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4 CSIM2 > multisystem disease > Flashcards

multisystem disease Flashcards

1
Q

pathophysiology of autoimmune connective tissue disease?

A

immune response against self antigen, producing antibodies, immune complexes and cellular activity against self-tissue.

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2
Q

common history complaints of someone with an aCTD

A

fatigue, anorexia, fever, weight loss

skin changes/rashes, joint paints, muscle pains, dry eyes/mouth, sicca symptoms, raynauds phenomenon

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3
Q

what are sicca symptoms

A

dry eyes, dry mouth, conjunctivities, episcleritis, vaginal dryness

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4
Q

what can be seen on examination of someone with aCTD

A

rash, joint pain, pigmentation, alopecia, endocarditis

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5
Q

what are some aCTDs

A

SLE, antiphospholipid syndrome, systemic sclerosis, polymyositis/myositis, dermatomyositis, mixed CTD

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6
Q

how does SLE happen

A

failure to clear apoptosed cells, leaving remnants that are ingested by APC who then trigger lymphocyte production of antibodies against self tissue. along with failure of self-regulation, inflammatory state and immune-complex deposition, causes multisystem disease.

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7
Q

systems that can be affected in SLE

A

CNS, eyes, mouth, joints, skin, heart, lungs, GI, kidneys, vasculature, blood

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8
Q

what are some CNS symptoms in SLE

A

psychosis, confusion, hemiplegia, ataxia, cranial nerve lesions, fits

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9
Q

what are some eye symptoms in SLE

A

dry eyes, scleritis, episcleritis, conjunctivitis, optic neuritis

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10
Q

what are some mouth symptoms in SLE

A

mouth ulcers, dry mouth

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11
Q

what are some joint symptoms in SLE

A

joint pains, muscle pains,

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12
Q

what are some GI symptoms in SLE

A

mesenteric vasculitis

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13
Q

what are some skin symptoms in SLE

A 
lupus erythema
discoid lupus
raynauds phenomenon
photosensitive rash
butterfly rash
livedo
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14
Q

what are some cardiovascular signs in SLE

A

percarditis
endocarditis
aortic valve lesions

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15
Q

what are some vascular signs in SLE

A

livedo
raynauds
nail and finger tip lesions

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16
Q

what are some kidney signs in SLE

A

glomerulonephritis, Proteinuria, haematuria, AKI

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17
Q

what are some haematological signs of SLE

A

lymphopenia, neutropenia, thrombocytopenia,
anaemia of chronic dz or haemolytic anaemia
low C3, C4
raised ESR (but often normal CRP)
auto antibodies

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18
Q

what are some lung complications of SLE

A 
restrictive lung disease
raised hemidiaphrams - "shrinking lung"
pleural effusions (exudative)
pneumonitis
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19
Q

is the pleural effusion in SLE transudative or exudative?

A

exudative

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20
Q

what are the antibodies associated with SLE?

A 
anti-dsDNA 
anti-Sm
anti-ro anti-la
anti-histone (drug induced SLE)
anti RNP
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21
Q

what auto antibody if seen, is specific to SLE?

A

anti-smith

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22
Q

what is the most commonly seen auto antibody in lupus?

A

anti dsDNA

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23
Q

what auto antibodies produce a ‘speckled’ sign on immunohistochemistry

A

anti-RO anti-LA, SCL-70, and RNA polymerase III

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24
Q

what auto antibody is associated with drug induced SLE?

A

anti-histone

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25
Q

what will ESR and CRP be like in SLE?

A

high ESR, normal CRP

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26
Q

what can be seen on histology in SLE?

A

immune-complex deposition in skin and/or kidneys

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27
Q

what does a false +ve VDRL suggest

A

SLE

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28
Q

what are some life threatening complications of SLE

A

renal crisis
pulmonary haemorrhage
catastrophic antiphospholipid

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29
Q

what coagulation syndrome is associated with SLE ?

A

anti-phospholipid syndrome

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30
Q

what antibodies are associate with antiphospolipid syndrome?

