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MSK > R: Autoimmune Disease > Flashcards

R: Autoimmune Disease Flashcards

1
Q

AI disease of joints can be divided into…

A

NMJ transmission disease, connective tissue disease and muscle disease

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2
Q

the main disease in NMJ transmission disease is…

A

myasthenia gravis

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3
Q

what is MG?

A

AI disorder of NMJ transmission

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4
Q

epi of MG?

A

4/100,000, W, 30s

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5
Q

which type of hypersensitivity reaction is MG?

A

T2

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6
Q

pathophysiology of MG?

A

auto IgG bind to Ach receptors on muscle cells which blocks ligand binding leading to defects in nerve impulse transmission at skeletal muscle NMJs

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7
Q

aetiology of MG?

A

thyme hyperplasia (70% of MG pt have this), thymic tumour, some due to HLA association/ other AI disease

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8
Q

s/s of MG?

A

severe muscular weakness, progressive fatigue

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9
Q

what is a common early and late sign of MG?

A

early: ocular myasthenia
late: difficulty with mastication

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10
Q

which 2 antibodies would be seen in a patient with MG?

A

anti AchR IgGs

anti MuSK abs

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11
Q

which study may be useful in MG?

A

nerve conduction studies (EGM)

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12
Q

mx of MG?

A

acute: O2, plasmapheresis, IV IgGs, high-dose corticosteroids
chronic: inc neurotransmission (anti-cholinesterase agents), surgery (thyroidectomy), immunosuppression

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13
Q

what are the 5 connective tissue diseases?

A

systemic lupus erythematosus (SLE), sjogren’s syndrome, systemic sclerosis, antiphospholipid syndrome, mixed connective tissue disease

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14
Q

what is SLE?

A

chronic autoimmune condition that attacks cells and tissues resulting in inflammation and tissue damage

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15
Q

epi of SLE?

A

F (9:1), women of childbearing age, afro-carribean/ hispanic/ asian
20/100,000

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16
Q

types of SLE?

A

Mild: rash, no organ involvement
Moderate: arthritis, rash
severe: major organ involvement- nephritis, pneumonitis etc

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17
Q

aetiology of SLE?

A

genetic + environmental (smoking, silica exposure)

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18
Q

antibodies for SLE?

A

anti-nuclear antibody, dsDNA, anti-sm, anti-Ro, anti-RNP

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19
Q

pathogenesis of SLE?

A

inc apoptosis > cells release necrotic material that act as auto-antigens> B& T cells stimulated so produce antibodies > complexes form and deposited in basement membrane + activation of inflammatory cells = necrosis/ scarring

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20
Q

s/s of SLE?

A

fever/ fatigue

  • skin: malar rash, cutaneous lupus/ discoid lupus, ulcers
  • rheumatological: arthritis, synovitis >2jts
  • neuro: delirium, psychosis, seizure
  • serositis: effusions (pleural/ pericardial)
  • haematological: leukopenia, thrombocytopenia
    renal: nephritis
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21
Q

ix for SLE?

A

serology: ANA, dsDNA (highly specific), anti-sm (highly specific), anti-Ro
bloods: complement proteins (C3/C4)
urinalysis
imaging for organ involvement

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22
Q

SLE score for monitoring/prescribing is called…

A

SLEDAI score

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23
Q

SLE mx?

A

sun protection, minimise steroid use
mild= HCQ
moderate: MTX
severe: biologics/ immunosuppression e.g. rituximab

24
Q

SLE prognosis?

A

10 year survival is 90%

25
Q

what is Sjogren’s syndrome?

A

characterised by lymphocytic infiltrates in exocrine organs causing excessive dryness

26
Q

s/s of sjogren’s?

A

dry everything !
- dry eyes, mouth, vagina
bilateral parotid gland enlargement, joint pain, fatigue

27
Q

types of sjogren’s

A

1y- absence of other pathology

2y- along with RA or other AI disease

28
Q

antibodies associated with sjogren’s?

A

ANA, anti-Ro, anti La

29
Q

ix for sjogren’s?

A

serology- ANA, anti-Ro, anti La, inc IgG, inc plasma viscosity
tests
lip gland biopsy

30
Q

what are the 2 tests done to investigate sjogren’s?

