CT diseases Flashcards

1
Q

SLE areas it can affect in the body?

A

anywhere!

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2
Q

SLE:
F:M
which ethnicities have a higher prevalence

A
F9:1M
Asain 
African Americans
Afro-caribbean
hispanic americans
Asain indians in the UK
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3
Q

aetiology factors of SLE

A

gentic
hormonal - high oestrogen increases incidence
EBV, UV, silica dust
immunological

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4
Q

pathogenesis of SLE:

__+__ apoptosis > ___ released by necrotic cells act as ___ => __ as extended exposure to these >+ stimulated > ___

A
increased and defective
nuclear material
auto Ig
AI
B and T
auto Igs
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5
Q

why renal disease in SLE?

A

immune complexes deposited in mesangium > activate complement > leucocyte attracted and release cytokines => perpetuates inflam => necrosis and scarring

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6
Q

mucocutaneous features of SLE =

A
malar rash
photosensitive rash (devs 2-3 days after exposure)
discoid LE
subacute cutaneous lupus
painless mouth ulcers
non-scarring alopecia
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7
Q

constitutional features of SLE

A
fever 
malaise
decreased appetite
wt loss
fatigue
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8
Q

MSK features of SLE

A

non deforming polyarthritis/algia (RA distribution but no erosion)
deforming arthropathy - Jaccoud’s arthritis
erosive arthritis (rare)
myopathy
myalgia
myositis

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9
Q

serositis in SLE can cause

A

pericarditis
pleurisy
pleural/pericardial effusion

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10
Q

renal involvement in SLE =

A

proteinuria >500mg in 24hrs

red cell casts

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11
Q

neuro features of SLE

A
seizures
psychosis/depression
migraine
mononeuritis multiplex
cranial/peripheral neuropathy
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12
Q

haematological features of SLE

A

lymphadenopathy, leucopenia (susceptible to infections), haem anaem, thrombocytopenia

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13
Q

SLE is often ass with ___ syndrome => (5)

A
anti-phospholipid
arterial and venous thrombosis
recurrent miscarriage
livedo reticularis
thrombocytopenia
prolonged APTT
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14
Q

Ix for diagnosis of SLE

A

autoIgs

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15
Q

screening in SLE for organ involvement test = (8)

A
CXR
PFT
CT chest
urinalysis
renal biopsy
echocardiogram
nerve conduction studies
brain MRI
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16
Q

autoIgs in SLE (5)

A

ANA (+ve in 95% but not v specific)
anti-dsDNA (+ve in 60%, highly specific and titre correlates to disease activity)
anti-ENA (cutaneous manifestations) = anti -Ro +La
anti-Sm (in 10%, highly specific)
anti-RNP (30%)

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17
Q

autoIg highly specific for SLE and its titre correlates to disease activity =

A

anti dsDNA

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18
Q

anti phospholipid autoIgs (3)

must be ___ for diagnosis

A

anti-cardiolipin Ig
lupus anticoagulant
anti β2 glycoprotein
+ve 2x 12 wks apart

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19
Q

monitor activity of SLE by: (3)

A

clinical assessment eg. bp, FBC, biochem, urine for protein, cells and casts
anti dsDNA Ig
C3/4 levels (increase if activity decreases)

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20
Q

treatment for SLE

A
limit sun exposure
pregnancy support
NSAIDs and analgesia
hydroxychloroquine
steroids
IS
biologics
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21
Q

biologics used for SLE

A

anti-CD20 (rituximab)

anti B Igs (belimumab)

22
Q

low dose steroids <15mg/d of prednisolone is used if ++_ features of SLE

A

skin rashes
arthritis
serositis

23
Q

mod dose steroids 0.5mg/kg/d used if these features of SLE (3)

A

resistant serositis
haematological
class V glomerulonephritis

24
Q

high dose steroids 1mg/kg/d used if these features of SLE (3)

A

major organ involvement
severe/resistant haematological
diffuse glomerulonephritis

25
Q

mild SLE drugs =

A

hydroxychloroquine
topical steroids
NSAIDs

26
Q

moderate SLE drugs (no major organ involvement)

