Alterations in Immunity and Alterations in Immunity and InflammationInflammation

Chapter 8Chapter 8

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HypersensitivityHypersensitivity

Altered immunologic response to an antigen; Altered immunologic response to an antigen; results in disease/damage to hostresults in disease/damage to host AllergyAllergy

• Deleterious effects of hypersensitivity to environmental Deleterious effects of hypersensitivity to environmental (exogenous) antigens(exogenous) antigens

AutoimmunityAutoimmunity• Disturbance in immunologic tolerance of self-antigensDisturbance in immunologic tolerance of self-antigens

AlloimmunityAlloimmunity• Immune reaction to tissues of another individualImmune reaction to tissues of another individual

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HypersensitivityHypersensitivity Characterized by the immune mechanismCharacterized by the immune mechanism

Type IType I• IgE mediatedIgE mediated

Type IIType II• Tissue-specific reactionsTissue-specific reactions

Type IIIType III• Immune complex mediatedImmune complex mediated

Type IVType IV• Cell mediatedCell mediated

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HypersensitivityHypersensitivity Immediate hypersensitivity reactionsImmediate hypersensitivity reactions AnaphylaxisAnaphylaxis Delayed hypersensitivity reactionsDelayed hypersensitivity reactions

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Type I HypersensitivityType I Hypersensitivity IgE mediatedIgE mediated Against environmental antigens (allergens)Against environmental antigens (allergens) IgE binds to Fc receptors on surface of mast IgE binds to Fc receptors on surface of mast

cells (cytotropic antibody)cells (cytotropic antibody) Histamine releaseHistamine release

HH11 and H and H22 receptors receptors

Antihistamines Antihistamines

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Mast CellMast Cell

Type IType I

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Type I HypersensitivityType I Hypersensitivity ManifestationsManifestations

ItchingItching UrticariaUrticaria ConjunctivitisConjunctivitis RhinitisRhinitis HypotensionHypotension BronchospasmBronchospasm DysrhythmiasDysrhythmias GI cramps and malabsorptionGI cramps and malabsorption

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Clinical Symptoms of IgE Type I Clinical Symptoms of IgE Type I Reactions Reactions

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AnaphylaxisAnaphylaxis

Severity depends on level of sensitizationSeverity depends on level of sensitization Can be very small dose (ordinary skin testing)Can be very small dose (ordinary skin testing) Within minutes after exposure:Within minutes after exposure:

ItchingItching HivesHives Skin erythemaSkin erythema Contraction of respiratory bronchiolesContraction of respiratory bronchioles Laryngeal edema results in hoarsenessLaryngeal edema results in hoarseness Vomiting, abdominal cramps, diarrheaVomiting, abdominal cramps, diarrhea Laryngeal obstructionLaryngeal obstruction

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Type I HypersensitivityType I Hypersensitivity Genetic predispositionGenetic predisposition TestsTests

Food challengesFood challenges Skin testsSkin tests Laboratory testsLaboratory tests

DesensitizationDesensitization IgG-blocking antibodiesIgG-blocking antibodies

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Type II HypersensitivityType II Hypersensitivity Tissue specificTissue specific

Specific cell or tissue (tissue-specific antigens) is Specific cell or tissue (tissue-specific antigens) is the target of an immune responsethe target of an immune response

Five mechanismsFive mechanisms Cell is destroyed by antibodies and complementCell is destroyed by antibodies and complement Cell destruction through phagocytosisCell destruction through phagocytosis Soluble antigen may enter the circulation and Soluble antigen may enter the circulation and

deposit on tissuesdeposit on tissues Antibody-dependent cell-mediated cytotoxicityAntibody-dependent cell-mediated cytotoxicity Causes target cell malfunctionCauses target cell malfunction

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Phagocytosis Phagocytosis

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Neutrophil-Mediated DamageNeutrophil-Mediated Damage

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Antibody-Dependent Cell-Mediated Antibody-Dependent Cell-Mediated CytotoxicityCytotoxicity

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Induced Cell MalfunctionInduced Cell Malfunction

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Type III HypersensitivityType III Hypersensitivity Immune complex mediatedImmune complex mediated Antigen-antibody complexes formed in the Antigen-antibody complexes formed in the

circulation and later deposited in vessel walls circulation and later deposited in vessel walls or extravascular tissuesor extravascular tissues

Not organ specificNot organ specific Immune complex clearanceImmune complex clearance

Large—macrophages Large—macrophages Small—renal clearanceSmall—renal clearance Intermediate—deposit in tissuesIntermediate—deposit in tissues

