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    BCA Immune2020

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    Inukaicchi Takumichi
    The document summarizes the different types of hypersensitivity and immune responses. It discusses the four main types of hypersensitivity reactions (Type I-IV), providing examples for each type. It also covers autoimmune diseases like systemic lupus erythematosus, Sjogren's syndrome, and systemic sclerosis. The document concludes by discussing several primary immunodeficiencies like Bruton's agammaglobulinemia, DiGeorge syndrome, SCID, and Wiskott-Aldrich syndrome.

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    BCA Immune2020

    Uploaded by

    Inukaicchi Takumichi
    9 views40 pages
    The document summarizes the different types of hypersensitivity and immune responses. It discusses the four main types of hypersensitivity reactions (Type I-IV), providing examples for each type. It also covers autoimmune diseases like systemic lupus erythematosus, Sjogren's syndrome, and systemic sclerosis. The document concludes by discussing several primary immunodeficiencies like Bruton's agammaglobulinemia, DiGeorge syndrome, SCID, and Wiskott-Aldrich syndrome.

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    The document summarizes the different types of hypersensitivity and immune responses. It discusses the four main types of hypersensitivity reactions (Type I-IV), providing examples for each type. It also covers autoimmune diseases like systemic lupus erythematosus, Sjogren's syndrome, and systemic sclerosis. The document concludes by discussing several primary immunodeficiencies like Bruton's agammaglobulinemia, DiGeorge syndrome, SCID, and Wiskott-Aldrich syndrome.

    Copyright:

    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    9 views40 pages

    BCA Immune2020

    Uploaded by

    Inukaicchi Takumichi
    The document summarizes the different types of hypersensitivity and immune responses. It discusses the four main types of hypersensitivity reactions (Type I-IV), providing examples for each type. It also covers autoimmune diseases like systemic lupus erythematosus, Sjogren's syndrome, and systemic sclerosis. The document concludes by discussing several primary immunodeficiencies like Bruton's agammaglobulinemia, DiGeorge syndrome, SCID, and Wiskott-Aldrich syndrome.

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    Immune System

    Course Audit
    Revised 2020
    Immune Diseases
    Hypersensitivity
    Type I Immediate
    • Within minutes
    • Antigen-IgE-
    mast/basophil
    • Requires previous
    sensitization
    • Histamine, IL 4 & 5
    Type I Immediate
    • Local • Systemic anaphylaxis
    ▫Cutaneous swelling ▫Vascular shock
    –Hives, wheal ▫Widespread edema
    ▫Discharge ▫Difficulty in
    –Allergic rhinitis breathing
    ▫Bronchial asthma
    ▫Hay fever
    ▫Food allergy
    Type II Antibody Mediated

    • Antibodies (auto) directed against


    antigens on cell surface or tissue
    • Three mechanisms seen
    ▫ Phagocytosis with Opsinization
    ▫ Complement mediated inflammation
    ▫ Antibody mediated cellular dysfunction
    Type II Antibody Mediated
    ▫ Phagocytosis with Opsinization
    ▫ Complement mediated inflammation (ADCC)

    • Examples
    ▫ Transfusion reaction
    ▫ Autoimmune hemolytic anemia, HDN
    ▫ Goodpasture Syndrome
    ▫ Rheumatic Fever
    Type II Antibody Mediated

    • Antibody mediated cell dysfunction


    ▫ No injury
    ▫ Blocks normal function

    ▫ Examples
    – Graves Disease
    – Myasthenia Gravis
    Type III Immune Complex
    • Complexes produce inflammation when
    they are deposited
    • Examples
    ▫ Arthus reaction
    ▫ SLE
    ▫ Post Streptococcal Glomerulonephritis
    ▫ Serum sickness
    ▫ PAN
    Type III Immune Complex
    Complexes deposited
    complement and mononuclear cells
    activated
    microthrombi formed
    vasculitis
    glomerulonephritis
    arthritis
    Type III Immune Complex

    • Acute necrotizing vasculitis


    ▫ Fibrinoid necrosis
    – Intense neutrophilic infiltration,
    deposition of complexes, complement
    and plasma proteins produce a smudgy
    appearance
    Type IV Cell Mediated

    • Antigen activated T cells


    ▫ Delayed hypersensitivity - CD4
    ▫ Direct cell cytotoxicity - CD8
    • Intracellular microbes, contact
    dermatitis
    Type IV Cell Mediated

    • Delayed Type
    ▫ Vascular cuffing by mononuclear cells
    ▫ Induration
    ▫ Granuloma formation
    Type IV

    Granuloma
    Type IV
    Examples
    • Tuberculin test
    • Contact dermatitis
    • Hashimoto’s thyroiditis
    • Rheumatoid arthritis
    • Guillain-Barre Syndrome
    Autoimmune Diseases
    • Presence of autoantibodies
    • Autoantibodies cause damage
    • Absence of another well defined cause

    May be
    ØOrgan specific disease
    ØGeneralized/systemic disease
    Antinuclear Antibodies ANA

