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Connective Tissue Disease Flashcards

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1
Q

who is lupus most common in?

A

mostly women
afro-caribbeans
afro-americans
Asians uncommon in black africans

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2
Q

what are the contributing factors

A

genetic predisposition
environmental factors
immunological factors
humoral factors

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3
Q

what genetic factors affect lupus?

A

high concordance in monozygotic twins

and relatives

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4
Q

what hormonal factors can influence lupus?

A

increased incidence in people with higher oestrogen exposure - early menarche, on oestrogen containing contraceptives and HRT

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5
Q

pathogenesis of lupus?

A

defective apoptosis = dead cells aren’t cleared properly and their material which is released floats around the body for too long (acts as auto-antigens)
immune system activates T and B cells and develops antibodies against these
antibodies attack dead cell material as well as healthy cells

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6
Q

how does lupus cause renal disease?

A

deposition of immune complexes in mesangium/walls of blood vessels and activate complement which attracts leukocytes (e.g neutrophils) which release cytokines
cytokines perpetuate inflammation which cause necrosis and scarring
results in non functioning kidney

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7
Q

what drives lupus disease?

A

immune complex formation

= hypersensitivity type 3

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8
Q

is B cell function normal?

A

no

producing auto-antibodies

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9
Q

common clinical features of lupus?

A 
skin involvement
mucosal involvement
alopecia
joint involvement
serositis (pleura, pericardium involvement)
renal involvement
neurological (brain fog > seizures)
haemolytic anaemia
leukopenia (low WCC)
thrombocytopenia (low platelets)
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10
Q

what parts of FBC may be altered in lupus?

A

all of them

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11
Q

what are some common constitutional symptoms of lupus?

A 
fever
malaise
poor appetite
weight loss
fatigue
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12
Q

name some mucocutaneous features of lupus?

A 
photosensitivity - malar rash
discoid lupus erythematous
subacute cutaneous lupus
mouth ulcers
alopecia (hair on the pillow)
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13
Q

how do lupus rashes appear?

A

hours-days after sun exposure and last for weeks

sparing of fold between face and nose

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14
Q

what is discoid lupus?

A

rash with coin shaped lesions

can often be a separate disease - not systemic lupus

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15
Q

MSK features of lupus?

A

joint pain or inflammation (often in smaller joints)
muscle pain, weakness or inflammation
deforming and non-deforming arthropathy

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16
Q

what is Jaccoud’s arthritis?

A

type of deforming arthropathy
normal X ray
looks like rheumatoid deformity from outside but has normal joints
due to laxity of ligaments

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17
Q

what is serositis?

A

inflammation of the pericardium or pleura

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18
Q

what can occur in serositis?

A

pleural effusion
pericardial effusion
pericarditis
pleurisy

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19
Q

renal features of lupus?

A

tends to cause no symptoms

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20
Q

clinical signs of renal disease?

A

proteinuria (>500mg)
haematuria sometimes
red cell casts

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21
Q

what is red cell cast a sign of?

A

glomerulonephritis

- renal disease

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22
Q

next step if high protein found in urine?

A

renal biopsy

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23
Q

neurological features of lupus?

A 
depression/psychosis
migranes
seizures
cranial or peripheral neuropathy
mononeuritis multiplex (inflammation of blood supply to a long nerve causing a palsy)
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24
Q

MRI features of naeurological disease?

A

white spots

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25
haematological features of lupus?
``` lymphadenopathy leucopenia lymphopenia haemolytic anaemia thrombocytopenia often not that low so don't really cause much of a problem ```
26
what is anti-phospholipid syndrome?
causes blood clots in veins and arteries can cause recurrent miscarriage associated with lupus
27
what does anti-phospholipid syndrome look like?
mottling of skin | = livido reticularis
28
are lupus patients more pron to infection?
yes | due partly to features of disease but a lot to do with immunosuppressive treatment
29
what intrinsic factors cause susceptibility to infection in lupus?
low complement impaired cell mediated immunity defective phagocytosis poor antibody response to certain antigens
30
aims of lupus investigation?
confirm diagnosis | determine degree of organ involvement
31
how good an indicator of lupus is ANA (anti-nuclear antibodies)?
present in low levels in 20% of population present in high levels in 98% of lupus sensitive but not specific
32
when should an ANA positive result be taken seriously?
if others are present | if the patient has clinical features and symptoms
33
what antibodies are associated with lupus?
anti- dsDNA (most important) anti - Sm anti - Ro anti - RNP
34
how does anti- dsDNA present?
in 60% of lupus patients highly specific can go up and down with activity of disease can be associated with renal disease
35
how does anti-Ro present?
60% of lupus patients | associated with cutaneous problems and congenital heart block
36
what are the 2 anti ENA antibodies?
Anti Sm - neurological involvement | ANti RNP - overlap features
37
3 antibodies of anti-phospholipid syndrome?
anti-cardiolipin antibody lupus anticoagulant anti-beta 2 glycoprotein must be positive on 2 occasions 12 weeks apart for diagnosis
38
what is the most sensitive auto antibody for lupus?
anti - double stranded DNA (dsDNA) antibody
39
how do anti dsDNA levels change?
titre rises when disease is active or flaring
40
first test after lupus diagnosis?
urinalysis (must test for renal involvement first!)
41
what other tests are used in lupus?
depend on symptoms | i.e - chest involvement = CXR
42
what would indicate that someone is experiencing a flare of their lupus?
fall in complement levels as its being consumed
43
what inflammatory markers vary in lupus?
ESR | plasma viscosity
44
does ANA levels correlate to disease activity?
no
45
how can activity of SLE be monitored?
``` clinical assessment anti dsDNA C3/4 levels urine examination FBC blood biochemistry ```
46
general management of lupus?
counselling regular monitoring avoid excessive sun exposure pregnancy issues
47
SLE drug treatment?
hydroxychloroquine = MAIN NSAIDs and simple analgesia steroids (only for short periods of time) Immunosuppressives if anything more than mild
48
how do steroids work in SLE?
dose related to the severity of the disease | only used short term (few weeks) usually for flare up
49
what other drugs can be used?
biologics - anti CD20 rituximab - anti Blys (belimumab)
50
treatment for mild SLE?
HCQ topical steroids NSAIDs
51
treatment for moderate SLE?
oral steroids azathioprine methotrexate
52
treatment for severe SLE?
IV steroids cyclophosphamide rituximab belimumab
53
what goes down when anti dsDNA levels go up?
complement
54
first line tests for suspected SLE?
urinalysis anti dsDNA complement FBC
55
pain in one side of chest in lupus patients?
possible PE due to anti- phospholipid syndrome
56
PE X ray?
normal
57
lupus anticoagulant indicates what?
anti-phospholipid syndrome
58
what is cyclophosphamide?
potent immunosuppressive
59
what are connective tissue diseases?
not diseases of connective tissue spontaneous over activity of the immune system with specific auto antibodies evolve over years and lead to organ failure/death
60
what is lupus?
systemic autoimmune disease that can affect any part of the body immune system attacks body cells and tissue causing inflammation and tissue damage

MSK (66 decks)

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