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Y5 Pathology > Imm - Autoinflammatory and Autoimmune Disease 2 > Flashcards

Imm - Autoinflammatory and Autoimmune Disease 2 Flashcards

1
Q

Give examples of polygenic auto-immune disease that involve organ-specific antibodies

A

Grave’s disease
Hashimoto’s thyroiditis
T1DM
Pernicious anaemia
Myasthaenia Gravis
Goodpasture’s syndrome

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2
Q

Give example of polygenic auto-immune disease that involve anti-nuclear antibodies (systemic disease)

A

SLE
Sjogren’s syndrome
Systemic sclerosis
Dermato/polymyositis
ANCA-associated vasculitis

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3
Q

What is the immune pathophysiology of Graves’ disease

A

Type 2 hypersensitivity
IgG antibodies against the TSH receptor → TSHr activation → excessive production of thyroid hormone

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4
Q

What are the clinical features of Graves’ disease

A

Nervous
Palpitations
Heat intolerant
Diarrhoea
Exophthalmos

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5
Q

What is the evidence for Graves disease as a T2HS reaction

A

Antibodies stimulate thyrocytes in vitro
Passive transfer of IgG from patients to rats produces similar symptoms
Babies born to mothers with Graves’ may show transient hyperthyroidism

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6
Q

What is the immune pathophysiology of Hashimoto’s thyroiditis

A

T2 and T4 hypersensitivity
Anti-thyroid peroxidase (TPO) + anti-thyroglobulin antibodies
Thyroid becomes infiltrated with T and B cells

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7
Q

What are the clinical features of Hashimoto’s thyroiditis and which investigation should be done

A

Lethargic
Dry skin and hair
Constipation
Cold intolerant
Goitre

TFTs (anti-TG and TPO Abs are highly prevalent so NOT useful)

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8
Q

What is the immune pathophysiology of T1DM

A

T4 hypersensitivity
CD8 T-cells infiltrate the pancreas and recognise auto-antigens presented by MHC class I on pancreatic beta cells → destruction → lymphocyte infiltration → further islet destruction

Antibodies: anti-islet cell, anti-insulin, anti-GAD, anti-IA-2

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9
Q

What are the clinical features of T1DM and what investigations should be done on first presentation

A

Thirsty
Polyuria
Malaise
Glycosuria

TFts, LFTs, coeliac screen

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10
Q

What is the immune pathophysiology of Pernicious anaemia

A

T2 hypersensitivity
Autoantibodies against intrinsic factor or gastric parietal cells→ no absorption of vitamin B12 → pernicious anaemia and SCDC

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11
Q

What are the clinical features of pernicious anaemia and what are the complications

A

Anaemia (macrocytosis): tired, pale, numbness of feet
May lead to sub-acute combined degeneration of the cord → peripheral neuropathy, optic neuropathy

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12
Q

Which auto-antibodies are associated with the following GI diseases: Pernicious anaemia, coeliac’s disease, Ulcerative colitis

A

Pernicious anaemia: anti-parietal, anti-intrinsic factor
Coeliac’s disease: anti-tTG, anti-endomysial antibodies
UC: P-ANCA

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13
Q

Which autoantibodies are associated with the following liver diseases: Autoimmune hepatitis, primary biliary cholangitis

A

Autoimmune hepatitis: Anti-nuclear (ANA), anti-smooth muscle (SMA), anti liver kidney microsomal proteins (LKM), P-ANCA

PBC: ANA, anti-mitochondrial antibodies (AMA), P-ANCA

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14
Q

What is the immune pathophysiology of myasthenia Gravis

A

T2 Hypersensitivity
Autoantibodies against ACh receptors → failure of depolarisation → absence of muscle action potential

Anti-ACh-R ABs present in ~75% patients

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15
Q

What are the clinical features of myasthenia Gravis and what investigations can be done for it

A

Drooping eyelids (ptosis)
Weakness on repetitive activity (fluctuating)
Symptoms worse at the end of the day

Tensilon test positive - inject edrophonium (anti-cholinesterase) to prolong the life of ACh and allow it to act on the receptors

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16
Q

Which autoantibodies are associated with the following: myasthenia Gravis, neuromyelitis optica spectrum disorder (NMOS), optic neuritis, Encephalitis, seizures

A

Myasthenia Gravis: anti-ACh, anti-striational,
Neuromyelitis optica spectrum disorder (NMOS): anti-aQP4 (aquaporin)
Optic neuritis: anti-MOG
Encephalitis: anti-NMDA
Seizures: anti-GABA

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17
Q

What is the immune pathophysiology of Goodpasture’s disease

A

T2 Hypersensitivity
Anti-basement membrane antibodies (lungs and kidneys) - smooth linear deposition

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18
Q

What are the clinical features of Goodpasture’s

A

Haemoptysis with widespread crackles in lungs
Swelling of legs
Reduced urine ouput
Microscopic haematuria + proteinuria

19
Q

Which alleles are associated with Rheumatoid arthritis

A

HLA DR4, HLA DR1
PAD2 and PAD4
PTPN22

Leads to high levels of citrullination and binding to citrullinated peptides with a higher affinity

20
Q

Which environmental factors are associated with rheumatoid arthritis and why

A

Smoking → development of erosive disease, and increased citrullination
Gum infection with Porphyromonas gingivalis (expresses PAD)

21
Q

Which antibodies are present in rheumatoid arthritis

A

anti-cyclic citrullinated peptide (anti-CCP) - more specific and sensitive
Rheumatoid factor (directed against the Fc region of human IgG) - igM is most commonly tested

22
Q

What type of hypersensitivity reaction is involved in rheumatoid arthritis

A

T2: antibody binds to citrullinated proteins → complement, macrophage, and NK cell activation

