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MOD 1 Exam 2 and 3 > Ch 6: Immunopathies (Part 3 - Autoimmune diseases) > Flashcards

Ch 6: Immunopathies (Part 3 - Autoimmune diseases) Flashcards

1
Q

What is central tolerance?

A

Learns to tolerate self-ag prior to release from generative lymphoid organs

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2
Q

What is peripheral tolerance?

A

Learns to tolerate self-ag during on-going regulation in peripheral tissues

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3
Q

What is the mechanism behind central tolerance in the thymus/bone marrow?

A

Thymus:
Production of TCRs that cross-react with self-ag detected and apoptosed
Dev. of Tregs

Bone marrow:
Receptor editing
Apoptosis

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4
Q

What are the mechanisms behind peripheral tolerance?

A

For T-cells:
Anergy
Suppression by t-Regs
Apoptosis

For B-cells:
Anergy

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5
Q

Describe how anergy occurs.

A

T-cells have inhibitory receptors: CTLA AND PD-1

Help t-cell downregulate when self-ag are present

CTLA binds B7(CD80) which prevents T-cell activation b/c B7 can’t attach to CD28 (activator)

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6
Q

How do cancer evade T-cell killing? How can this be solved?

A

Uses PD-1 binding to PD-1L to evade T-cell

Anti-PD-1 (and 1L) can reverse this
Also anti-CTLA4

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7
Q

Describe T-Regs. Induced by? Positive for? Express? Actions?

A

Induced by TGF-B

Positive for CD4

Express CD25 and FOXP3

Actions = cytokine immunosupression (using IL-10 AND TGF-B), CTLA-4 inhibition

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8
Q

What is AIRE? What is its purpose and where does it assert its effects? A mutation here causes what?

A

AIRE = autoimmune regulator

It stimulates expression of some “peripheral tissue-restricted” self-Ag in thymus and is responsible for deletion of immature T-cells specific for those Ags

Mutation leads to polyendocrine disorders due to autoimmunity

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9
Q

What is IPEX? What mutation is involved? What normal process fails?

A

IPEX = immune dysregulation, polyendocrinopathy, enteropathy, x-linked

Mutations in FOXP3 cause this

This leads to loss in maintenance of T-regs, which leads to attacks on normal cells.

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10
Q

What 3 requirements are there to define an autoimmune disease?

A

Immune rxn is directed against a self-Ag
Immune rxn primarily responsible for pathologic condition
There is no other pathophysiology responsible

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11
Q

What are required for genesis of an autoimmune disease?

A

Combo of:
Genetic susceptibility
Immune regulation
Environmental contribution - infection, damaged tissue

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12
Q

What is Ankylosing Spondylitis? What gene is it associated with?

A

Hereditary inflammatory condition of joints, particularly spine
Inflammation leads to degeneration and fusion of vertebrae

Strongly associated with Class 1 HLA allele B27

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13
Q

What is Crohn’s disease? What gene is associated?

A

Polymorphisms in NOD2 gene render panted cells in intestinal epithelium ineffective at microbial killing

Defective killing and cleanse allows accusation of bacteria and exaggerated immune response

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14
Q

Describe what happens with OLP and epitope spreading.

A

With OLP, there is an initial T-cell response leading to keratotic lesions in oral and conjunctival mucosa

Basement membrane disruption exposes antigenic proteins

A secondary B-cell response occurs

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15
Q

What is ANA? What can it test for?

A

Anti-nuclear antibody test
SENSITIVITY

Tests for multiple autoimmune diseases:
Systemic lupus erythematous (SLE) - discoid and drug-induced
Sjogren syndrome
Systemic sclerosis

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16
Q

For SPECIFICITY, what can you use to test for SLE?

A

Anti DS DNA

Anti Smith

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17
Q

For SPECIFICITY, what can you use to test for Sjogren syndrome?

A

Anti Ro/SS-A

Anti Ro/SS-B

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18
Q

For SPECIFICITY, what can you use to test for Systemic sclerosis?

A

Anti DNA topoisomerase (Scl-70)

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19
Q

What is SLE?

A

Autoimmune disease associated with vast array of auto-ab

Genetic and environmental influences combine to degrade self-tolerance
Genetic association = family patterns, HLA-DQ
Female bias = X-chromosome
UV light

Multiple immune mechanisms involved:
B-cells and CD4 T-cells
Immune complex formation

20
Q

When do you start to suspect Lupus?

A

Requires 4 or more criteria (ex: discoid rash, malar rash)

Prevalent:
Arthritis
Skin rashes - malar rash
Fever
Fatigue 
Hemolytic anemia 
Edema
21
Q

What is the first class of lupus nephritis? Last class?

A

Minimal mesangial lupus first

Advanced Sclerosing lupus last
fibrosis often end-stage process

22
Q

Describe Diffuse Lupus Nephritis (Class 4).

A

MOST COMMON

Pts symptomatic = proteinuria, hematuria

Glomeruli show increased cellularity
Proliferation of endothelial, mesangial, and epithelial cells
EM shows immune deposits in subendothelium

23
Q

Describe the skin changes you see with SLE.

A

Basal layer degeneration of skin - vacuolated spaces b/w degenerating cells
Malar rash

24
Q

What are some cardiovascular complication with SLE?

