Immuno 11: Autoimmune And Auto-inflammatory Disorders 2

Question 1

Patient presents with nervousness, diarrhoea, heat intolerance and on examination has exophthalmos. What is the most likely auto-immune disease causing this ?

Answer 1

Grave’s disease

Question 2

What causes hyperthyroidism in Grave’s disease ?

Answer 2

IgG antibodies that stimulate TSH receptors

Question 3

What type of hypersensitivity reaction is grave’s disease ?

Answer 3

Type 2

Question 4

What is a goitre ?

Answer 4

Enlarged thyroid due to T and B cell infiltration

Question 5

What causes hypothyroidism in Hashimoto’s Thyroiditis ?

Answer 5

Anti- thyroid peroxidase antibodies and anti-thyroglobulin antibodies

Question 6

What type of hypersensitivity reaction is hashimoto’s thyroiditis ? (2 option)

Answer 6

Type 2 and type 4

Question 7

List 2 of the auto-antigens presented by MHC class 1 molecules to cytotoxic CD8+ T cells that causes destruction of the Beta cells of the pancreas in Type 1 diabetes mellitus

Answer 7

islet antigen 2 (IA2)

Glutamic acid dehydrogenase (GAD)

Question 8

List the 4 antibodies associated with T1DM ?

Answer 8

Anti-islet cell antibodies
Anti-GAD antibodies
Anti-insulin antibodies
Anti-IA2 antibodies

Question 9

Which auto- antibodies are associated with pernicious anaemia ?

Answer 9

Anti-IF antibodies (Intrinsic factor)

Question 10

Pernicious anaemia is an example of a …………. anaemia

Answer 10

Macrocytic

Question 11

Anti-IF antibodies in pernicious anaemia causes failure of …….. absorption

Answer 11

Vitamin B12

Question 12

Pernicious anaemia can cause subacute …………

Answer 12

Subacute degeneration of the spinal cord

Question 13

Apart from anti IF antibodies which other auto-antibodies are seen in pernicious anaemia ?

Answer 13

Anti-parietal cell antibodies

Question 14

Patient presents with drooping eyelids and weakness which is worse at the end of the day. What is the likely diagnosis ?

Answer 14

Myasthenia gravis

Question 15

Deficiency of which vitamin causes subacute degeneration of the spinal cord ?

Answer 15

Vitamin B12

Seen in pernicious anaemia

Question 16

Which Autoantibodies cause Myasthenia gravis ?

Answer 16

Acetyl choline receptor antibodies

Question 17

Which test is used to diagnose Myasthenia Gravis ?

Answer 17

Tension test

Question 18

Which autoimmune disease causes haematuria, proteinuria and pulmonary haemorrhage ?

Answer 18

Goodpasture’s syndrome

Question 19

What Histological finding on renal biopsy suggests Goodpasture’s syndrome ?

Answer 19

Crescentric nephritis

Question 20

Which auto-antibodies are associated with Goodpasture’s syndrome ?

Answer 20

Anti-basement membrane antibodies

Question 21

Patient presents with Pain and stiffness of multiple joints in the hands. He also has a normocytic anaemia and elevated ESR and CRP. What is the most likely diagnosis ?

Answer 21

Rheumatoid arthritis

Question 22

Which HLA polymorphisms are associated with Rheumatoid arthritis ?

Answer 22

HLA DR4

HLA DR1

Question 23

Which bacterial gum infection is associated with Rheumatoid arthritis ?

Answer 23

P.Gingivalis

Question 24

Is smoking associated with increased or decreased risk of rheumatoid arthritis ?

Answer 24

Increased

Question 25

Which antibodies are characteristically seen in rheumatoid arthritis ?

Answer 25

Anti-CCP antibodies | Anti-Rheumatoid factor

Question 26

Which 3 types of hypersensitivity reactions occur in Rheumatoid arthritis ?

Answer 26

Hypersensitivity 2- antibody binds to CCP (peptides) causing complement activation (classical pathway), Macrophage and NK cell activation Hypersensitivity 3- Immune complexes deposit Hypersensitivity 4- APCs present peptides (citrulinated, self) to CD4+ T cells causing release of IFN gamma and IL-17 which activate macrophages and fibroblasts. MMPs and TNF-alpha are then released

Question 27

What is a ‘Pannus’ in Rheumatoid arthritis ?\

Answer 27

Inflammed synovial cartilage which invades the articular cartilage and adjacent bone structures.

Question 28

Which auto-antibodies are characteristic of connective tissue diseases ?

Answer 28

ANA

Question 29

How do you test for ANA ?

Answer 29

Flourescently label anti human antibodies which will bind to ANA. Mix with patients serum and Hep 2 cells.

Question 30

List 6 pathologies seen in SLE ?

