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YR5 Immunology > Auto-inflammatory and Auto-Immune Disease 3 > Flashcards

Auto-inflammatory and Auto-Immune Disease 3 Flashcards

1
Q

What are anti-nuclear antibodies? How are they detected?

A

Group of antibodies that bind to nuclear proteins
Test: staining of Hep-2 cells (nucleus stains green)
V common to have low titres esp. old women

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2
Q

Which diseases are characterised by anti-nuclear antibodies?

A

SLE
Sjogrens syndrome
Systemic sclerosis
Dermatomyositis
Polymyositis

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3
Q

Describe the abnormalities causing genetic predisposition to SLE

A

Clearance of apoptotic cells- nuclear antigens exposed + accumulate

Cellular activation: cytokine expression, co-stimulatory molecules, intracellular signalling molecules leads to B cell hyper-reactivity + loss of tolerance

-> Production of antibodies directed at intracellular proteins: nuclear + cytoplasmic antigens

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4
Q

Describe the formation of complexes and subsequent consequences in SLE

A

Ab’s bind to Ag to form immune complexes

Complexes deposit in tissues: Skin, Joints, Kidney

Complexes activate Complement (classical)

Complexes stimulate cells expressing Fc + complement receptors

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5
Q

List 11 features of SLE

A

Serositis
Oral or Nasal ulcers
Arthritis in >2 joints
Photosensitivity

Blood disorders
Renal involvement
ANA+
Immunologic
Neurologic Sx

Malar rash
Discoid rash

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6
Q

Compare the staining of the glomerular basment membrane in SLE nephritis against Goodpasture’s disease

A

SLE: “Lumpy bumpy”, as complexes deposit
Goodpasture’s: Smooth, as Ab’s to BM

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7
Q

CRP or ESR… which is more sensitive in SLE flares?

A

ESR is MORE sensitive

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8
Q

How is measurement of unactivated complement proteins a surrogate marker of disease activity in SLE?

A

Ab-Ag complexes activate classical complement pathway
Components deplete if constantly consumed
Quantification of C3 + C4: less complement = more active disease
C4 decreases first, then C3 if very severe

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9
Q

How do you determine the type of ANA?

A

Staining:
Homogenous: anti-dsDNA

Speckled: anti-extractable nuclear antigens, confirm with ELIZA

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10
Q

What are anti-dsDNA antibodies associated with?

A

SLE (specificity 95%)
Occur in 60-70%
High titres a/w severe disease acitvity

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11
Q

What test can give definitive confirmation of SLE after detection of anti-dsDNA antibodies?

A

Crithidia Luciliae staining

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12
Q

List 4 ribonucleoproteins

A

Ro
La
Sm
RNP

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13
Q

In which diseases may anti-Ro and La be present?

A

Sjogren’s (+SLE)

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14
Q

The following extractable nuclear antigens are associated with which diseases:
anti-Scl70 (topoisomerase)
anti-Centromere
anti-Mi2, SRP, Jo1

A

Scl70 (topoisomerase): Diffuse Scleroderma

Centromere: Limited Scleroderma

Mi2, SRP, Jo1: Idiopathic inflammatory myopathies

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15
Q

What acts as a surrogate marker of disease activity?

A

Quantification of C3 + C4

Abs binding to Ag consuming complement, will get low C3 + C4
Tend to deplete C4 first, then if v severe will deplete C3 as well
C3 low: very unwell

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16
Q

How is complement quantified?

A

Measure unactivated compliment proteins, not activated forms

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17
Q

What is the triad characterising anti-phospholipid syndrome?

A
  • Recurrent venous or arterial thrombosis
  • Recurrent miscarriage
  • (slight) Thrombocytopenia: Livedo reticularis
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18
Q

How may anti-phospholipid syndrome present?

A

Alone (primary) e.g. patients with recurrent miscarriage of unexplained cause or unexplained thrombosis
or
in conjunction with AI disease (secondary) e.g. consider if dx of SLE

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19
Q

What is the most common treatable cause of miscarriage?

A

Anti-phospholipid syndrome

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20
Q

Which 3 antibodies may be present in anti-phospholipid syndrome?

A

Lupus anti-coagulant
Anti-cardiolipin
Anti-B2 glycoprotein 1

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21
Q

What happens in the presence of lupus anti-coagulant?

A

Prolongs phospholipid dependent coagulation tests- APTT

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22
Q

Why take caution with APTT in patients with lupus anticoagulant?

A

Prolonged APTT- normally would indicate prone to bleeding but phospholipids are low because of antibodies

APTT is distorted, they are procoagulant

Clotting time can be corrected by adding phospholipids to saturate antibodies (though not if on anti-coagulant)

23
Q

What disordered haemostatic state are those with anti-phospholipid syndrome in?

A

Paradoxically pro-coagulant
Low platelets, but v prone to clots due to antibodies
Anti-cardiolipin + Lupus anticoagulant promote coagulation

24
Q

What characterises primary Sjogren’s syndrome?

A

Inflammatory infiltration + destruction of exocrine glands

Particular involvement of lacrimal + salivary glands

25
Q

Give 5 features of primary Sjogren’s syndrome

A

Dry eyes
Dry mouth
Arthralgias
Fatigue
Increased risk of certain lymphomas e.g. MALT

26
Q

What lab results would be seen in a patient with primary sjogrens syndrome?

