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MSK > Autoimmune conditions > Flashcards

Autoimmune conditions Flashcards

1
Q

what antibodies in type 2 and 3 hypersensitivity reactions

A

IgG (type 2 also IgM)

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2
Q

what is IL-2

A

t cell growth factor produced by CD4 +T cells

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3
Q

how do T and B cells bond to antigens

A

B cells can bind to free antigens

T cells must be presented by MHC I and II

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4
Q

what is autoimmunity

A

the presence of adaptive immune responses against self tissue/cells

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5
Q

when does autoimmunity cause significant tissue/organ damage and or chronic inflammation

A

when there is high titres of autoantibodies and/or auto-reactive T cells

when there is a failure of autoimmunogical tolerance (mechanisms defective or overwhelmed)

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6
Q

what are the normal tolerance mechanisms for auto reactive lymphocytes

A

immature self reactive lymphocytes are killed as they develop in primary lymphoid tissues

activity of mature self reactive lymphocytes is suppressed by regulatory T cells in peripheral tissues

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7
Q

what does the FOXP3 gene do

A

essential for the development of CD4+ regulatory T cells

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8
Q

what happens if the FOXP3 gene is mutated

A

decreased numbers of TREGS and loss of immune regulation

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9
Q

describe four environmental factors that can initiate an autoimmune disease

A

molecular mimicry- when T and B cells with cross reactive antigen receptors are activated by non self peptides that mimic endogenous ones

intercurrent change- immune responses potential ongoing immune reactions

tissue damage- causes previosly hidden (from immune system so not deleted) self antigens to be released and exposed

superantigens- bacterial, can activate T cells non specifically which can then differentiate into effector T cells and attack tissues expressing self antigens

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10
Q

what type of hypersensitivity is SLE

A

type 3

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11
Q

what innate and adaptive immune mediators are involved in type 3 reactions

A

B cells, IgG, complement, neutrophils

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12
Q

what innate and adaptive immune mediators are involved in type 2 reactions

A

B cells, IgG/IgM, complement, NK cells, phagocytes

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13
Q

give 2 examples of type 2 reactions

A

goodpastures syndrome, rheumatic fever

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14
Q

what happens when IgG and IgM bind to antigens on cells in the body mistakenly detected as foreign

A

complement is activated
cellular lysis or phagocytosis
(type 2 reaction)

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15
Q

describe how immune complexes formed from antigens and IgG causes type three reactions

A

deposited in the tissue, initiates complement cascade, release of lysosomal enzymes from neutrophils, cell death, inflammation, vasculitis

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16
Q

what is myasthenia gravis

A

autoimmune neuromusclar disorder characterised by severe muscle weakness and progressive fatigue caused by disrupted neurotransmission at the NMJ (due to AChR antibodies)

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17
Q

what do autoreactive antibodies in MG bind to

A

post synaptic acetylcholine receptors in muscle cells

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18
Q

what age can you get MG

A

any

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19
Q

what are the clinical manifestations of MG

A

insidious onset
fatigue after exercise- resolves after rest
progressive muscle weakness
muscles of eyes, face, mouth, throat and mouth usually affected first

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20
Q

when may symptoms of MG first appear

A

during pregnancy or after some anesthetic agents

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21
Q

what antibody in MG

A

IgG anti-AChR antibodies

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22
Q

what is the final stage of MG

A

acetylecholine receptors destroyed, paraylsis, respiratory insufficiency

AChR antibodies bind complement, leading to the destruction go the muscle endplate via membrane attack complex

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23
Q

what is the treatment of MG

A

anti cholinesterase agents (increase neurotransmission)

immunosuprpessants

complement inhibitors

surgery (thymectomy)

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24
Q

what is rheumatoid arthritis characterised by

A

destruction of joint cartilage and inflammation of the synovium

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25
Q

what gene is associated with RA

A

HLA-DR4

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26
Q

what type of reaction is RA

A

type 4, infiltration of synovium by self reactive CD4+T cells (and lymphocytes, plasma cells, dendritic cells and macrophages)

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27
Q

what produces rheumatoid factor

A

B cells

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28
Q

what produces TNF alpha in RA

A

synovial macrophages

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29
Q

what do pro inflammatory cytokines do in RA

A

act on synovial fibroblasts to promote swelling of the synovial membrane and damage to soft tissues

