

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26
Q

What causes skin tightening in scleroderma?

A

excess collagen deposition in the dermis

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27
Q

stomach changes in scleroderma

A

excess collagen and fibrosis -> causes oesophageal dysmotility

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28
Q

What is mixed connective tissue disease?

A

Patients have features of:

SLE
scleroderma
polymyositis
dermatomyositis

OVERLAP

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29
Q

What enzyme is elevated in poly/dermatomyositis and why?

A

CK

it is leaking out of inflamed muscle

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30
Q

What pattern of ANA can you see in mixed connective tissue disease?

A

speckled pattern

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31
Q

ANA - speckled pattern - dx?

A

mixed connective tissue disease

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32
Q

ANA - nucleolar pattern - dx?

A

scleroderma

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33
Q

ANA - entire nucleus stained pattern - dx?

A

SLE

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34
Q

What ANA patterns can you see and what conditions are they suggestive of?

What type of test is ANA?

A

whole nucleus involved - SLE

nucleolar pattern - scleroderma

speckled pattern - mixed connective tissue disease

ANA is a screening test, you would want to do further testing for autoantibodies.

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35
Q

What are Gottron’s papules and what condition are they a feature of?

A

erythematous rash over knuckles on dorsal aspect of hands

dermatomyositis

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36
Q

Dermatomyositis features

A

Tender, inflamed muscles

raised CK

Gottron’s papules

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37
Q

Features of sarcoidosis

A

Joints (e.g. back pain)
skin (nodules, papules, lupus pernio, erythema nodosum)
lungs (BHL, fibrosis, cough, lymphocytosis - CD4+ on BAL)
lymphadenopathy
parotids (bilateral enlargement)

heart
eyes (uveitis, conjunctivitis)
neuro (meningitis, cranial nerve lesions)
liver (hepatitis, cirrhosis, cholestatis )

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38
Q

What is the pathological hallmark of sarcoidosis?

A

non-caseating granulomas

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39
Q

What conditions give you erythema nodosum?

A

sarcoidosis
IBD
Bechets

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40
Q

Causes of bilateral parotid enlargement

A

Infection: mumps

Inflammation: sarcoidosis, Sjorgens syndrome

alcoholism

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41
Q

BHL

A

bilateral hilar lymphadenopathy

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42
Q

histopathological features of sarcoidosis

A

non-caseating granulomas

epithelioid looking cells (with elongated nuclei)

Langhans cells (multinucleate giant cells with horse shoe appearance/distribution of nuclei; multiple macrophages fusing)

43
Q

what does non-caseating mean?

A

there is no necrosis occurring

44
Q

Test findings in sarcoidosis?

A
  • hypergammaglobulinaemia
  • raised ACE due to abnormalities of capillaries in the lungs where ACE is made
  • hypercalcaemia (vit D hydroxylation by activated macrophages)
45
Q

What causes hypercalcaemia in sarcoidosis?

A

increased Vitamin D3 hydroxylation by activated macrophages

46
Q

Types of vasculitis (3)

A

large vessel
medium vessel
small vessel

47
Q

types of large vessel vasculitis

A

Giant cell arteritis
takayasus arteritis

48
Q

types of medium vessel arteritis

A

kawasaki’s
polyarteritis nodosa

49
Q

types of small vessel vasculitis

A

Immune Complex SVV
- cryoglobulinaemic vasculitis
- IgA vasculitis (Henoch Schonlein)
- Hypocomplementeric urticarial vasculitis

ANCA-associated SVV
- microscopic polyangiitis
- granulomatosis with polyangiitis (Wegener’s)
- eosinophilic granulomatosis with polyangitis (Churg-Strauss)

50
Q

What infection is polyarteritis nodosa associated with?

A

Hep B

51
Q

What vasculitis is Hep B infection associated with?

A

polyarteritis nodosa (medium vessel vasculitis)

52
Q

What can be seen on angiogram in polyarteritis nodosa?

A

multiple beads (small aneurysms)

53
Q

What kind of rash is characteristic of vasculitis?

A

palpable purpuric rash

can be seen in any type of vasculitis

54
Q

primary and secondary vasculitis

A

primary vasculitis (large, medium, small vessel)

OR

secondary to infective endocarditis, RA, SLE…

55
Q

histology of temporal arteritis

A

narrowing of lumen
granulomas
multinucleate giant cell with horseshoe arrangement of nuclei
lymphocytic infiltration of tunica media

56
Q

Histopath of polyarteritis nodosa

A

necrotising arteritis

inflammatory cell infiltrates around vessels: polymorphs, lymphocytes and eosinophils seen

arteritis is focal and sharply demarcated

heals by fibrosis

57
Q

what vessels are most commonly affected by polyarteritis nodosa?

A

renal and mesenteric arteries

58
Q

3 hallmarks of granulomatosis with polyangitis

A

ENT
Lung
kidneys

59
Q

What antibody to test for in ?GPA

A

C-ANCA (cytoplasmic ANCA) directed against proteinase 3

60
Q

What does C-ANCA bind to?

A

proteinase 3

61
Q

When is C-ANCA positive?