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Type III HypersensitivityType III Hypersensitivity

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Systemic lupus erythematosusSystemic lupus erythematosus VasculitisVasculitis Poststreptococcal glomerulonephritisPoststreptococcal glomerulonephritis Acute glomerulonephritisAcute glomerulonephritis Reactive arthritisReactive arthritis

Examples of Immune Complex–Examples of Immune Complex–Mediated Diseases: Type IIIMediated Diseases: Type III

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Type IV HypersensitivityType IV Hypersensitivity

Mediated by T lymphocytesMediated by T lymphocytes Destruction of the tissue usually caused by direct Destruction of the tissue usually caused by direct

killing by toxins from Tc cellskilling by toxins from Tc cells Th1 cells produce cytokines that recruit phagocytes, Th1 cells produce cytokines that recruit phagocytes,

especially macrophagesespecially macrophages ExamplesExamples

Acute graft rejection, skin test for TB, contact allergic Acute graft rejection, skin test for TB, contact allergic reactions, and some autoimmune diseasesreactions, and some autoimmune diseases

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Type IV HypersensitivityType IV Hypersensitivity

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Type IV HypersensitivityType IV Hypersensitivity

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Antigenic Targets of Antigenic Targets of Hypersensitivity ReactionsHypersensitivity Reactions

AllergyAllergy Environmental AgsEnvironmental Ags

Autoimmunity Autoimmunity Self Ags, with damage to host tissuesSelf Ags, with damage to host tissues

AlloimmunityAlloimmunity Against foreign tissue AgsAgainst foreign tissue Ags

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AllergyAllergy Environmental antigens that cause atypical Environmental antigens that cause atypical

immunologic responses in genetically immunologic responses in genetically predisposed individuals predisposed individuals Pollens, molds, fungi, foods, animals, etc.Pollens, molds, fungi, foods, animals, etc.

Allergen contained within a particle too large to Allergen contained within a particle too large to be phagocytosed or is protected by a be phagocytosed or is protected by a nonallergenic coatnonallergenic coat

Original insult is apparentOriginal insult is apparent

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AutoimmunityAutoimmunity Breakdown of toleranceBreakdown of tolerance

Body recognizes self-antigens as foreignBody recognizes self-antigens as foreign Sequestered antigenSequestered antigen

Self-antigens not normally seen by the immune Self-antigens not normally seen by the immune systemsystem

Infectious diseaseInfectious disease Molecular mimicryMolecular mimicry

NeoantigenNeoantigen Haptens become immunogenic when they bind to Haptens become immunogenic when they bind to

host proteinshost proteins

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AutoimmunityAutoimmunity Forbidden cloneForbidden clone

During differentiation, lymphocytes produce During differentiation, lymphocytes produce receptors that react with self-antigensreceptors that react with self-antigens

Ineffective peripheral toleranceIneffective peripheral tolerance Defects in regulatory cellsDefects in regulatory cells

Original insultOriginal insult Genetic factorsGenetic factors

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Autoimmune ExamplesAutoimmune Examples Systemic lupus erythematosus (SLE)Systemic lupus erythematosus (SLE)

Chronic multisystem inflammatory diseaseChronic multisystem inflammatory disease Autoantibodies against:Autoantibodies against:

• Nucleic acids, erythrocytes, coagulation proteins, Nucleic acids, erythrocytes, coagulation proteins, phospholipids, lymphocytes, platelets, etc. phospholipids, lymphocytes, platelets, etc.

Deposition of circulating immune complexes Deposition of circulating immune complexes containing antibody against host DNAcontaining antibody against host DNA

More common in femalesMore common in females

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Systemic Lupus ErythematosusSystemic Lupus Erythematosus Clinical manifestationsClinical manifestations

Arthralgias or arthritis (90% of individuals)Arthralgias or arthritis (90% of individuals) Vasculitis and rash (70%Vasculitis and rash (70%––80%)80%) Renal disease (40%Renal disease (40%––50%)50%) Hematologic changes (50%)Hematologic changes (50%) Cardiovascular disease (30%Cardiovascular disease (30%––50%)50%)

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Systemic Lupus ErythematosusSystemic Lupus Erythematosus Eleven common findingsEleven common findings Serial or simultaneous presence of at least Serial or simultaneous presence of at least

four indicates SLEfour indicates SLE Facial (malar) rash, discoid rash, photosensitivity, Facial (malar) rash, discoid rash, photosensitivity,

oral or nasopharyngeal ulcers, nonerosive arthritis, oral or nasopharyngeal ulcers, nonerosive arthritis, serositis, renal disorders, neurologic disorders, serositis, renal disorders, neurologic disorders, hematologic disorders, immunologic disorders, hematologic disorders, immunologic disorders, and presence of antinuclear antibodies (ANAs)and presence of antinuclear antibodies (ANAs)

