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FPP Weeks 1 & 2 > Tolerance and Autoimmunity > Flashcards

Tolerance and Autoimmunity Flashcards

1
Q

Central Tolerance

A

Occurs in the generative lymphoid organs

Positive selection produces immature T cells that then move to the medulla. Any cells that recognize high avidity self antigens presented on MHC class II or class I are killed through apoptosis (exception of T-reg cells)

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2
Q

Peripheral Tolerance (Random process)

Apoptosis:

Anergy:

Suppression:

A

Apoptosis: Fas/Fas ligand binding results in apoptosis/ cytokine withdrawal

Anergy: The functional inactivation of T-lymphocyte - Antigen presenting cells that lack costimulatory molecules such as B7 promote anergy (2nd signal = CD28)

Suppression: Dominant suppression by T-regs

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3
Q

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED)

A

Clinical syndrome characterized by autoimmune adrenal and parathyroid diseases (also associated with fungal infections)

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4
Q

APECED

Defect in _____ gene

Failure of _______ tolerance

Deletion of _________ T-cells

A

Defect in *Aire *gene

Failure of Central tolerance

Deletion of self-reactive T-cells

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5
Q

APECED

Lab findings:

A
  • Low cortisol
  • Low calcium, Low PTH
  • Hyponatremia, hypocalcemia, hyperkalemia
  • Thrush
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6
Q

Autoimmune lymphoproliferative syndrome (ALPS) presents with:

A
  • Lymphadenopathy
  • Splenomegaly
  • Autoimmune cytopenias
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7
Q

ALPS

Mutations in ______ or _____ _____

High levels of _____

Failure of ______ tolerance

A

Mutations in fas or fas ligand

High levels of immunoglobulins (IgG, IgA, IgM)

Failure of peripheral tolerance

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8
Q

ALPS

Lab findings:

A
  • Hypergammaglobulinemia
  • Coomb’s positive hemolytic anemia
  • Flow cytometry shows increase in T-cell receptor positive, CD4 and CD8 negative T cells
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9
Q

Immune dysregulation polyendocrinopathy, X-linked (IPEX) presents with:

A
  • IBD (inflammatory bowel disease)
  • Severe eczema and food allergies
  • Type 1 diabetes mellitus
  • Thyroiditis
  • Autoimmune hemolytic anemia
  • Thrombocytopenia
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10
Q

IPEX

Due to mutation in ______ and loss of _________ cells

Very high ____ levels

A

Due to mutation in Foxp3 and loss of T-regulatory cells

Very high IgE levels

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11
Q

IPEX

Lab findings:

A
  • Hypergammaglobulinemia
  • Coomb’s positive hemolytic anemia
  • IgE of 14,000IU/dL, skin test positive to all foods
  • Low albumin, Fe deficiency
  • Hyperglycemia, glucosuria, low insulin
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12
Q

Central Tolerance: T cells and T regulatory cell (Treg) development

A
  • Negative selection is essential to tolerance, however some self-reactive CD4 cells are not deleted but become T-regulatory cells
  • These cells express Foxp3 and suppress immune responses when activated in the periphery
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13
Q

HLA linkage

A
  • Affects what peptides are presented to lymphocytes
  • HLA determines susceptibility but not what diseases you will get
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14
Q

HLA-B27 is a strong risk factor for _______

A

Ankylosing spondylitis

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15
Q

What besides genetics needs to be considered in cases of autoimmunity

A

Environmental “triggers” and microbial infections

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16
Q

Type 1 (Immediated Hypersensitivity)

  • Ig_
  • T-cell type?
  • Diseases?
A
  • IgE
  • T-cell type? - TH2 development
  • Diseases? Allergic rhinitis, asthma, eczema, food allergies, etc…
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17
Q

Type 2 (Antibody mediated hypersensitivity)

General Characteristics?

Diseases?

A
  • General Characteristics
    • Antibodies are produced to self proteins resulting in disease
    • Crosslinking Fc receptors on macrophage/neutrophils leads to activation and inflammation
    • Phagocytosis
  • Diseases
    • Myasthenia gravis; graves disease
18
Q

What is an aschoff Body?

A

Nodules found in the hearts of individuals with rheumatic fever

19
Q

Acute Rheumatic Fever: J♥NES criteria

A
  • Joints
  • Heart
  • Nodules
  • Erythema marginatum
  • Sydenham’s chorea

2 major or 1 major and 2 minor to make Dx

20
Q

Duration of PCN prophylaxis for…

  1. Rheumatic fever without carditis
  2. Rheumatic fever with carditis, but without residual heart disease
  3. Rheumatic fever with carditis and residual heart disease
A
  1. 5 years since last episode of ARF or until 21 yr of age - whichever is longer
  2. 10 yrs since last ARF episode ore until age 21 - whichever is longer
  3. 10 yrs since last episode of ARF or until 40 years old - whichever is longer (consider lifelong prophylaxis if severe valvular disease or strep A infection)
21
Q

M proteins are expressed by __________ as virulence factors

A

S. Pyogenes

22
Q

ARF pathophysiology

Type of hypersensitivity reaction?