A

lupus coagulant
anti-cardiolipin
anti-b2GP

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31
Q

what is the behaviour of lupus coagulant? in vitro/vivo

A

in vitro it is antocoagulative

in vivo it is prothrombotic

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32
Q

what is anti-cardiolipin associated with

A

antiphospholipid syndrome

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33
Q

what syndrome is associated with lupus anticoagulant?

A

antiphospholipid syndrome

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34
Q

how will antiphospholipid syndrome affect pregnancy?

A

recurrent miscarriages

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35
Q

what can be seen on bone marrow biopsy in SLE?

A

lupus erythema cell (LE cell)

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36
Q

what is more worrying of SLE - 1:80 ANA titre found in a 22 year old, or 1:80 ANA titre found in a 79 year old?

A

the younger patient.

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37
Q

pathphysiology of systemic sclerosis

A

endothelial damage leading to immune/inflammatory response, leading to cellular infiltration which causes more damage, ischaemia. also activation of fibroblasts

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38
Q

who is the pathophysiology of fibrosis in systemic sclerosis

A

inflammation activates fibroblasts which produces more collagen, causing fibrosis in lower dermis and internal organs

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39
Q

common clinical features of systemic sclerosis

A 
raynauds phenomenon
lung disease - pulm H/T and/or fibrosis
skin tightening - fingers, arms, face, neck
GI dysfunction - dysphagia
dilated nail capillary loops
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40
Q

how to tell between limited cutaenous Sc and diffused cutanaeous Sc

A

LcSSc is associated with CREST syndrome. more pulm hypertension, skin sclerosis usually distal to elbows, less systemic symptoms

DcSSc is more associated with fibrotic lung disease, systemic symptoms (fatigue, anorexia, weight loss), more widespread organ involvement, renal impairment, edema, more severe skin sclerosis that can lead to ulceration

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41
Q

difference in autoantibodies found in LcSSc and DcSSc

A

LcSSc is associated with anti-centromere antibodies

DsSSc is associated with antiSCL70 and antipolymerase

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42
Q

what are auto antibodies are found in systemic sclerosis?

A

rheumatoid factor (30%) and ANA (95%)

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43
Q

how to investigate systemic sclerosis?

A

FBC - Nchr Ncyt anaemia, or haemolytic microangiopathy in renal involvement

U&Es, urine dip, microscopy, ACR

autoantibody testing

nail fold capilloscopy

pulmonary function test

ECHO, BNP

imaging - CXR, xray hands, HRCT, barium swallow/OGD

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44
Q

explain what can be found on imaging in systemic sclerosis

A

CXR - heart enlargement, lung disease
xray hands - calcinosis
HRCT - fibrotic lung disease
barium swallow/OGD - oesophageal dysmotility

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45
Q

U&Es are always derranged in systemic sclerosis - T or F?

A

F, only if renal involvement

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46
Q

what is anti-topoisomerase antibody AKA?

A

anti-scl70

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47
Q

which auto antibody is associated with pulmonary hypertension?

A

anti RNA polymerase antibody

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48
Q

complications of systemic sclerosis?

A

pulmonary function
renal function
erectile dysfunction
GI dysfunction

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49
Q

why is pulmonary function testing indicated in systemic sclerosis?

A

monitoring of fibrotic lung disease

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50
Q

what is CREST syndrome?

A 
calcinosis
raynauds
esophageal dysmotility
sclerodactyly
telangeictasia
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51
Q

what is morphea and when is it seen?

A

localised scleroderma - thickening of skin due to increased collagen production

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52
Q

symptoms of sjogrens syndrome

A

sicca
fatigue
joint involvement
renal tubular acidosis

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53
Q

how does sicca symptoms present

A

dry eyes
dry mouth/throat - coughing
dry vagina

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54
Q

what cancer is sjogrens associated with

A

non-hodgkins lymphoma

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55
Q

what antibodies is sjogrens syndrome associated with

A

ANA
rheumatoid factor
anti Ro, anti LA

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56
Q

how to investigate sjogrens syndrome

A 
auto antibodies
ESR (raised)
complement (low)
immunoglobulins (raised)
schirmer's tear test (test how much tears produced)
salivary flow test (reduced)
parotid and mandibular gland USS
lip biopsy (can see inflammation)
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57
Q

how to tell the difference between myalgia secondary to an autoimmune disease VS primary inflammatory myositis?

