Immunopathology: Hypersensitivity and Autoimmune Diseases

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The main objective of the immune response is to ______ and remove the antigen/pathogen.

neutralize

In hypersensitivity, the antigens are not destructive, but the responses to them cause ______ damage.

tissue

Hypersensitivity reactions are classified based on the mechanisms of ______ damage.

tissue

Type I Hypersensitivity Reactions involve ______ mediated TH2 cells and IgE antibodies.

Ig E Signup and view all the answers

Mast Cell Mediators include ______, which causes vasodilation and increased vascular permeability.

histamine Signup and view all the answers

Type I Hypersensitivity Reactions can be ______ or systemic.

localized Signup and view all the answers

Anaphylaxis is a ______ reaction that follows injection or ingestion of an antigen in a susceptible individual.

systemic Signup and view all the answers

Atopy is a predisposition to develop ______ immediate hypersensitivity reactions to inhaled and ingested antigens.

localized Signup and view all the answers

Alpha gal syndrome is an ______ food allergy caused by IgE-mediated hypersensitivity responses to the glycan galactose-alpha-1,3-galactose.

unconventional Signup and view all the answers

The example case of the 22-month-old baby boy involved an ______ reaction to peanut butter.

IgE-mediated Signup and view all the answers

Autoimmune diseases occur when some aspect of ______ is lost and an adaptive immune response is directed toward normal components of the healthy human body.

self-tolerance Signup and view all the answers

A defining characteristic of autoimmune diseases is the presence of ______ and T cells specific for antigens expressed by the targeted tissue.

antibodies Signup and view all the answers

Type III autoimmune diseases involve the deposition of ______ immune complexes in tissues.

soluble Signup and view all the answers

Systemic Lupus Erythematosus is a type of ______ autoimmune disease.

systemic Signup and view all the answers

The binding of autoantibodies against cell - surface components initiates ______ reactions that cause cell and tissue destruction.

inflammatory Signup and view all the answers

Rheumatoid Arthritis is characterized by the presence of ______ Factor (RF) in 80% of patients.

Rheumatoid Signup and view all the answers

The thyroid gland regulates the basal ______ rate of the body.

metabolic Signup and view all the answers

Rheumatoid Arthritis involves the infiltration of ______ blood cells into the joint synovium.

white Signup and view all the answers

Autoimmune Diseases often involve the impairment of ______ cells within the endocrine gland.

epithelial Signup and view all the answers

Thyroid cells take up ______ used to iodinate and crosslink the tyrosine residues of stored thyroglobulin.

iodine Signup and view all the answers

The epithelial cells of the thyroid make a large ______ called thyroglobulin stored within follicles.

glycoprotein Signup and view all the answers

Tissue erosion in Rheumatoid Arthritis is increased by the action of ______ and collagenases produced by inflammatory cells.

proteinases Signup and view all the answers

DiGeorge syndrome is not an inherited/______ disorder

familial Signup and view all the answers

More than 50% of people with DiGeorge syndrome have a deletion of a gene that maps to chromosome ______q11

22 Signup and view all the answers

People with DiGeorge syndrome often have low numbers of ______ lymphocytes in the blood and lymphoid tissues

T Signup and view all the answers

The T-cell zones of ______ organs are depleted in people with DiGeorge syndrome

lymphoid Signup and view all the answers

Severe Combined Immunodeficiency (SCID) results in defects in both ______ and cell-mediated immune responses

humoral Signup and view all the answers

Infants with Severe Combined Immunodeficiency (SCID) often present with ______ candidiasis and failure to thrive

oral Signup and view all the answers

Phagocytes lack functional ______ – unable to migrate in Leukocyte Adhesion Deficiency.

integrins Signup and view all the answers

Phagocytes cannot produce ______ radicals in Chronic Granulomatous Disease.

superoxide Signup and view all the answers

Infections caused by ______ virulent or opportunistic organisms may indicate primary immunodeficiencies.

poorly Signup and view all the answers

A patient with a neutrophil count of less than 1500/μl is said to have ______.

neutropenia Signup and view all the answers

Early diagnosis of primary immunodeficiencies results in improved ______ outcomes.

clinical Signup and view all the answers

Severe combined immunodeficiency is associated with a ______ of T cells.

progressive depletion Signup and view all the answers

The patient was given epinephrine, saline, ______, and a corticosteroid at the emergency room.

