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MedEd + PathSoc + Laz's Qs + Romesh Path > Immuno 11: Autoinflammatory and AI disease 2 > Flashcards

Immuno 11: Autoinflammatory and AI disease 2 Flashcards

1
Q 
Name the autoantigen in the following diseases:
Goodpastures disease
Pemphigus vulgaris
Graves
Myasthenia gravis
A

Goodpasture’s disease
Non-collagenous domain of basement membrane collagen IV

Pemphigus vulgaris
Epidermal cadherin

Graves’ disease
TSH receptor

Myasthenia gravis
Nicotinic acetylcholine receptor

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2
Q

What is the autoantigen in:
SLE
Rheumatoid arthritis

A

SLE:
DNA
Histones
RNP

Rheumatoid arthritis:
Fc portion of IgG

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3
Q

Describe the pathophysiology of Graves’ disease

A

Excessive production of thyroid hormones mediated by IgG antibodies that stimulate the TSH receptor

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4
Q

What type of reaction is seen in T1DM + what antibodies?

A

T1DM - type II and IV

Anti-GAD (glutamic acid decarboxylase) = MOST IMPORTANT
Anti-islet cell
Anti-insulin
Anti-IA2

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5
Q

What type of reaction is seen in pernicious anaemia + what antibodies?

A

Pernicious anaemia – type II

Anti-gastric parietal cell – most sensitive
Anti-intrinsic factor – most specific

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6
Q

What type of reaction is seen in Myasthenia gravis + what antibodies?

A

Myasthenia gravis – type II

Anti-nAChR (MAIN)
Anti-MuSK (muscle-specific kinase )
Anti-VGCC (Voltage gated calcium channel) – Lambert-Eaton

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7
Q

What type of reaction is seen in Goodpastures disease + what antibodies?

A

Goodpasture’s syndrome – type II

Anti-basement membrane type IV collagen

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8
Q

List some genetic polymorphisms that predispose to rheumatoid arthritis.

A

PAD 2 and PAD 4 polymorphisms - MOST IMPORTANT

HLA DR1
HLA DR4
PTPN22
Polymorphisms affecting TNF, IL1, IL6 and IL10

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9
Q

Name and describe the antibodies that are often detected in the diagnosis of rheumatoid arthritis

A

Anti-cyclic citrullinated peptide antibodies – bind to peptides where arginine has been converted to citrulline, 95% specific, 60-70% sensitive

Rheumatoid factor – IgM antibody directed against Fc region of human IgG
NOTE: there are IgA and IgG variants of RF

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10
Q

B cell involvement in the pathophysiology of rheumatoid arthritis

A

Type II – antibodies bind to citrullinated peptides leading to activation of macrophages, NK cells and complement

Type III – immune complexes form and get deposited leading to complement activation

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11
Q

T cell involvement in the pathophysiology of rheumatoid arthritis

A

CD4 activation -> IFNg and IL17 production -> fibroblast and macrophage activation -> MMPs, IL-1, TNFa

CD8 infiltrate synovium -> inflammation -> pannus formation -> invades cartilage and bone

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12
Q

What Gell and Coombs classification is myasthenia gravis?

Type I
Type II
Type III
Type IV

A

Type II – anti-nAChR antibodies

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13
Q

What are antinuclear antibodies and how are they tested?

A

Group of antibodies against nuclear proteins

Tested by staining Hep-2 (human epidermoid cancer line) cells

NOTE: these are very common and are often present in healthy individuals

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14
Q

What are the two different types of antinuclear antibodies + what conditions are they seen in?

A

Anti-dsDNA (looks homogenous) - V. specifc for SLE

Anti-ENA (looks speckled) - there are different types:

  • Ro, La, Sm, RNP – SLE, Sjogrens (Ro and La)
  • Centromere – limited cutaneous systemic sclerosis
  • Scl70 – diffuse cutaneous systemic sclerosis
  • Jo1 – dermatomyositis
  • SRP – polymyositis
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15
Q

Other than dsDNA, which other quantifiable component can be measured as a surrogate marker for disease activity in SLE? what to use in sle flare ups?

A

C3 and C4 – C4 will decrease before C3

Classical pathway affected

nb in flares ESR is better than CRP

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16
Q

What is the triad of antiphospholipid syndrome?

A

Recurrent venous or arterial thrombosis
Recurrent miscarriage
Thrombocytopaenia

17
Q

What antibodies are present in antiphospholipid sydrome?

A

Anti-cardiolipin antibody – immunoglobulins directed against phospholipids and β2 glycoprotein-1

Lupus anticoagulant – prolongation of phospholipid-dependent coagulation tests (aPTT)

NOTE: cannot be assessed if the patient is on anticoagulant therapy
NOTE: both tests should be performed as 40% of patients have disconcordant antibodies

18
Q

What test maybe be falsely raised in antiphospholipid syndrome?

A

APTT

19
Q

What is the mechanism of systemic sclerosis?