A

secondary mylagia usually doesn’t cause weakness, and will have normal muscle enzymes (CK, LDH)

myositis will cause muscle weakness, and raised CK and LDH, usually proximal muscles

58
Q

clinical features of polymyositis

A

insidious onset with systemic symptoms (tiredness, weight loss. anorexia, fevers)

muscle weakness and pain (proximal)

59
Q

describe the course of muscle weakness in polymyositis

A

starts with proximal weakness, then pain, usually shoulder and pelvis, in later stages, can involvement pharynx, larynx causing dysphonia/dysphagia, then respiratory muscles become affected causing respiratory failure

60
Q

facial and hand muscles are not commonly involved in polymyositis - T or F

A

T

61
Q

what is dermatomyositis associated with

A

cancer

62
Q

what are signs of dermatomyositis

A

myositis + skin involvement

gottron's papules
shawl sign (photosensitive rash)
heliotropic rash (around eyes)

periorbital edema

63
Q

what are gottron’s papules

A

purplish/red lichenoid rash around fingers, knucles, extensor surfaces

64
Q

how to investigate (dermato/poly)myositis

A

bloods - commonly raised ESR/CRP, muscle enzymes (CKD, LDH, Trop T). autoantibodies

EMG, MRI, muscle biopsy

cancer screen in dermatomyositis

65
Q

what autoantibodies are commonly found in polymyositis

A

ANA

anti-Jo-1

66
Q

why is having sjogrens in pregnancy dangerous for the fetus?

A

sjogrens is associated with anti-Ro antibodies which can cross the placenta to cause heart blocks

67
Q

pathogenesis of antiphospholipid syndrome?

A

antiphospholipid antibodies bind to a protein which causes inhibition of protein C. protein C is a anticoagulation protein (cleaves factor 5). without which, the system will become prothrombotic

68
Q

how can antiphospholipid syndrome present?

A

any clotting event - DVT, stroke, PE

recurrent miscarriages

69
Q

what is a skin sign of antiphospholipid syndrome

A

livedo

70
Q

what are the 2 associated antibodies in antiphospholipid syndrome

A

anti-cardiolipin

lupus anticoagulant

71
Q

what pregnancy conditions are women more at risk at if they have an aCTD?

A

preeclampsia, eclampsia, fetal growth restriction, preterm delivery.

72
Q

what should be done if a woman with aCTD wants to get pregnant

A

planning, counselling, disease remission for 6 months before trying.

73
Q

what should be prescribed prophylactically if someone has an aCTD and is pregnant

A

low dose aspirin

74
Q

what DMARDs are safe in pregnancy?

A

hydroxychloroquine, azathioprine, methylprednisolone, rituximab in 1st trimester.

75
Q

why might a woman with SLE give birth to a baby with rashes

A

could be neonatal lupus - self limiting

76
Q

what autoantibody is mixed CTD associated with

A

anti-RNP

77
Q

what conditions is mixed CTD more associated with

A

joint erosions

pulmonary hypertension

78
Q

what is anti-Jo-1 associated with

A

myositis

79
Q

what conditions can be seen in mixed CTD

A

rheumatoid arthritis
SLE
scleroderma
myositis

80
Q

why might someone with dry eyes also complain of a cough?

A

(sjogrens) dry mouth/throat can cause chronic cough

81
Q

why might someone with sjogrens complain of swollen lymph nodes?

A

could be lymphoma

82
Q

which auto antibodies give a homogenous picture on Immunofluorescence?

A

anti-dsDNA

anti-histone

83
Q

pathophysiology of giant cell arteritis?

A

granulomatous inflammation of large arteries

84
Q

clinical features of PMR

A 
pain shoulder, hips, lumbar, neck (worse in morning)
systemic symptoms (fever, tiredness, weight loss, anorexia)
85
Q

who gets PMR?