T3: immune complex formation between RF and anti-CCP

T4: antigen presenting cells → CD4 T cell → production of IF-gamma and IL17 → production of MMPs, IL1, TNFalpha

23
Q

How are non-organ specific autoimmune diseases tested for

A

Stain Hep2 cells
Presence of anti-nuclear antibodies (ANA)

24
Q

What are the clinical features of SLE

A

CNS – seizures
Skin – butterfly rash, discoid lupus
Heart – endocarditis, myocarditis, serositis, pleuritis, pericarditis
Glomerulonephritis
Haematological – haemolytic anaemia, leukopenia, thrombocytopenia
Arthritis and lymphadenopathy

25
Q

What is the immune pathophysiology of SLE

A

T3 hypersensitivity:
(1) Antibodies bind to antigens to form immune complexes
(2) Immune complexes deposit in tissues (NOT binding) → skin, joints, kidney
- Immune complexes activate complement → classical pathway
- Immune complexes stimulate cells expressing Fc and complement receptors

26
Q

What investigations should be done for SLE

A

ANA via titre (SLE = >1: 640 [normal <1:80])
Anti-dsDNA (highly specific)
Anti-ENA, Ribonucleoproteins: Ro, La, SM, U1RNP
Complement depletion(C4 first then C3)

ELISA: ‘lumpy bumpy’, speckled

27
Q

What does measurement by titre mean

A

minimal dilution at which antibody can be detected

I.E. 1: 640 means it has taken 640 dilutions to get to minimal concentration
I.E. 1: 60 means it has only taken 60 dilutions to get minimal concentration

28
Q

Which markers of SLE can be used to measure disease activity

A

Anti-dsDNA
Complement (unactivated)
Inactive lupus = normal C3 and C4
Moderate active lupus = low C4
Very active lupus = low C3 and C4

29
Q

Describe the complement classical pathway

A
  1. C1, C4, C2
  2. C3
  3. final common pathway
  4. membrane attack complex formation
30
Q

What are the clinical features of anti-phospholipid syndrome

A

Recurrent venous or arterial thrombosis
Recurrent miscarriage

31
Q

What investigations are done for anti-phospholipid syndrome

A

Both parents should be tested

Anti-cardiolipin antibody
Anti-beta 2 glycoprotein 1 antibody
Lupus anti-coagulant (cannot be assessed if the patient is on anticoagulant therapy)

32
Q

What are the clinical features of Sjogren’s syndrome

A

Dry eyes
Dry mouth
Enlargement of parotid glands

33
Q

What is the immune pathophysiology of Sjogren’s syndrome

A

Inflammatory infiltration and destruction of the exocrine glands

Anti-nuclear antibodies
Speckled staining
ENA +ve Ro and La

34
Q

Why are Ro and La ENA significant in pregnancy

A

Can cross the placenta and cross react with foetal cardiac condition tissue to cause neonatal heart block or rash

35
Q

What are the features of limited cutaneous systemic sclerosis and which parts of the skin are involved/not involved

A

CREST
Calcinosis
Raynaud’s
Oesophageal dysmotility
Sclerodactyly
Telengiectasia

Hands are involved (does NOT spread proximally) and peri-oral skin

36
Q

What antibodies are found in limited cutaneous systemic sclerosis

A

Anti-centromere AB

37
Q

What differentiates diffuse from limited systemic sclerosis

A

More GI, pulmonary and renal involvement
Skin involvement of hands AND PROXIMAL PAST the forearms (unlike CREST)
Anti-topoisomerase/Anti-scl70 Ab, RNA polymerase, Fibrillarin

38
Q

What is the immune pathophysiology of dermatomyositis

A

Within muscle → perivascular CD4 T and B cells
Immune complex mediated vasculitis – T3 response

Anti-Jo1 (t-RNA synthetase), Mi2, SRP

39
Q

What is the immune pathophysiology of polymyositis

A

Within muscle – CD8 T cells surround HLA Class 1 expressing myofibers
CD8 cells kill myofibers via perforin/granzymes – T4 response

Anti-Jo1 (t-RNA synthetase), Mi2, SRP

40
Q

Give examples of small vessel vasculitides and which auto-antibody is associated with them

A

Microscopic polyangiitis / Microscopic polyarteritis / MPA
Granulomatosis with polyangiitis / Wegener’s granulomatosis / GPA
Eosinophilic granulomatosis with polyangiitis / Churg-Strauss syndrome / eGPA

Associated with ANCA (Anti-Neutrophil Cytoplasmic Antibody)

41
Q

What is ANCA (Anti-Neutrophil Cytoplasmic Antibody)

A

Antibodies for antigens located in primary granules within cytoplasm of neutrophils
Inflammation → expression of these antigens on cell surface of neutrophils → antibody engagement with cell surface antigens leading to neutrophil activation (T2 hypersensitivity) → activated neutrophils interact with endothelial cells causing damage to vessels → VASCULITIS
Different than ANA

42
Q

what is the difference between cANCA and pANCA

A

cANCA
cytoplasmic fluorescence
associated with antibodies to enzyme proteinase 3
occurs in >90% of patents with GPA with renal involvement

pANCA
perinuclear staining pattern
associated with antibodies to myeloperoxidase (MPO)
less sensitive and specific than cANCA
associated with MPA and eGPA

43
Q

Give examples of large vessel vasculitides

A

Takayasu’s arteritis
Giant cell arteritis/polymyalgia rheumatica

44
Q

Give examples of medium vessel vasculitides

A

Polyarteritis nodosa
Kawasaki disease

Y5 Pathology (71 decks)

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