A 
Libman-Sacks endocarditis: 
Verrucous (warty) valve deposits 
Comprised of fibrin 
Not infective 
Can embolize (rare)

Coronary artery disease

25
Q

What is an L-E cell? Is it still used for diagnosis?

A

Neutrophil or macrophage that ingest nucleus of damaged cell.

It is not used for diagnosis but may be seen in blood or body fluids

26
Q

How does Discoid Lupus erythematosus differ from SLE in regards to characteristics seen?

A

It does have:
discoid rash
positive ANA
positive immunofluorescence

Discoid DOESN’T have malar rash, renal disease, heart disease, hematologic disorders or positive ds DNA associated with it like SLE does

27
Q

Describe Discoid lupus.

A

Typically, just face and scalp are affected
Positive ANA
Negative Anti-DS DNA

Progression is possible
Disseminated skin lesions
Systemic organ involvement

28
Q

How does Drug-induced Lupus erythematosus differ from SLE in regards to characteristics seen?

A 
It has:
Arthralgia (joint aches), fever
Positive ANA
Discoid rash 
Hematologic disease 
Positive immunofluorescence

DOESN’t have:
renal and CNS disease, positive ds DNA

29
Q

What is Drug-induced lupus (DIL)?

A

Meds induced breakdown of self tolerance (procainamide, hydralazine)

HAs Arthralgia, fever, positive ANA
Positive anti-histone Ab
Resolves with drug discontinuation

30
Q

What is the relationship between certain drugs and HLA allele with risk of DIL?

A

Hydralazine = high risk of DIL in ppl with HLA-DR4 allele

Procainamide = high risk of DIL in ppl with HLA-DR6 allele

31
Q

What is Sjogren syndrome?

A

Autoimmune diease resulting in destruction of lacrimal and salivary gland tissue

32
Q

What is the pathogenesis of Sjogren syndrome?

A

B and T cell mediated inflammatory rxn to target tissues with inflammatory damage followed by fibrotic destruction

33
Q

What clinical presentations do you see with Sjogren syndrome?

A

Dye eyes - due to decreases lacrimal secretions
Dry mouth (xerostomia)
Difficulty swallowing
Root caries - due to dry mouth
Smooth tongue with candida yeast infection due to dry mouth

34
Q

How do you diagnose Sjogren syndrome?

A

Anti-Ro/SS-A and Anti-La/SS-B

Biopsy of lip to look for inflammation of minor salivary gland tissue

35
Q

What are some further complications that can arise with Sjogren syndrome?

A

Extraglandular disease = pulmonary fibrosis

Lymphoid proliferation becoming clonal = lymphoma

36
Q

What is systemic sclerosis (scleroderma)?

A

Fibrosis throughout body (skin, GI tract, kidneys, heart, lungs)

Can occur in limited form = skin + late visceral involvement

Can be part of CREST syndrome

37
Q

What 3 things need to occur for systemic sclerosis to occur?

A

Microvasculopathy

Immune dysregulation

Fibrosis

38
Q

What histological characteristics are seen with systemic sclerosis?

A

Dense collagenous deposition consistent with subcutaneous fibrosis

Vascular hyalinizaion

39
Q

What clinical presentations can be seen with systemic sclerosis?

A

Clubbing of fingers
Progression to Raynaud phenomenon = cold, ischemic fingers become necrotic and distal phalangeal bone undergoes ischemic resorption

GI = GI reflux, esophageal ulceration

Renal vascular disease

Pulmonary = Pulmonary HTN and fibrosis

40
Q

What test is used for systemic sclerosis? What is the ANA pattern?

A

Anti Scl-70 (DNA topoisomerase)

ANA = speckled pattern

41
Q

When and using what test would you see an centromere ANA pattern?

A

Using Anticentromere antibody

CREST syndrome

42
Q

What is CREST syndrome? What does it stand for?

A

Unique form of limited sclerosis
Better prognosis than diffuse sclerosis

C = calcinosis - calcium deposits in skin
R = Raynaud phenomenon - spasm of blood vessels in response to cold or stress 
E = esophageal dysfunction - acid reflux and decrease in motility of esophagus 
S = sclerodactyly - thickening and tightening of skin on finger and hands 
T = tenlangiectasia - dilation of capillaries causing red marks on surface of skin
43
Q

What is mixed connective tissue disease? Has a high titer for?

A

A combination of SLE, systemic sclerosis, and polymyositis

Arguing in favor of it being unique entity
High titer for Anti-ribonucleoprotein (RNP)

44
Q

What is a common presenting feature of mixed connective tissue disease?

A

Raynaud phenomenon

45
Q

What is IgG4-related disease?

A

Newly understood

Unites pathophysiology tying together many disease we know about

46
Q

What diseases are being tied into IgG4-related disease?

A 
Autoimmune pancreatitis 
Reidel thyroiditis 
Mukulicz's syndrome 
Idiopathic retroperitoneal fibrosis 
Inflammatory pseudotumors 
Inflammatory aortitis
47
Q

What is the pathophysiology behind IgG4-related disease?

A

IgG4-producing plasma cell + T-cells, fibrosis

Eventual fibrotic scarring and irreversible damage to involved area

MOD 1 Exam 2 and 3 (9 decks)

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