A

usually >50 and female

86
Q

investigations to do in PMR?

A

FBC (CRP, ESR raised, normochromic normocytic anaemia)

raised alk phos and GGT (acute phase reactants)

temporal artery biopsy if indicated

87
Q

signs/symptoms of giant cell arteritis

A

severe headache
scalp tenderness
eye symptoms
jaw claudication

88
Q

what are some occular complications u can get with giant cell arteritis ?

A

amurosis fugax
blurred vision
diplopia
permanent loss of viseion

89
Q

what will be seen on a histological biopsy in affected giant cell arteritis

A

T cell, macrophage, granulomatous inflammation. +/- giant cell

90
Q

what imaging should be done in someone with GCA?

A

serial CXR to monitor for AA/AD

91
Q

what kind of vasculitis is polyarteritis nodosa?

A

medium sized vessels

92
Q

who gets polyarteritis nodosa

A

middle aged men

93
Q

clinical features of polyarteritis nodosa?

A 
systemic symptoms
mononeuritis multiplex
abdominal pain
GI bleeding
acute/chronic renal failure
cardiac (mi, pericarditis, heart failure)
skin (purpura, livedo, gangrene)
94
Q

investigations in polyarteritis nodosa?

A 
FBC (anaemia, raised esr, raised wcc)
U&Es
ECG
aniogram 
biopsy
investigation of any affected organ
95
Q

what is the pathophysiology of a vasculitis?

A

a trigger causing endothelial damage, causing immune response towards endothelium of vessels, creating antibody-immune complex deposition onto endothelium, causing inflammation and imune destruction of endothelium, causing damage to vessels leading ischaemia, necrosis, gangrene

96
Q

common symptoms of small vessel vasculitides

A

systemic symptoms - fever, weight loss, fatigue, myalgia, arthralgia

eye involvement - scleritis, scleromalacia

ENT - sinusitis, epistaxis, nose collapse, stridor

mucus membranes - genitooral ulcers

skin - rash, livedo, purpura, ulceration, necrosis, gangrene

pulmonary - lung haemorrhage, haemoptysis, chest pain, crackles, wheeze

kidney - AKI/chronic kidney disease

abdo - pain, bloody stools, obstruction

97
Q

difference between GPA and MPA

A

GPA is more associated with C-ANCA, MPA is more associated with P-ANCA

GPA tends to be more diffused and multisystemic

MPA tends to involve kidneys more

98
Q

what is the classical presentation of EGPA

A

late onset asthma with no prior hx of atopy

99
Q

what is HSP

A

henoch schoenlein purpura

rash, arthalgia, abdo pain, with possible AKI

100
Q

what is important to be done in HSP

A

urine dipstick, kidney involvement testing

101
Q

which ANCA antibody is HSP associated with?

A

none

102
Q

small vasculitic purpuric rashes vs one bigger vasculitic lesion, which is more serious?

A

the bigger patch, because it indicates a larger vessel involvement

103
Q

how to tell the difference between a vasculitic purpura VS a thrombotic purpura?

A

vasculitis involves inflammation which will cause the lesions to be raised - palpable

104
Q

what does a blue/purple edge of a lesions indicate?

A

vasculitis, due to start of necrosis

105
Q

causes of raynauds ?

A

primary = idiopathic

secondary: SLE, SSC, dermatomyositis/polymyositis, sjogrens, RA, mCTD, takayasu’s arteritis, some drugs

106
Q

what are some drugs that can cause raynauds

A

beta blockers, cytotoxic drugs (e.g. bleomycin), sulfasalazine

107
Q

what does unilateral raynauds imply?

A

localised vascular problem

108
Q

what HLA gene is antiphospholipid syndrome associated with?

A

HLA-DR7

109
Q

what is the pathophysiology of livedo reticularis?

A

blocking/obstruction/constriction of capillaries

110
Q

what are some autoimmune causes of livedo?

