Week 2 - SLE Flashcards

1
Q

what are the main connective tissue diseases?

A

SLE (lupus)
sjogrens syndrome
systemic sclerosis
mixed connective tissue disease (MCTD)

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2
Q

what is SLE?

A

chronic autoimmune disease affecting any organ system in the body but mainly the skin, joints, kidneys, blood cells and nervous system

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3
Q

how is lupus diagnosed?

A

4 or more criteria from the SLICC classification guide
(at least 1 from both clinical and immunological criteria)
OR
biopsy proven lupus nephritis with ANA or anti DNA antibody present

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4
Q

what is the pathogenesis of SLE?

A

defective apoptosis means more cell death and defective clearance of dead cell debris
cell debris acts as antigens after floating around the body for so long
Immune system forms antibodies against this material
the persistence of these antigens allows large immune complexes to form which can become lodged in small vessels or basement membranes of skin and kidneys
this activates complement and triggers inflammation which can lead to fibrosis, necrosis etc

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5
Q

describe the epidemiology of SLE

A

more common in women (child bearing age)
common in black people in UK and US but not reported in African black people
Genetic factor - often in monozygotic twins

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6
Q

through what systems can SLE present?

A
constitutional
MSK
mucocutaneous
renal
respiratory
haematological
cardiovascular
GI
neuropsychiatric
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7
Q

constitutional symptoms?

A

fever
weight loss
fatigue

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8
Q

MSK symptoms?

A

arthralgia
myalgia
inflammatory arthritis
avascular necrosis

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9
Q

mucocutaneous symptoms?

A
photosensitivity - malar rash
discoid lupus
subacute cutaneous lupus
oral/nasal ulceration
raynauds
alopecia
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10
Q

renal symptoms?

A

lupus nephritis (glomerulonephritis)

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11
Q

resp symptoms?

A
pleurisy
PE
pleural effusion
pneumonitis
pulmonary hypertension
ILD
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12
Q

cardio symptoms?

A

pericarditis
pericardial effusion
elevated ischaemic heart disease

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13
Q

haematological symptoms?

A

leukopenia
lymphopenia
thrombocytopenia
anaemia

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14
Q

neuropsychiatric symptoms?

A

seizures
depression/psychosis
headache

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15
Q

GI symptoms?

A

rare
hepatitis
pancreatitis

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16
Q

how is SLE investigated?

A
urinalysis - protein and blood indicate renal involvement
auto antibodies
FBC - anaemia etc
c3/c4 - low levels
imaging for organ involvement
17
Q

how is SLE managed?

A

always give hydroxychloroquine
mild = add steroids and NSAIDs
organ involvement = add immunosuppression and corticosteroids (short term)
severe organ involvement = IV steroids and cyclophosphamide
unresponsive = IV immunoglobulin and rituximab

18
Q

what are the SLE autoantibodies?

A

ANA - general for all connective tissue disease
anti dsDNA = most important
anti Ro, anti Ra, anti RNP
anti Sm

19
Q

how is SLE monitored?

A

regularly check anti dsDNA and complement (dsDNA can indicate a flare)
check urine for blood or protein
monitor other risk factors (cardio etc)