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AlloimmunityAlloimmunity Immune system reacts with antigens on the Immune system reacts with antigens on the

tissue of other genetically dissimilar members tissue of other genetically dissimilar members of the same speciesof the same species Transient neonatal alloimmunityTransient neonatal alloimmunity

• Fetus expresses parental antigens not found in the Fetus expresses parental antigens not found in the mothermother

Transplant rejection and transfusion reactionsTransplant rejection and transfusion reactions

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Graft RejectionGraft Rejection Transplant rejection classified according to timeTransplant rejection classified according to time

HyperacuteHyperacute• Immediate and rareImmediate and rare

• Preexisting antibody to the antigens of the graftPreexisting antibody to the antigens of the graft

AcuteAcute• Cell-mediated response against unmatched HLA antigensCell-mediated response against unmatched HLA antigens

ChronicChronic• Months or yearsMonths or years

• Inflammatory damage to endothelial cells of vessels due to Inflammatory damage to endothelial cells of vessels due to a weak cell-mediated reaction against minor HLA antigensa weak cell-mediated reaction against minor HLA antigens

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Transfusion ReactionsTransfusion Reactions Antibodies against blood group antigensAntibodies against blood group antigens ABO systemABO system

Two major carbohydrate antigensTwo major carbohydrate antigens• A and B (codominant)A and B (codominant)

• Individuals have naturally occurring antibodies to the A Individuals have naturally occurring antibodies to the A and B antigens they lackand B antigens they lack

• Anti-A and anti-B antibody production is induced by Anti-A and anti-B antibody production is induced by similar antigens on naturally occurring bacteria in the similar antigens on naturally occurring bacteria in the intestinal tractintestinal tract

• Antibodies are usually of the IgM classAntibodies are usually of the IgM class

• O blood type (universal donor)O blood type (universal donor)

• AB blood type (universal recipient)AB blood type (universal recipient)

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ABO Incompatibility: ABO Incompatibility: Alloimmune ReactionAlloimmune Reaction

Blood Blood typetype

AgAg AbAb Incompatible with Incompatible with blood typeblood type

AA AA Anti-BAnti-B B, ABB, AB

BB BB Anti-AAnti-A A, ABA, AB

ABAB

Universal Universal recipientrecipient

A+BA+B NoneNone NoneNone

OO

Universal Universal donordonor

NoneNone Anti-A and Anti-BAnti-A and Anti-B A, B, ABA, B, AB

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ImmunodeficienciesImmunodeficiencies

Result of impaired function of T cells, B cells, Result of impaired function of T cells, B cells, phagocytes and/or complementphagocytes and/or complement Primary (genetic)Primary (genetic) Secondary (acquired)Secondary (acquired) Hallmark is recurrent infections often with Hallmark is recurrent infections often with

opportunistic organismsopportunistic organisms Type of infection can lead to diagnosis of type of Type of infection can lead to diagnosis of type of

deficiency deficiency • Gonorrhea suggests complement deficiencyGonorrhea suggests complement deficiency

• Viral infections suggest T cell deficiencyViral infections suggest T cell deficiency

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ImmunodeficienciesImmunodeficiencies Clinical presentationClinical presentation

Development of unusual or recurrent, severe Development of unusual or recurrent, severe infectionsinfections

T cell deficienciesT cell deficiencies• Viral, fungal, yeast, and atypical microorganismsViral, fungal, yeast, and atypical microorganisms

B cell and phagocyte deficienciesB cell and phagocyte deficiencies• Microorganisms requiring opsonizationMicroorganisms requiring opsonization

Complement deficienciesComplement deficiencies

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ImmunodeficienciesImmunodeficiencies

Primary (congenital) immunodeficiencyPrimary (congenital) immunodeficiency Genetic anomalyGenetic anomaly

Secondary (acquired) immunodeficiencySecondary (acquired) immunodeficiency Caused by another illnessCaused by another illness More commonMore common

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Primary ImmunodeficienciesPrimary Immunodeficiencies Most are the result of a single gene defectMost are the result of a single gene defect Five groupsFive groups

B lymphocyte deficienciesB lymphocyte deficiencies T lymphocyte deficienciesT lymphocyte deficiencies Combined T and B cell deficienciesCombined T and B cell deficiencies Complement defectsComplement defects Phagocyte defectsPhagocyte defects