A

PO steroids
azathioprine
methotraxate

27
Q

severe SLE drugs

A

IV steroids
glycophosphamide
rituximab

28
Q

Rx of anti-phospholipid syndrome

A

lifelong anticoagulation
aspirin and heparin during pregnancy
decrease vascular risk factors

29
Q

Diagnosis of antiphospholipid syndrome is by 1 lab + 1 clinical feature :
clinical features =

A

arterial/ venous thrombosis
unexplained preg loss 10-34wks in/ 3 losses unexplained <10wks / prem <34wk due to eclampsia, severe pre eclampsia or placental insufficency

30
Q

Diagnosis of antiphospholipid syndrome is by 1 lab + 1 clinical feature :
lab =

A

anti cardiolipin Ig +/ lupus anticoagulant +/ antiβ2 glycoprotein
on two occasions at least 12 wks apart

31
Q

F:M for anti phospholipid syndrome

A

F3.5:1M

32
Q

s+s of anti-phospholid syndrome

A
superficial thrombophlebitis
livedo reticularis
moderate thrombocytopenia
migraine
transverse myelitis
Libman-Sachs endocarditis
33
Q

AI lymphocyte infiltration of exocrine glands => ___+___ = ___ syndrome

A

xerostomia
keratoconjunctivitis sicca
Sjogrens

34
Q

diagnosis of Sjogrens is dependent on

A
4/6 of:
ocular / oral sympts (daily >3months)
ocular dryness
salivary gland involved
anit Ro +/La
PLUS lymphocytic infiltrate on biopsy
35
Q

test to test ocular dryness

A

Schirmer test

36
Q

s+s of Sjogrens (11)

A
fatigue
arthralgia
Raynauds
salivary swelling
lymphadenopathy
skin and vaginal dryness
ILD
neuropathy
lymphoma (40x risk)
renal tubular acidosis
neonatal complete heart block (anti-Ro)
37
Q

primary Sjogrens age, M:F

rarely have __

A

40-60yo
F9:1M
rarely have serious complications

38
Q

Rx of Sjogrens

A
eye drops
punctal plugs
saliva replacement
pilocarpine
hydroxychloroquine
steroids and IS
CV risk factors
39
Q

systemic sclerosis 4 types =

A

localised scleroderma
limited systemic sclerosis
diffuse systemic sclerosis
systemic sclerosis sine scleroderma

40
Q

AI that causes vasculopathy (__), inflam and fibrosis - excess deposition of collagen in skin and organs =

A

Raynauds

Systemic sclerosis

41
Q

signs of localised scleroderma =

A

morphoea (skin patches/plaques)
linear scleroderma (long narrow thickening of skin)
only affects the SKIN

42
Q

s+s of limited systemic sclerosis

A

CREST
Calconosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasia
30% = pulmonary hbp

43
Q

limited systemic sclerosis is ass with ____ Igs

A

anti-centromere

44
Q

diffuse systemic sclerosis ass. with ___ Igs
early ___ involvement
__ changes within 1 yr of Raynauds
__+__ skin involvement

A

anti- Scl 70
significant organ involvement (lungs, kidneys, gut, muscle, joints, heart)
skin
truncal and acral

45
Q

treatment of systemic sclerosis

A
CCBs
prostacyclin (Iloprost)
ACEI
prednisolone
IS
bosentan
sildenafil
46
Q

Mixed CT disease (MCTD)

major features -

A
severe myositis
pulmonary involvement
Raynauds
swollen hands
sclerodactyly
anti U1 RNP > 1:10000
47
Q

minor features of Mixed CT disease (MCTD)

A
alopecia
lecuopenia
anaemia
pleuritis
pericarditis
arthritis
trigeminal neuralgia
malar rash
thrombocytopenia
mild myositis
Hx of swollen hands
48
Q

auto Igs in Sjogrens =

A

Anti Ro and La

49
Q

auto Igs in systemic sclerosis

A

anti centromere

anti Scl 70

50
Q

MCTD auto IG

A

anti RNP

51
Q

polymyositis auto Ig =

A

anti Jo1