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30
Q

what activates osteoclasts

A

RANKL

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31
Q

what activates chondrocytes to destroy cartilage

A

TNF alpha

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32
Q

who is more likely to get autoimmune conditions

A

women

33
Q

what symptoms should make you suspect an autoimmune conditions

A

arthralgia, arthritis, muscle pain/ weakness, photosensitivity, raynauds, sicca symptoms (dry eyes/mouth/throat/vagina), dysphagia, SOB, neurological symptoms, recurrent pregnancy loss

34
Q

what is the general screening test for autoimmune conditions

A

ANA

35
Q

what does a titre mean

A

higher titre (1;640) more important than lower (1;80)

36
Q

what happens if ANA is positive

A

analysed for specific antibodies

37
Q

RA:
what antibodies,
disease activity markers,
specific associations

A

rheumatoid factor, anti CCP

ESR/CRP

CCP and erosive disease, extra articular manifestations, smoking

38
Q

SLE:
what antibodies,
disease activity markers,
specific associations

A

ANA, dsDNA, anti smith, ribosomal P

low C4 (and C3), dsDNA

antiphospholipid syndrome ribosomal P

39
Q

drug induces SLE

A

anti-histone

ESR/CRP

hydralazine, cholpromazine, isoniazid, anti TNF

40
Q

dermatomyositis/ polymyolitis:
what antibodies,
disease activity markers,
specific associations

A

anti jo, anti-PM-Scl

CK

DM and malingnancy, anti-synthetase

41
Q

limited sleroderma:
what antibodies,
disease activity markers,
specific associations

A

anti-centromere

ESR/CRP

pulmonary hypertension

42
Q

diffuse scleroderma:
what antibodies,
disease activity markers,
specific associations

A

anti Scl 70, anti RNA polymerase, antifibrillarin

ESR/CRP

interstitial lung disease, GI, cardio, renal

43
Q

scleroderma renal crisis

A

anti RNA polymerase III

creatinine, BP

renal crisis (hypertension, acute rise in creatone, papillodema)

44
Q

sjogrens syndrome:
what antibodies,
disease activity markers,
specific associations

A

anti-SSA (Ro), Anti-AAB (Ia), ANA, RF

ESR/CRP

sicca symptoms, risk lymphoma

45
Q

congential heart:
what antibodies,
disease activity markers

A

Anti-SSA (ro)

heart rate

46
Q

mixed connective tissue disease:
what antibodies,
disease activity markers,
specific associations

A

anti-U1RNP (daDNA must be negative)

ESR/CRP

puffy hands, overlap with other CTD

47
Q

Microscopic polyangiitis:
what antibodies,
disease activity markers,
specific associations

A

P-ANCA (MPO+ve)

ESR/CRP

pulmonary, renal (puci immune focal segmental necrotising glomerulonephritis), neuro

48
Q

churg strauss:
what antibodies,
disease activity markers,
specific associations

A

p-ANCA (MPO=ve) and eosinophilia

ESR/CRP

asthma, eosinophilla, atopy

49
Q

granulomatosis polyangiitiis:
what antibodies,
disease activity markers,
specific associations

A

C-ANCA (PR3 +ve)

ESR/CRP

granulomas- renal, pulmonary

50
Q

what causes raynauds

A

vasospasm of artery= vascular occlusion= digital ischemia

cyanosis as capillaries and venules are filled with deoxygenated blood

rubor as vasospasm releases and capillaries still dilated filled with oxygenated blood

51
Q

when does raynauds suggest underlying autoimmune disease

A

when in secondary, older age group

52
Q

what is the treatment for raynauds

A

keep warm, vasodilators (CCN, PDE5 inhibitors)

53
Q

what do 90% of systemic sclerosis patients present with

A

raynauds

54
Q

what are the two types of systemic sclerosis

A

diffuse cutaneous systemic sclerosis (dsCC0

limited cutaneous systemic sclerosis (lsCC)

55
Q

what is systemic sclerosis (scleroderma)

A

autoimmune disease in which there is increased fibroblast activity resulting in abnormal growth of connect tissue= vascular damage and fibrosis