Answer 30

``` Butterfly rash Discoid rash Endocarditis Pleuritis GLomerulonephritis Arthritis Haemolytic anaemia ```

Question 31

Histology of the kidney shows “Lumpy bumpy” pattern of immunoflourescence. Which disease is more likely: A) SLE B) Goodpasture’s disease

Answer 31

A- SLE causes lumpy bumpy pattern due to immune complex deposition (type 3 hypersensitivity reaction)

Question 32

Which Auto-antibody is useful for monitoring disease severity in SLE ?

Answer 32

Anti- dsDNA

Question 33

Which of these Auto-antibodies is not often seen in SLE ? ``` A) ANA B) dsDNA C) topoisomerase (Scl70) D)smith E)Ro D)La ```

Answer 33

C- Anti- topoisomerase (Scl70) is seen in Diffuse Cutaneous Systemic Sclerosis and not in SLE All the other antibodies can be seen in SLE Ro and La are more present in Sjögren’s syndrome

Question 34

What is the Triad for Antiphospholipid syndrome ?

Answer 34

Recurrent venous or arterial thrombosis Recurrent miscarriage Thrombocytopenia

Question 35

List the 2 main antibody tests in APLS ?

Answer 35

Anti-Cardiolipin antibody | Lupus anticoagulant test- coagulation dependent on phospholipids is prolonged

Question 36

List the 6 pathologies seen in Limited Cutaneous Systemic Sclerosis (CREST) ?

Answer 36

``` Calcinosis Raynauds Oesophageal dysmotility Sclerodactyly Telangectasia Primary pulmonary hypertension ```

Question 37

Which features of cutaneous Systemic Sclerosis are different to CREST ?

Answer 37

Skin involvement goes beyond forearm Renal involvement: scleroderma kidney/renal cysts Also has all the other features of CREST

Question 38

Which 2 antibodies allows differentiation of CREST from Diffuse cutaneous Systemic Sclerosis ?

Answer 38

CREST- Anti-centromere antibodies | Diffuse cutaneous systemic sclerosis- Anti-Topoisomerase antibodies (Scl70)

Question 39

Which disease causes weakness, malaise, peri-orbital heliotrope rash and gottron’s papules ?

Answer 39

Dermatomyositis

Question 40

How is polymyositis different to dermatomyositis ?

Answer 40

There is no rash in Polymyositis

Question 41

Name 1 autoantibody seen in dermatomyositis ?

Answer 41

Anti-aminoacyl tRNA synthetase antibody (anti-Jo1) Anti- Mi2 is more common in DM than PM

Question 42

Name 1 autoantibody seen in Polymyositis ?

Answer 42

anti-signal recognition peptide antibody

Question 43

Patient presents with recurrent nosebleeds and breathlessness. Examination reveals collapse of the nose bridge. X ray shows cavitation get lesions. What is the likely cause ?

Answer 43

ANCA associated vasculitis (small vessel)

Question 44

List 3 types of small vessel vasculitis (ANCA associated vasculitis) ?

Answer 44

Microscopic polyangitis (MPA) Granulomatosis with polyangitis (GPA) (aka wegener’s) Eosinophilic granulomatosis with polyangitis (eGPA) (Churg-Strauss syndrome)

Question 45

Which type of ANCA is associated with granulomatosis with polyangitis ? A) cANCA B) pANCA

Answer 45

A- suggests Granulomatosis with polyangitis with renal involvement (pANCA suggests MPA or eGPA)

Question 46

Which auto-antibodies can be seen in Primary sclerosis cholangitis / Autoimmune hepatitis ?

Answer 46

Anti- SMA | Autoimmune hepatitis can also have anti-LMK-1

Question 47

Which type of ANCA is associated with eosinophilic granulomatosis with polyangitis (Churg-strauss syndrome) ?

Answer 47

P-ANCA

Question 48

Which antibodies are associated with Primary billiard cirrhosis ?

Answer 48

Anti-mitochondrial antibodies

Question 49

Patient presents with blurry vision and weakness in her legs. CSF shows Oligoclonal bands of IgG on electrophoresis. Most likely diagnosis ? What type of hypersensitivity reaction is this ?

Answer 49

Multiple sclerosis (MS) Optic neuritis and demyelination. Type 4 hypersensitivity reaction targeting oligodendrocyte and myelin proteins.

Question 50

Which antibody is characteristic of Pemphigus vulgaris ?

Answer 50

Anti- Demoglein 3

Question 51

Which disease is suggested by a +ve Nikolsky's sign ?

Answer 51

Pemphigus vulgaris Bullae rupture easily

Question 52

Skin histology shows acantholytic cells (separation of keratinocytes caused by loss of intercellular cadherin connections). Leading to blistering. Most likely diagnosis ?

Answer 52

Pemphigus vulgaris

Question 53

Immunofluorescence shows A linear pattern of IgG and C3 at the basement membrane. Causes blistering. Most likely diagnosis ?

Answer 53

Bullous pemphigoid.

Question 54

List 2 diseases for which interferon Beta is a treatment ?

Answer 54

- Relapsing MS | - Bechets

Question 55

for which disease is inteferon gamma used as a treatment ?

Answer 55

Chronic granulomatous disease