A

Systemic sclerosis aka scleroderma:

Th2 and Th17 mediated inflammation

Cytokines activate fibroblasts -> fibrosis
Endothelial cell activation -> microvascular disease

20
Q

What are the two different types of systemic sclerosis?

A

Limited cutaneous systemic sclerosis (CREST Syndrome)

Diffuse cutaneous systemic sclerosis

21
Q

What antibody for:

Limited cutaneous systemic sclerosis

A

Anti-centromere antibodies

Anti-Scl70 (topoisomerase) antibodies

22
Q

What are the features of limited cutaneous systemic sclerosis

A

CREST:

Skin involvement of hands, forearms, feet and face

Calcinosis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
(can also get pulmonary hypertension)
23
Q

Features of diffuse cutaneous systemic sclerosis?

A

More extensive skin involvement

Gastrointestinal disease

Interstitial pulmonary disease/fibrosis

Scleroderma renal crisis – endothelial cells proliferate leading to a small lumen and poor renal blood supply –> severe hypertension (like renal artery stenosis)

24
Q

Which pts are likley to have the following ANA antibodies raised:
Anti-Jo1
Anti- Mi2
Anti-SRP

A

Anti-Jo1 – dermatomyositis, associated with lung involvement

Anti-Mi2 – more commonly found in dermatomyositis (but can be found in polymyositis)

Anti-SRP – polymyositis

25
Q

Features of dermatomyositis v polymyositis?

A

Dermatomyositis – perivascular CD4+ T cell and B cells are seen, this can cause an immune complex-mediated vasculitis (type III response)

Polymyositis – CD8+ T cells surround HLA Class I expressing myofibres, CD8+ T cells kill these myofibres via granzyme/perforin (type IV response)

26
Q

Features of Polymyositis?

A

Within the muscle, you will see CD8 T cells surrounding myofibres that express HLA class I

CD8 T cells kill myofibres via perforin/granzyme (type IV)

27
Q

Which classification system is used for systemic vasculitides?

A

Chapel Hill

28
Q

What are ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitidies?

A

Small vessel vasculitidies

29
Q

How does ANCA cause vasculitis?

A

Anti-neutrophil cytoplasmic antibody – antigens located in granules within neutrophil cytoplasm

Inflammation can lead to expression of these antigens on neutrophil cell surface – antibody binding can activate neutrophils (type II hypersensitivity)

Neutrophils then interact with endothelial cells –> vasculitis

30
Q

Which small vessel vasculitides are associated with ANCA?

A 
Microscopic polyangiitis (pANCA)
Churg-Strauss syndrome (pANCA)

Granulomatosis with polyangiitis (cANCA) - aka wegners disease

31
Q

What condition shows:
Glomeruloephritis
Epitaxis
Necrosis of nasal cartilage -> saddle nose

A

Granulomatosis with polyangiitis (cANCA) - aka wegners disease

32
Q

Describe the key difference between cANCA and pANCA

A

cANCA - CYTO
Cytoplasmic fluorescence
Associated with antibodies against proteinase 3
Occurs in > 90% of Wegener’s patients with renal involvement

pANCA - PERINUC
Perinuclear staining pattern
Associated with antibodies to myeloperoxidase
Less sensitive and specific than cANCA
Associated with MPA and Churg-Strauss syndrome

33
Q

Link the following antibodies to the disease they are most associated with.

Anti-IgG antibody
Anti-Sm
cANCA
pANCA
Anti-centromere
A

Anti-IgG antibody - Rheumatoid arthritis

Anti-Sm - form of ANA associated w SLE

cANCA - GPA

pANCA - MPA

Anti-centromere - Limited cutaneous systemic sclerosis

34
Q

A patient presents with lesions over their knuckles and shortness of breath. He is slow to take a seat in your clinic. Which of the follow antibodies is most likely to be positive in this patient?

Anti-Jo1
Anti-dsDNA
Anti-Ro
Anti-Scl70

A

Anti-Jo1 – associated with Mechanic’s hands and pulmonary involvement (Dermatomyositis)

35
Q

Which of the following antibodies is most sensitive for rheumatoid arthritis?

Rheumatoid factor
Anti-SRP
Anti-CCP
Anti-RNP

A

Anti-CCP – more sensitive for RA than rheumatoid factor

36
Q

What are the following associated w/ antibodies against?

cANCA

pANCA

A

cANCA = proteinase 3

pANCA = myeloperoxidase

37
Q

What ANAs are found in the following diseases:

SLE
Sjorgens
Limited Cutaneous Systemic Sclerosis
Diffuse Cutaneous Systemic Sclerosis
Dermatomyositis
Polymyositis
A

SLE = Anti-dsDNA (MAIN), anti-Ro, anti-La, anti-RNP, anti-RNP

Sjorgens = Anti-Ro and Anti-La

Limited Cutaneous Systemic Sclerosis = Anti-centromere

Diffuse Cutaneous Systemic Sclerosis = Anti-scl70 (topoisomerase)

Dermatomyositis = Anti-Jo1

Polymyositis = Anti-SRP

MedEd + PathSoc + Laz's Qs + Romesh Path (99 decks)

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