HLA associations for…

  • African American:
  • Whites:
  • Native American:
  • Brazilians:
A

Type II Hypersensitivity

  • African American: HLA-DR2
  • Whites: DR4
  • Native American: DR3
  • Brazilians: DR7
23
Q

Type II hypersensitivity diseases (7)

A
  • Autoimmune hemolytic anemia
  • Pemphigus vulgaris
  • Goodpasture’s syndrome
  • ARF
  • Myasthenia Gravis
  • Grave’s diseases (hyperthyroidism)
  • Pernicious anemia
24
Q

Treatment of antibody mediated diseases

  • Non life-threatening diseases
  • Life-threatening diseases
A
  • Non life-threatening diseases
    • High dose intravenous immunoglobulin (IVIG)
    • Corticosteroids
    • Rituximab - kills B cells
  • Life-threatening diseases
    • ​Plasmapharesis/plasma exchange
    • Basically remove plasma and antibody and replace with new
25
Q

Type 3 hypersensitivity - Immune complex disease

Characteristics?

A
  • When antigens/antibodies combine in circulation they may form immune complexes (IC)
  • Activate complement and lead to clearance of IC
  • Failure to clear, IC can deposit in vasculature and lead to disease
26
Q

Systemic Lupus Erythematosus

  • Description
  • Type __ hypersensitivity
  • Complement defects
A
  • Description: Autoimmune disease characterized by auto-antibody production
  • Type III hypersensitivity
  • Complement defects: C1q, C2, C4 - leads to early onset SLE
27
Q

SLE lab results?

A
  • Coombs+
  • Hemolytic anemia
  • Low serum protein
  • High creatinine
  • Low serum complement levels
  • Proteinuria
  • High ANA
28
Q
  • Mutation in __, __, __ cause Neisseiria infections
A

C3, C4, C5

29
Q

Type III hypersensitivity diseases

A
  • SLE
  • Polyarteritis nodosa
  • Poststreptococcal glomerulonephritis
  • Serum sickeness
  • Arthus reaction
30
Q

Type IV hypersensitivity diseases

A

Cell mediated disease (CD4/CD8)

31
Q

Rheumatoid arthritis pathogenesis

A

Monocytes, macrophages, synovial fibroblasts activated by T cells

32
Q

Three main inflammatory cytokines

A

IL-1, IL-6, TNF

33
Q

What types of immunity contributes to the inflammatory process in rheumatoid arthritis

A

Both cell-mediated and humoral response

34
Q

Rheumatoid Arthritis Treatment

A
  • NSAIDS
  • Anti-metabolites (methotrexate, azathioprine)
  • Glucocorticoids
  • Biologic therapy
    • Anti-TNF agents
    • Anti-IL-1 etc…
35
Q

Symptoms of Dermatomyositis

A
  • Heliotropic rash - eyelids, bridge of nose and cheeks
    • Sun sensitive
  • Gottron’s papules - shiny, erythematous, scaly plaques
    • usually on PIP joints of hand
  • Proximal muscle weakness
  • Capillary changes
    • Dilation, dropout hemmorhage
36
Q

MAC staining in dermatomyositis (3)

A
  1. Ulex europas staining of capillaries
  2. C5b-9 staining showing the membrane attack complex
  3. Two color immunoflorescence showing MAC and capillary co-localization
37
Q

Dermatomyositis - Pathology

4 parts

A
  1. Perifascicular atrophy (outside of muscle dies first)
  2. Perivascular inflammatory infiltrate with CD4+ T cells, B Cells, plasma cells and macrophages
  3. Necrosis of muscle occurs in periphery or in large groups in a wedge like fashion suggestive of infarcts
  4. Microvascular injury with vessel destruction leading to capillary depletion (capillary changes of nailbeds)
38
Q

Polymyositis (pathology)

3 parts

A
  1. Diffuse muscle fiber death next to normal fibers
  2. Mononuclear infiltrates throughout fascicle
  3. Largely CD8 mediated
39
Q

Vasculopathy (in dermatomyositis)

A

Capillary depletion followed by compensatory capillary dilation

40
Q

Autoimmune diseases: Organ-specific

A
  • Multiple Sclerosis - CNS motor and sensory neurons
  • Thrombocytopenia - Platelets
  • Guillan Barre Syndrome - Schwann cell membranes
  • Hashimoto’s thyroiditis - Thyroid
  • Celiac disease - Gluten enteropathy
41
Q

Autoimmune Diseases: Systemic

A
  • Systemic Lupus erthematous (SLE) - autoantibodies to dsDNA, histones, and other nuclear proteins
  • Rheumatoid arthritis - autoantibodies against joint antigens
  • Rheumatic fever - autoantibodies to myosin

FPP Weeks 1 & 2 (18 decks)

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