56
Q

where does fibrosis occur

A

skin, GI tract, heart, lungs and other internal organs

57
Q

what cell type leads to vasculopathy and tissue fibrosis in systemic sclerosis

A

myofibroblasts (formed from fibroblasts, firbocytes and endothelial cells)

58
Q

describe diffuse cutaneous SSc

A

skin involvement on extremities above AND below elbows and knees (plus face and trunk) (everywhere)

59
Q

describe limited cutaneous SSc

A

skin involvement on extremities and ONLY below elbows and knees (plus face)

60
Q

what are the limited (CREST) symptoms of systemic sclerosis (scleroderma)

A

CREST

Calcinosis (calcium deposits in the skin)

Raynauds phenomenon- spasm of blood vessels in response to cold or stress

Esophageal dysfunction (acid reflux, decreased motility of esophagus)

Sclerodactyly- thickening and tightening of the skin on the fingers and hands

Telangiectasias- dilation of capillaries causing red marks on surface of the skin

61
Q

what are pulmonary complications of systemic sclerosis

A

pulmonary fibrosis, pulmonary hypertension

62
Q

what is scleroderma renal crisis

A

fibrinoid necrosis in the preglomerulase afferent arterioles- the normal muscle layer of the media is replaced with fibrinoid material

63
Q

what is the treatment for digital ulcers in raynauds

A

prostacyclin analogues, botox injections, endothelin receptor antagonists

64
Q

what is the management for systemic sclerosis

A

yearly echo and PFTs

treat raynauds and digital ulcers

treat reflux woth PPi +/- rantidine +/-gaviscon

pulmonary fibrosis- immunosuppession

PHT- [rostacyclin analogues, endothelin receptor anatagonists, PDE5 inhibitors

BP- ACE INHIBITORS

65
Q

what is sjogrens syndrome

A

characterised by lymphocytic infiltration of exocrine glands

66
Q

what are the symptoms of sjorgens

A

keratoconjuctivitis sicca

  • gritty eyes
  • thirsty
  • dry mouth
  • vaginal dryness
  • blepharitis
  • salivary gland inflammation
  • tooth decay
  • lymphoma
  • dry cough
  • multisystem involvement
67
Q

describe the antibodies, blood marjers and diagnostic markers of sjorgens

A

usually ANA positive
anti ro and anti La-more specific than sensitive
if antibodies negative salivary gland ultrasound and biopsy

usually high ESR/ PV

raised IgG

cytopaenias

68
Q

what is the treatment of sjogrens syndrome

A

artificial tears and salivary supplements, vaginal lubricants

good dental hygiene- strong fluoride toothpaste

hydroxychloroquine for fatigue and arthralgia

immunosuppressants for major organ involvement

69
Q

what is undifferentiated CTD

A

components of: MCTD, SLE, SSc, DM/PM, sjogrens

70
Q

what is mixed connective tissue disease

A

components of SLE, SSc, RA and PM

71
Q

what is polymyositis

A

chronic inflammation of the muscles

72
Q

what is dermatomyositis

A

inflammatory disease causing skin rash, muscle weakness, inflammatory myopathy/ inflamed muscles

73
Q

what is antiphospholipid syndrome

A

autoimmune disorder characterised by arterial and venous thrombosis, thrombocytopaenia, miscarriages/ pregnancy complications and raised levels of antiphospholipid (aPL) antibodies

74
Q

what biochemical markers are seen in antiphopholipid antibody syndrome

A

Presence of antiphospholipid antibodies on 2 occasions 12 weeks apart-
Anti cardiolipin antibody(IgM and IgG)
Lupus anticoagulant.
Beta 2 glycoprotein.

75
Q

what is thrombocytopaenia

A

too few platelets

76
Q

what is the difference between primary and secondary antiphopholipid antibody syndrome

A

primary- no other underlying autoimmune disease

secondary- seen in 20% of lupus patients

77
Q

what is the management for antiphopholipid antibody syndrome

A

lifelong anticoagulants if thombosis

pre pregnancy- LMWH and aspirin into post partum period

if major organ invlovement- immunosuprpession. if not- Hydroxychloroquine, symptomatic treatment

78
Q

how do you asses the severity of antiphospholipid antibody syndrome

A

urinalysis, CXR, pulmonary function, ECHO

MSK (71 decks)

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