Benadryl Signup and view all the answers

In Anaphylaxis, ______ antibody is bound to mast cells at FcεRI receptor.

IgE Signup and view all the answers

Inflammatory mediators released by mast cells include ______, which increases vascular permeability.

Histamine Signup and view all the answers

Type II Hypersensitivity Reaction involves ______ mediated injury to cells.

antibody Signup and view all the answers

Transfusion reactions are an example of ______ Hypersensitivity Reaction.

Type II Signup and view all the answers

In Type III Hypersensitivity Reaction, and antibodies bind antigens in the circulation.

IgG and IgM Signup and view all the answers

Neutrophils and monocytes produce tissue damage by releasing ______ enzymes in Type III Hypersensitivity Reaction.

lysosomal Signup and view all the answers

Autoimmune hemolytic anemia is an example of ______ Hypersensitivity Reaction.

Type II Signup and view all the answers

Rheumatoid Arthritis involves the infiltration of ______ cells into the joint synovium.

white blood Signup and view all the answers

Prostaglandins and leukotrienes produced by inflammatory cells mediate ______ in Rheumatoid Arthritis.

inflammation Signup and view all the answers

In autoimmune diseases, each disease is due to impairment of a single type of ______ cell within the endocrine gland.

epithelial Signup and view all the answers

The thyroid gland regulates the basal ______ rate of the body by secreting tri-iodothyronine (T3) and tetra iodothyronine (thyroxine or T4).

metabolic Signup and view all the answers

Autoimmune CD4 T cells are activated by ______ cells and these activate macrophages.

dendritic Signup and view all the answers

Thyroid cells are uniquely specialized to take up ______ used to iodinate and crosslink the tyrosine residues of stored thyroglobulin.

iodine Signup and view all the answers

Rheumatoid factor is produced by ______ cells in Rheumatoid Arthritis.

plasma Signup and view all the answers

In Rheumatoid Arthritis, tissue erosion is increased by the action of ______ and collagenases produced by inflammatory cells.

proteinases Signup and view all the answers

Antibodies react with epitopes present on human ______ tissue

heart Signup and view all the answers

Rheumatic fever is a transient ______ disease

autoimmune Signup and view all the answers

The incidence of rheumatic fever has greatly ______ since streptococcal infections began to be treated with antibiotics

diminished Signup and view all the answers

Primary immunodeficiencies are often ______ and may present initially in children and adults

inherited Signup and view all the answers

DiGeorge syndrome is a primary immunodeficiency characterized by ______ hypoplasia

thymic Signup and view all the answers

More than 150 primary ______ have been identified

immunodeficiencies Signup and view all the answers

The most common primary immunodeficiency is ______ deficiency

IgA Signup and view all the answers

When outside temperature drops, the nervous system triggers the pituitary to secrete ______ hormone (TSH)

thyroid-stimulating Signup and view all the answers

In Graves' Disease, symptoms are caused by autoantibodies that bind to the ______ receptor

TSH Signup and view all the answers

Patients with primary immunodeficiencies have an increased susceptibility to ______ and autoimmune diseases

infections Signup and view all the answers

In Hashimoto Disease, patients become ______ and eventually unable to make thyroid hormone

hypothyroid Signup and view all the answers

Type 1 Diabetes Mellitus involves selective autoimmune destruction of the ______ of Langerhans

Islets Signup and view all the answers

In Type 1 Diabetes Mellitus, CD8 T cells specific for peptide antigens unique to ______ cells mediate β – cell destruction

β Signup and view all the answers

During infections, antigen presenting cells may present self-antigens and express ______ receptors to activate T cells

co-stimulatory Signup and view all the answers

Microbial antigens may mimic ______ - antigens (molecular mimicry)

self Signup and view all the answers

Rheumatic Fever is triggered by an initial infection with ______ pyogenes

stretptococcus Signup and view all the answers

Examples of phagocyte deficiencies include ______ and Leukocyte Adhesion Deficiency.