    Targets Fluorescence
    ØDNA (Smith) ØRim/peripheral
    Øhistones ØHomogenous staining
    Ønon histone proteins ØSpeckled
    (SS-A, SS-B)
    Ønucleoli ØNucleolar
    Systemic Lupus Erythematosus
    • Prototype of multi-system disease
    • acute or insidious, chronic, relapsing,
    remitting febrile illness
    • injury to skin, joints, kidney and serosal
    membranes
    • M:F ratio = 1:9, 20 – 30 years, African
    Americans
    Systemic Lupus Erythematosus

    Autoantibodies found against


    ▫ nuclear components
    ▫ cytoplasmic components
    ▫ cell surface antigens of blood cells
    SLE Tests
    • ANA
    ▫ (+) in every patient – sensitive but
    not specific
    • Anti-ds DNA and Anti Sm
    ▫ specific, virtually diagnostic of SLE
    Other Auto Antibodies
    • Anti phospholipid
    ▫ (+) 40-50% SLE
    ▫ bind to cardiolipin Ag – false (+) VDRL
    ▫ interfere with APTT (in vitro), “lupus
    anticoagulant”
    ▫ Hypercoagulable state
    Tissue Injury in SLE

    • Immune Complex Disease


    ▫ Type III hypersensitivity

    • Phagocytosis of cells bearing


    antibodies
    ▫ Type II hypersensitivity
    SLE Criteria for Diagnosis
    • Serositis • Blood disorder
    • Oral ulcers • Renal disorder
    • Arthritis • ANA
    • Photosensitivity • Immunologic disorder
    • Neurologic disorder

    Any 4 • Malar rash


    SOAP BRAIN MD • Discoid rash
    SLE
    Wire Loop Lesion
    • Homogeneous
    thickening of capillary
    wall
    • Seen in class III, IV
    and V
    Libman Sacks Endocarditis

    • deposits on either
    side of valves
    • Mitral and aortic
    • Leads to
    dysfunction
    Non Infective
    endocarditis
    Sjogren Syndrome
    • Dry eyes
    (keratoconjunctiva sicca),
    dry mouth (xerostomia)
    • Destruction of lacrimal
    and salivary glands
    • Sicca Syndrome
    • Associated with HLA class II
    and viruses
    Sjogren Syndrome

    • Mikulicz syndrome enlargement of


    salivary and lacrimal glands of whatever
    cause: SS, Sarcoidosis, leukemia,
    lymphoma and other tumors

    • 40 % chance of developing Marginal zone


    lymphoma
    Sjogren Syndrome

    • Dx- biopsy of lip


    ▫ 90% (+) SS-A (Ro); SS-B (La)
    Systemic Sclerosis

    • Fibrous tissue accumulation in skin and


    multiple organs

    • CREST SYNDROME calcinosis, Raynaud's,


    esophageal dysfunction, sclerodactyly,
    telangiectasia
    Systemic Sclerosis

    • Skin
    ▫ Claw-like hands,
    mask-like face
    ▫ Auto amputation of
    fingers

    • Dx – anti nucleolar
    Agammaglobulinemia of Bruton

    Ø X linked
    Ø failure of precursor B cells to mature
    Ø Males seen at 6 months
    Ø Recurrent bacterial infections
    Ø B cells are absent or decreased
    Ø T cell mediated reaction are normal
    Di George Syndrome

    T cell deficiency – developmental failure of the


    3rd and 4th pharyngeal pouch

    ØDeletion of Chromosome 22q11. (CATCH 22)


    ØCardiac defect, Abnormal facies, Thymic
    hypoplasia, Cleft palate, Hypocalcemia
    ØPoor defense against viruses and fungus,
    tetany
    Severe Combined Immunodeficiency
    ØBoth humoral and cell mediated
    ØInfants – thrush, diaper rash and failure
    to thrive
    ØDefect in Adenosine DeAminase
    ØHistologic: hypoplasia of thymus and
    other lymphoid tissue
    ØTreatment: BM transplantation
    Wiskott Aldrich Syndrome

    • X linked, recessive; Thrombocytopenia,


    eczema, recurrent infections, early death
    ØWASP gene
    ØThymus – normal
    ØLow IgM; N IgG; high IgA and IgE
    ØProne to develop malignant lymphoma
    AIDS

    • Caused by the retrovirus, HIV


    • Leading cause of death in ages 25-44
    • Profound immunosuppression
    leading to
    ▫ Opportunistic infections
    ▫ Secondary neoplasm
    ▫ Neurologic manifestations
    AIDS Epidemiology
    • Adults • Children under 13
    ▫ Homosexual and ▫ maternal-infant
    bisexual men transmission
    ▫ IV drug abusers ▫ hemophiliacs etc.
    ▫ Heterosexual
    contacts
    ▫ Recipients of
    blood/blood
    components
    ▫ Hemophiliacs
    HIV Pathogenesis
    2 major targets
    1. Immune system, cell mediated
    immunity
    ØViral gp120 binds to CD4+T cells
    ØMacrophages and dendritic cells
    2. Central Nervous System
    Co receptors of HIV
    Ø CCR5 – M tropic strains
    Ø Infects macrophages, monocytes, dendritic
    cells and freshly isolated blood T cells
    Ø Acutely ill patients – 90% of cases are M tropic

    Ø CXCR4 – T tropic strains


    Ø Can only infect T cells
    Ø More virulent and cause final rapid phase of
    disease progression

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