A

Anti-nuclear antibody +ve
Speckled staining
ENA +ve: Ro +/- La

27
Q

Why should those with anti-Ro or La that are pregnant be monitored?

A

IgG Abs (monomers)
Can cross placenta, may cross react with foetal cardiac conduction tissue + cause neonatal heart block/ rash

Need specialist foetal cardiac monitoring

28
Q

Give 5 features of limited cutaneous systemic sclerosis

A

CREST
Calcinosis
Raynauds- vascular disease (+ in lungs- primary pulmonary HTN)
Oesophageal dysmotility
Sclerodactlyly
Telangectasia

29
Q

Describe the skin involvement in limited cutaneous systemic sclerosis

A

Does not progress beyond forearms
(may involve peri-oral skin)

30
Q

Describe skin involvement in diffuse cutaneous systemic sclerosis

A

Progresses beyond forearms e.g. often chest + abdomen

31
Q

What characterises diffuse cutaneous systemic sclerosis?

A

CREST features
More extensive GI disease
Interstitial pulmonary disease
Scleroderma kidney/ renal crisis

32
Q

Describe the pathophysiology in systemic sclerosis

A

Type IV: Th2 + Th17 mediated inflammation

Cytokines activate fibroblasts: fibrosis

Endothelial cell activation: microvascular disease

Extractable nuclear antibodies also present

33
Q

Which antibodies are present in systemic sclerosis?

A

Limited: Anti-centromere
Diffuse: Anti-Scl70 (topoisomerase)

34
Q

What are idiopathic inflammatory myopathies? What can they be associated with?

A

Connective tissue disorders characterised by muscle weakness:
Dermatomyositis + Polymyositis

Malignancy

35
Q

Describe the pathophysiology of dermatomyositis

A

Type III: immune complexes deposit in vasculature + irritate skin
In muscle: perivascular CD4 T cells + B cells
+/-Anti-Jo1 + Anti-Mi2 antibodies

36
Q

Describe the pathophysiology of polymyositis

A

Type IV
In muscle, CD8 T cells surround HLA I expressing myofibres
CD8 T cells kill myofibres via perforin/ granzymes
+/-Anti-SRP

37
Q

What investigations should be requested in idiopathic inflammatory myopathies?

A

Extended Myositis panel
+ Screen for malignancy

38
Q

Give 7 features of dermatomyositis

A

Heliotrope rash (peri-orbital)
Mechanics hands: dry, scaly, cracked
Proximal muscle weakness
Gottron’s papules (on extensors of fingers)
Respiratory muscle weakness
Interstitial lung disease (fibrotic)
Raynauds

39
Q

Give 4 features of polymyositis

A

Proximal muscle weakness
Respiratory muscle weakness
Interstitial lung disease (fibrotic)
Raynauds

40
Q

What autoantibody can be used to screen for connective tissue disease?

A

Anti-nuclear antibody

41
Q

What disease is associated with anti-CCP?

A

Rheumatoid Arthritis

42
Q

What disease is associated with Rheumatoid factor?

A

Rheumatoid arthritis
Primary Sjogren’s

43
Q

What disease is associated with anti-dsDNA?

A

SLE

44
Q

What disease is associated with anti-Sm?

A

SLE

45
Q

What disease is associated with anti-RNP?

A

SLE
Mixed connective tissue disease

46
Q

What disease is associated with anti-Ro and anti-La?

A

Primary Sjogren’s
SLE

47
Q

What disease is associated with anti-centromere?

A

Limited cutaneous systemic sclerosis

48
Q

What disease is associated with anti-SCL70?

A

Diffuse cutaneous systemic sclerosis

49
Q

What disease is associated with anti-Jo-1 and other anti-tRNA synthetase antibodies?

A

Idiopathic inflammatory myositis

50
Q

What disease is associated with anti-cardiolipin, anti-B2GPI and lupus anticoagulant?

A

Anti-phospholipid syndrome

51
Q

What are Anti-neutrophil cytoplasmic antibodies? How do they cause disease?

A

Against antigens in granules in neutrophil cytoplasm

Inflammation can lead to expression of the Ags on neutrophil cell surface

Ab-Ag binding can activate neutrophils (Type II/ V reaction)

Neutrophils interact with endothelial cells + damage vessels- vasculitis

52
Q

Which small vessel vasculitides are ANCA associated?

A

Microscopic polyangitis

Granulomatosis with polyangitis (Wegners)

Eosinophillic granulomatosis with polyangitis (Churg-Strauss)

53
Q

What are cANCAs? What are they associated with?

A

Cytoplasmic fluorescence staining ANCAs

A/w Abs to enzyme Proteinase 3

In >90% of GPA patients with renal involvement

54
Q

What are pANCAs? What are they associated with?

A

Perinuclear staining pattern ANCAs

A/w Abs to myeloperoxidase

Less sensitive + specific than cANCA
A/w MPA + eGPA
A/w UC + PSC + AI hepatitis

YR5 Immunology (14 decks)

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