Connective Tissue Disease Part 2 Flashcards

1
Q

what generally causes autoimmune disease?

A

breakdown of immunological tolerance to autoreactive immune cells
genetic, environmental and infectious pre-disposing mechanisms

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2
Q

what type of immune response is typically involved in autoimmunity?

A

adaptive

  • cell mediated
  • humoral
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3
Q

can autoimmune conditions coexist?

A

yes
overlap syndrome
- has features of more than 1 disease but cant be clearly defined as one type

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4
Q

who is autoimmune disease more common in?

A

women

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5
Q

2 types of autoimmune disease?

A

primary

secondary (due to another condition)

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6
Q

what are autoimmune diseases associated with?

A

auto antibody formation

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7
Q

when do you suspect autoimmune disease?

A
young women of joint and muscle problems
photosensitivity/malar rash
raynauds
sicca symptoms (dryness in mucosal areas) - in absence of other causes
dysphagia, SOB
neurological symptoms
pregnancy problems
unprovoked thrombosis
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8
Q

how are antibodies used in diagnosis of autoimmune disease?

A

more specific then sensitive
ANA = general test for all autoimmune test
- higher titre = more significant
if ANA positive, sample analysed for other antibodies

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9
Q

what is raynauds?

A

in 90% of systemic sclerosis

vasospasm causing white fingers in cold

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10
Q

types of raynauds?

A

primary (benign, gets better in summer)

secondary = with autoimmune, doesn’t improve

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11
Q

3 phases of raynauds?

A
1 = ischaemia (become pure white)
2 = cyanosis (blueish)
3 = rubor = (redness as blood returns)
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12
Q

what indicates secondary raynauds?

A

ulcers or gangrene in hands

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13
Q

how is raynauds treated?

A
keep warm
vasodilation - CCBs, PDE5 inhibitors
prostacycline inhibitors for finger ulcers
botox injections
endothelin receptor antagonists
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14
Q

systemic sclerosis vs scleroderma?

A

scleroderma = thickening of skin (symptom of systemic sclerosis)

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15
Q

what are the 2 main features of systemic sclerosis?

A

scleroderma

raynauds

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16
Q

what are the 2 types of systemic sclerosis?

A

diffuse

limited

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17
Q

what is diffuse systemic sclerosis?

A

skin on extremities above and below elbows and knees plus face and trunk

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18
Q

what is limited systemic sclerosis?

A

skin on extremities only below elbows and knees plus face

19
Q

pathogenesis of systemic sclerosis?

A

genetic predisposition + etiologic agent > endothelial cell and vascular alterations > chronic inflammation > fibroblasts, fibrocytes, endothelial cells activated > myofibroblasts > vasculopathy and tissue fibrosis

20
Q

what causes skin thickening in systemic sclerosis?

A

fibrosis

21
Q

what is CREST?

A

symptoms of systemic sclerosis
C = calcinosis
R = raynauds
E = esophageal dysmotility
S = sclerodactyly (thickening/tightening of hand skin)
T = telangiectasias (dilated blood vessels)

22
Q

why is lung fibrosis common in SS?

A

fibroblasts are activated

23
Q

how can SS cause pulmonary hypertension?

A

causes pulmonary fibrosis which restricts blood flow in pulmonary arteries

24
Q

what does lupus cause in the kidney?

A

glomerulonephritis (inflammation)

25
Q

what does SS cause in the kidneys?

A

fibrosis increases pressure

no inflammation

26
Q

2 main antibodies of SS? how do they appear?

A

Anticentromere
antitopoisomerase
if you have one you don’t have the other

27
Q

does everyone with SS have autoantibodies present?

A

no

28
Q

how is SS managed?

A

treat raynauds and hand ulcers
treat reflux
immunosuppression for pulmonary fibrosis and skin involvement
tight control of BP - ACE inhibitors
pulmonary hypertension - prostacyclin analogues, endothelin receptor antagonists, PDE5 inhibitors

29
Q

what is sjogrens syndrome?

A

multisystem autoimmune disease more common in men

lymphocytic infiltration of exocrine glands

30
Q

main symptom of Sjogrens disease?

A

Main symptom = sicca

ask about dryness in eyes, mouth and vagina

31
Q

what cancer are people with autoimmune diseases more at risk of?

A

lymphoma

32
Q

other symptoms of sjogrens?

A
blepharitis
salivary gland inflammation
tooth decay
lymphoma
dry cough
multisystem involvement
33
Q

sjogrens specific antibodies?

A

anti Ro

anti La

34
Q

how is sjogrens diagnosed?

A
usually ANA positive
anti La and anti Ro
high ESR/plasma viscosity
raised IgG
cytopenia
if antibodies are negative - salivary gland biopsy
35
Q

how is sjogrens treated?

A
artificial tears
salivary supplements
vaginal lubricants
strong fluoride toothpaste
Hydroxyquinone for fatigue and arthralgia
immunosuppression if organ involvement
36
Q

what is antiphospholipid antibody syndrome?

A

antiphospholipid antibodies cause the blood to become more coagulable

  • anticardiolipin antibody
  • lupus anticoagulant
  • beta 2 glycoprotein
37
Q

what must be present for antiphospholipid SYNDROME?

A

antibodies present on 2 occasions 12 weeks apart
pregnancy problems early or late loss)
thrombosis

38
Q

when do you suspect antiphospholipid syndrome?

A

young person with unprovoked thrombosis

several pregnancy losses

39
Q

primary vs secondary antiphospholipid syndrome?

A
primary = no underlying cause
secondary = often due to lupus
40
Q

how is antiphospholipid syndrome treated?

A

lifelong anticoagulation if thrombosis

LMWH and aspirin pre and post pregnancy

41
Q

how does Antiphosphlipid syndrome look?

A

mottling on legs

= levido reticularis

42
Q

why is anti Ro dangerous?

A

can cross the placenta into the foetus and cause congenital heart block

43
Q

when is immunosuppression used in autoimmune disease?

A

if there is major organ involvement

if not - HCQ involvement