A

schrimmer tear test, rose Bengal staining

31
Q

mx for Sjogren’s?

A

saliva replacement/tear replacement, HCQ for fatigue

32
Q

what is systemic sclerosis?

A

multisystem AI disease characterised by vasculopathy, fibrosis, atrophy of skin

33
Q

types of systemic sclerosis/ scleroderma?

A

limited cutaneous scleroderma*, diffuse cutaneous

34
Q

antibodies associated with scleroderma?

A

ANA, anti-centromere, anti-Scl-70

35
Q

s/s of scleroderma?

A

raynaud’s, skin thickening/ calcinosis

  • facial: small mouth with puckering, beaked nose, tight skin, telangiectasia
  • GI: dysphagia, GORD, GAVE, malabsorption
  • cardio/resp: ILDs, pulmonary HT
  • renal: scleroderma renal crisis
  • vasculopathy: digital ischaemia, sclerodactyl
36
Q

what is the process of Raynaud’s…

A
  1. blanching
  2. acrocyanosis
  3. reactive hyperaemia
37
Q

mx for Raynauds

A

Ca channel blockers (nifedipine), ARBs/nitrates PDE-5 inhibitors

38
Q

ix of scleroderma?

A

serology: ANA, anti-centromere, anti-poisimerase

organ screening= important!

39
Q

mx of scleroderma?

A

treat each issue, stem cell transplant

40
Q

what is antiphospholipid syndrome?

A

disorder of antiphospholipid ABs so blood prone to clotting which manifests clinically as recurrent thrombosis

41
Q

antibodies associated with antiphospholipid syndrome?

A

anti-cardiolipin, lupus anti-coagulant, anti-beta 2 glycoprotein

42
Q

s/s antiphospholipid syndrome?

A

miscarriage, headaches (migraines), levido articulares

MI/ stroke may be 1st time presentation

43
Q

which type of endocarditis is seen with people with APS?

A

Libman-Stacks endocarditis

44
Q

mx of APS?

A

warfarin, LMWH in pregnant women

45
Q

what is CAPS?

A

a complication of APS- catastrophic APS is multi organ infarction that occurs over days to weeks

46
Q

what is the main antibody associated to mixed connective tissue disease?

A

anti-RNP

47
Q

Polymyositis: _____ muscle ache and ______
polymyalgia rheumatica: Proximal _____ _______ and pain
myopathy: weakness but no ____

A

polymyositis: proximal muscle ache and weakness
polymyalgia rheumatica: proximal morning stiffness and pain
myopathy: weakness but no pain

48
Q

what is polymyositis/ dermatomyositis?

A

inflammation of striated muscles causing symmetrical proximal muscle weakness

49
Q

epi of polymyositis/ dermatomyositis?

A

females (2:1), rare, 45-60yo, inc risk of malignancy

50
Q

pathophysiology of polymyositis/ dermatomyositis?

A

t-cell mediated cytotoxic process directed against muscle antigens

51
Q

antibodies associated with polymyositis/ dermatomyositis?

A

anti-Jo-1, anti-SRP, ANA

52
Q

difference between polymyositis/ dermatomyositis?

A

polymyositis: just proximal muscle weakness
dermatomyositis: muscle weakness and skin rash

53
Q

what is dermatomyositis like?

A

V-shaped rash on chest, heliotrope rash

  • eyes: may have peri-orbital oedema
  • fingers: Gottron’s papules (purple patches on knuckles)
  • shawl sign
54
Q

ix for polymyositis/ dermatomyositis

A

exam: test power, isotonic testing
bloods: muscle enzymes- CK, inflammatory markers, antibodies
EMG
MRI
Muscle biopsy

55
Q

what are 2 signs that would be seen on muscle biopsy of polymyositis?

A

vascular inflammation and muscle necrosis

56
Q

tx for polymyositis/dermatomyositis

A

corticosteroids- prednisolone, immunosuppression- MTX

57
Q

T/F: malignancy is more common with dermatomyositis

A

true: breast, ovarian, stomach, lung, bladder, colon

MSK (26 decks)

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