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B Lymphocyte DeficienciesB Lymphocyte Deficiencies Hypogammaglobulinemia or Hypogammaglobulinemia or

agammaglobulinemiaagammaglobulinemia Bruton agammaglobulinemiaBruton agammaglobulinemia Autosomal agammaglobulinemiaAutosomal agammaglobulinemia X-linked hyper-IgM syndromeX-linked hyper-IgM syndrome IgG subclass deficiencyIgG subclass deficiency Selective IgA deficiencySelective IgA deficiency Common variable immunodeficiencyCommon variable immunodeficiency

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T Lymphocyte DeficienciesT Lymphocyte Deficiencies DiGeorge syndromeDiGeorge syndrome

Partial or complete absence of T cell immunityPartial or complete absence of T cell immunity Chronic mucocutaneous candidiasisChronic mucocutaneous candidiasis

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Combined T- and B-Cell Combined T- and B-Cell DeficienciesDeficiencies

Severe combined immunodeficiency (SCID)Severe combined immunodeficiency (SCID) Reticular dysgenesis Reticular dysgenesis

• Most severe formMost severe form

Adenosine deaminase (ADA) deficiencyAdenosine deaminase (ADA) deficiency X-linked SCIDX-linked SCID JAK3 deficiencyJAK3 deficiency IL-7 receptor deficiencyIL-7 receptor deficiency Purine nucleoside phosphorylase deficiencyPurine nucleoside phosphorylase deficiency

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Combined T and B Cell Combined T and B Cell DeficienciesDeficiencies

RAG-1 or RAG-2 deficiencyRAG-1 or RAG-2 deficiency Bare lymphocyte deficiencyBare lymphocyte deficiency MHC class I and II deficiencyMHC class I and II deficiency Wiskott-Aldrich syndromeWiskott-Aldrich syndrome Ataxia-telangiectasia (AT)Ataxia-telangiectasia (AT)

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Complement DeficienciesComplement Deficiencies C3 deficiencyC3 deficiency Mannose-binding lectin (MBL) deficiencyMannose-binding lectin (MBL) deficiency Properdin deficiencyProperdin deficiency Factor I and factor H deficiencyFactor I and factor H deficiency C9 deficiencyC9 deficiency

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Complement DeficienciesComplement Deficiencies

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Phagocytic DeficienciesPhagocytic Deficiencies Severe congenital neutropeniaSevere congenital neutropenia

Cyclic neutropeniaCyclic neutropenia Leukocyte adhesion deficiency (LAD)Leukocyte adhesion deficiency (LAD) C3 receptor deficiency C3 receptor deficiency ChChéédiak-Higashi syndromediak-Higashi syndrome Myeloperoxidase deficiencyMyeloperoxidase deficiency

Chronic granulomatous diseaseChronic granulomatous disease

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Secondary DeficienciesSecondary Deficiencies Also referred to as acquired deficienciesAlso referred to as acquired deficiencies Far more common than primary deficienciesFar more common than primary deficiencies

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Secondary DeficienciesSecondary Deficiencies

Causes Causes Normal physiology conditionsNormal physiology conditions Psychologic stressPsychologic stress Dietary insufficienciesDietary insufficiencies MalignanciesMalignancies Physical traumaPhysical trauma Medical treatmentsMedical treatments InfectionsInfections AIDSAIDS

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Graft-Versus-Host (GVH) DiseaseGraft-Versus-Host (GVH) Disease

Immunocompromised individuals are at risk for Immunocompromised individuals are at risk for GVH diseaseGVH disease T cells in the graft are mature and capable of cell-T cells in the graft are mature and capable of cell-

mediated destruction tissues within the recipientmediated destruction tissues within the recipient Not a problem if patient is immunocompetentNot a problem if patient is immunocompetent

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Evaluation of Immunity Evaluation of Immunity

Complete blood count (CBC) with a differentialComplete blood count (CBC) with a differential Subpopulations of lymphocytesSubpopulations of lymphocytes

Quantitative determination of immunoglobulinsQuantitative determination of immunoglobulins Subpopulations of immunoglobulinsSubpopulations of immunoglobulins

Assay for total complementAssay for total complement Skin testsSkin tests

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Treatment for Treatment for Immunodeficiencies Immunodeficiencies

Gamma-globulin therapyGamma-globulin therapy Transplantation or transfusionTransplantation or transfusion Treatment with soluble immune mediatorsTreatment with soluble immune mediators Gene therapyGene therapy