A

GPA

small vessel ANCA vasculitis

ENT, lung and kidney issues

(used to be called Wegeners)

62
Q

What is the triad of sx in eosinophilic granulomatosis with polyangitis?

A

asthma
eosinophlia
vasculitis

63
Q

What ab is +ve in eosinophilic granulomatosis with polyangitis?

A

P-ANCA (perinuclear ANCA)

directed against myeloperoxidase

64
Q

What is P-ANCA directed against?

A

myeloperoxidase

65
Q

What ab to test for in ?eosinophilic granulomatosis with polyangitis (Churg Strauss)

A

P-ANCA

66
Q

How do you manage Church Strauss syndrome?

A

glucocorticoids PLUS cyclophosphamide

Goal is induction of remission

67
Q

What do 1% of children affected by Kawasaki die off? What happens with the rest?

A

MI

99% have self limiting course

68
Q

onion skin - what is it a histopathological feature of?

A

onion skin - intimal proliferation of arterioles +/- obliteration of the lumen. may have thrombi.

systemic sclerosis

69
Q

What does anisopoikilocytosis mean?

A

there is a variation size (aniso) and shape (poikilo) of RBCs

70
Q

hypersegmented neutrophil - ddx

A

B12 deficiency
folate deficiency
drugs (e.g. methotrexate, 5-FU, hydroxyurea, hydroxycaarbamide)

71
Q

def of hypersegemnted neutrophils

A

> 5 nuclear segments

72
Q

codocytes

A

target cells

central area of increased haemoglobin

73
Q

target cells - ddx

A

iron deficiency
thalassemia
hyposplenism
liver disease

74
Q

Howell Jolly bodies - what are they and when are they seen?

A

remnants of nucleus

usually removed by spleen -> seen in hyposplenism

75
Q

features of iron deficiency on blood film

A

microcytosis
hypochromia
anisopoikilocytosis

76
Q

hyposplenic features on blood film

A

target cells
Howell jolly bodies

77
Q

Causes of iron deficiency

A

blood loss - major cause

other causes:
- poor diet
- malabsorption
- combinations of the above

78
Q

Causes of megaloblastic change

A

B12 and folate deficiency
drugs

79
Q

causes of hyposplenism

A

absent spleen (therapeutic, trauma)

poorly functioning spelen
IBD
ceoliac disease
SLE
sickle cell disease

80
Q

Deficiency seen in coeliac

A

iron
b12
folate
fat
ca

81
Q

deficiency seen in Crohn’s disease

A

b12
bile acids

82
Q

pancreatic disease deficiency

A
83
Q

xxx deficiency

A
84
Q

where is tissue traanssglutaminase expressed?

A

endomysosal layer

therefore also called

85
Q
A

also check IgA levels (some peeople are IgA deficient)

86
Q
A

also check IgA levels (some peeople are IgA deficient)

87
Q
A

antibody disappears as people stop eating gluten

88
Q

Why would you perform an endoscopy in ?coeliac disease?

A

baseline

89
Q

Gold standard for coeliac disease diagnosis

A

(disstal) duodenal biopsy

90
Q

endoscopy findings in coeliac disease

A

scalloping of ridges in duodenum (lumps rather than villi)

91
Q

normal villous : crypt ratio

A

3-5 : 1

villii are the majority of the biopsy

92
Q

normal duodenal vili intro epithelial lymphocyte number

A

20 intraepithelial lymphocytes / 100 epithelial cells

93
Q

Brunners glands - where are they ?

A

in the proximal half of the duodenum

they may distort villous architecture

94
Q

histopathology in coeliac disease

A

Villous atrophy - villous height is reduced (atrophy)

Crypt hyperplasticity - crypt thickness is increased (hyperplasticity)

increased IEL : >25 IEL / 100 epithelial cells

these changes disappear if they stop eating gluten

95
Q

Causes of increased IEL

A

coeliaca
infections
bacterial overgworh
drugs
IBD
Immune dysregulation

96
Q

most specific test for coeliac disease?

A

anti-tissue transglutaminase IgA

97
Q

Long term complications of coeliac disease

A
  • malabssorption
  • osteomalacia
  • osteoporosis
  • neurological disease (epilepsy, cerebral calcification)
  • T-cell lymphoma of the bowel
  • hyposplenism

Important to stick to the diet to prevent these complications

98
Q

genetic components of coeliac diesase

A

HLA-DQ2
increased risk to family members

99
Q

how often DEXA scan of hip in coeliac disease?

A

every 3-5 y

b/c of the risk of osteomalacia/osteoporosis

100
Q

Lambert-Eaton myasthenic syndrome - what malignancy is it associated with?

A

Small cell Lung cancer

101
Q

What syndromes is SCLC associated with?

A

SIADH (15% of patients)
Cushing’s syndrome (5%)
Lambert-Eaton Myasthenic syndrome (3%)
acromegaly

102
Q

What infection does the presence of galactomannan in the serum of a septic patient suggest?

A

Aspergillus

103
Q

Beta-D-Glucan in serum - what does it indicate?

A

candida
aspergillus
PCP

104
Q

Glucuronoxylomannan (GXM) in serum - what does it indicate?

A

cryptococcus

Year 5 😊✌🏼 (53 decks)

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