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MSK > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

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
Q

haematological features of lupus?

A 
lymphadenopathy
leucopenia
lymphopenia
haemolytic anaemia
thrombocytopenia
often not that low so don't really cause much of a problem
26
Q

what is anti-phospholipid syndrome?

A

causes blood clots in veins and arteries
can cause recurrent miscarriage
associated with lupus

27
Q

what does anti-phospholipid syndrome look like?

A

mottling of skin

= livido reticularis

28
Q

are lupus patients more pron to infection?

A

yes

due partly to features of disease but a lot to do with immunosuppressive treatment

29
Q

what intrinsic factors cause susceptibility to infection in lupus?

A

low complement
impaired cell mediated immunity
defective phagocytosis
poor antibody response to certain antigens

30
Q

aims of lupus investigation?

A

confirm diagnosis

determine degree of organ involvement

31
Q

how good an indicator of lupus is ANA (anti-nuclear antibodies)?

A

present in low levels in 20% of population
present in high levels in 98% of lupus
sensitive but not specific

32
Q

when should an ANA positive result be taken seriously?

A

if others are present

if the patient has clinical features and symptoms

33
Q

what antibodies are associated with lupus?

A

anti- dsDNA (most important)
anti - Sm
anti - Ro
anti - RNP

34
Q

how does anti- dsDNA present?

A

in 60% of lupus patients
highly specific
can go up and down with activity of disease
can be associated with renal disease

35
Q

how does anti-Ro present?

A

60% of lupus patients

associated with cutaneous problems and congenital heart block

36
Q

what are the 2 anti ENA antibodies?

A

Anti Sm - neurological involvement

ANti RNP - overlap features

37
Q

3 antibodies of anti-phospholipid syndrome?

A

anti-cardiolipin antibody
lupus anticoagulant
anti-beta 2 glycoprotein
must be positive on 2 occasions 12 weeks apart for diagnosis

38
Q

what is the most sensitive auto antibody for lupus?

A

anti - double stranded DNA (dsDNA) antibody

39
Q

how do anti dsDNA levels change?

A

titre rises when disease is active or flaring

40
Q

first test after lupus diagnosis?

A

urinalysis (must test for renal involvement first!)

41
Q

what other tests are used in lupus?

A

depend on symptoms

i.e - chest involvement = CXR

42
Q

what would indicate that someone is experiencing a flare of their lupus?

A

fall in complement levels as its being consumed

43
Q

what inflammatory markers vary in lupus?

A

ESR

plasma viscosity

44
Q

does ANA levels correlate to disease activity?

A

no

45
Q

how can activity of SLE be monitored?

A 
clinical assessment
anti dsDNA
C3/4 levels
urine examination
FBC
blood biochemistry
46
Q

general management of lupus?

A

counselling
regular monitoring
avoid excessive sun exposure
pregnancy issues

47
Q

SLE drug treatment?

A

hydroxychloroquine = MAIN
NSAIDs and simple analgesia
steroids (only for short periods of time)
Immunosuppressives if anything more than mild

48
Q

how do steroids work in SLE?

A

dose related to the severity of the disease

only used short term (few weeks) usually for flare up

49
Q

what other drugs can be used?

A

biologics

  • anti CD20 rituximab
  • anti Blys (belimumab)
50
Q

treatment for mild SLE?

A

HCQ
topical steroids
NSAIDs

51
Q

treatment for moderate SLE?

A

oral steroids
azathioprine
methotrexate

52
Q

treatment for severe SLE?

A

IV steroids
cyclophosphamide
rituximab
belimumab

53
Q

what goes down when anti dsDNA levels go up?

A

complement

54
Q

first line tests for suspected SLE?

A

urinalysis
anti dsDNA
complement
FBC

55
Q

pain in one side of chest in lupus patients?

A

possible PE due to anti- phospholipid syndrome

56
Q

PE X ray?

A

normal

57
Q

lupus anticoagulant indicates what?

A

anti-phospholipid syndrome

58
Q

what is cyclophosphamide?

A

potent immunosuppressive

59
Q

what are connective tissue diseases?

A

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
Q

what is lupus?

A

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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