A

SLE, dermatomyositis, rheumatoid arthritis, polyarteritis nodosa

111
Q

what are some vascular causes of livedo?

A

antiphospholipid syndrome
thrombocytosis
polycythaemia
cryoglobulinaemia

112
Q

what is mononeuritis multiplex?

A

neuropathy of multiple individual nerve trunks

113
Q

what can cause mononeuritis multiplex

A

diabetes

autoimmune: SLE, RA
vascular: GPA, MPG, polyarteritis nodosa

114
Q

what hand signs can be seen in Graves disease?

A

thyroid acropatchy

115
Q

what is the pattern of rashes seen in porphyria?

A

sun exposed areas - blistering, scarring, fragility

116
Q

which type of porphyria does not exhibit skin signs?

A

acute intermittent porphyria

117
Q

which dyslipidaemia condition is tendon xanthoma associated with

A

familiar hypercholesterolaemia

118
Q

what underlying condition is eruptive xanthoma associated with?

A

hypertriglyceridaemia

119
Q

how to tell the difference between a common viral rash and a rash caused by varicella?

A

common viral rashes are caused by e.g. measles, they diffused and erythematous, macula papular.

varicella rashes are raised vesicles with umbilication and monomorphic i.e all the same size

120
Q

what is a sign of primary syphillis infection?

A

painless chancres at site of infection (mouth, anus, vagina)

121
Q

what is the secondary sign of syphilis infection

A

coppery brown rash on the palms and soles

122
Q

how does lyme disease present?

A

after tick bite, erythema migrans (red patch), that then becomes an annular rash around the bite

123
Q

what are 2 skin manifestations of IBD?

A

erythema nodosum and pyoderma grangrenosum

124
Q

describe erythema nodosum

A

erythematous painful nodules usually found on the legs caused my inflammation in fats

125
Q

what is the rash exhibited in reactive arthritis?

A

PPPP - palmar plantar pustular psoriasis. or keratoderma blenorrhagica

126
Q

what other symptoms can be found along side keratoderma blenorrahgica?

A

urethritis, conjunctivitis, arthritis

127
Q

what are the symptoms found in behcet’s syndrome

A

orogenital ulcers, pyoderma, pathergy, uveitis

128
Q

what inflammatory condition canpathergy be associated with?

A

Behcet’s disease

129
Q

what conditions are associated with dilated nail fold capillaries?

A

raynauds phenomenon
systemic sclerosis
polymyositis/dermatomyositis
mixed CTD

130
Q

what condition is associated with a sun-exposed geographical shape ring-like rash?

A

subacute lupus

131
Q

what condition is associated with beaking of the nose, small tight mouth and telangectasia?

A

systemic sclerosis

132
Q

clinical featurs of scurvy ?

A

corkscrew hairs, perifollicular haemorrhages (small red petichiae), bleeding gums

133
Q

what are lisch nodules and what are they associated with?

A

brown streaks around the iris, associated with neurofibromatosis

134
Q

are lymphocytes raised or reduced in SLE?

A

reduced

135
Q

what ENT signs can be seen in small vessel vasculitis?

A

sinusitis, collapsed nose, epistaxis

136
Q

what is the triad found in lofgren’s syndrome? and what is it?

A

erythema nodosum, bilateral perihilar lymphadenopathy, arthralgia

137
Q

which patient is more worrying, a 19 year old with raynauds or a 52 year old with raynuds?

A

the 52 year old. the younger the patient presenting with raynauds, the more likely it is to be primary raynouds with no associated conditions. but the older presentation would be more likely to be associated with other autoimmune conditions e.g. SSc or dermatomyositis

138
Q

what is the difference on examination of between reactive lymphadenopathy and malignant lymphandenopathy?

A

reactive LNs are more mobile lumps compared to malignant LNs which are more rubbery

139
Q

what is erythema migrans associated with?

A

tick bite, lyme disease

140
Q

what autoimmune condition is associated with dysphagia, dysphonia and loss of finger pulp tissue?

A

systemic sclerosis

4 CSIM2 (11 decks)

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