Neutropenia Signup and view all the answers

In Leukocyte Adhesion Deficiency, phagocytes lack functional ______ – unable to migrate.

integrins Signup and view all the answers

In Chronic Granulomatous Disease, phagocytes cannot produce ______ radicals.

superoxide Signup and view all the answers

Examples of primary immunodeficiencies include Severe Combined Immunodeficiency (SCID), Wiskott–Aldrich syndrome, and ______.

DiGeorge Signup and view all the answers

Infections caused by poorly ______ or opportunistic organisms may indicate primary immunodeficiencies.

virulent Signup and view all the answers

Bone marrow transplant has up to ______% survival in treating primary immunodeficiencies.

95 Signup and view all the answers

In oral cavity, Leukocyte Adhesion Deficiency (LAD) can cause ______/periodontal infection.

gingival Signup and view all the answers

Common variable immunodeficiency (CVID) is an example of primary immunodeficiency that presents with ______ sino-pulmonary infections.

recurrent Signup and view all the answers

Systemic Lupus Erythematosus is a type of organ-specific autoimmune disease.

False Signup and view all the answers

Rheumatoid Arthritis involves the infiltration of T cells into the joint synovium.

True Signup and view all the answers

The binding of autoantibodies against cell-surface components initiates apoptosis that causes cell and tissue destruction.

False Signup and view all the answers

Type III Hypersensitivity Reaction involves the deposition of soluble immune complexes in tissues.

True Signup and view all the answers

Rheumatoid Factor is produced by T cells in Rheumatoid Arthritis.

False Signup and view all the answers

Autoimmune diseases often involve the impairment of T cells within the endocrine gland.

False Signup and view all the answers

The female-to-male ratio for Systemic Lupus Erythematosus is 1:1.

False Signup and view all the answers

Corticosteroids are used to suppress immune responses in the management of Systemic Lupus Erythematosus.

True Signup and view all the answers

Type I Hypersensitivity Reactions always involve localized immune responses.

False Signup and view all the answers

In Type I Hypersensitivity Reactions, the initial IgE production is favored by high antigen doses.

False Signup and view all the answers

The main objective of the immune response is to neutralize and remove the antigen/pathogen.

True Signup and view all the answers

Alpha-gal syndrome is a typical IgE-mediated hypersensitivity reaction to a food protein.

False Signup and view all the answers

Mast Cell Mediators include prostaglandins, which cause vasodilation and increased vascular permeability.

True Signup and view all the answers

Anaphylaxis is a delayed hypersensitivity reaction that occurs hours after exposure to an antigen.

False Signup and view all the answers

Atopy is a predisposition to develop delayed hypersensitivity reactions to inhaled and ingested antigens.

False Signup and view all the answers

In Type I Hypersensitivity Reactions, eosinophils and neutrophils are not involved in the late phase response.

False Signup and view all the answers

X-linked Hyper-IgM Syndrome is characterized by elevated levels of IgG and IgA.

False Signup and view all the answers

Common Variable Immunodeficiency is characterized by a deficient T cell response.

False Signup and view all the answers

Patients with Isolated IgA Deficiency are more susceptible to respiratory infections.

True Signup and view all the answers

DeGeorge Syndrome is caused by a failure of development of the third and fourth pharyngeal pouches.

True Signup and view all the answers

CD40L is necessary for Ig class switching and expansion of B cells in lymph nodes.

True Signup and view all the answers

X-linked Hyper-IgM Syndrome is characterized by a deficiency in CD40.

False Signup and view all the answers

Common Variable Immunodeficiency can present with an isolated IgG deficiency.

True Signup and view all the answers

DeGeorge Syndrome is a primary immunodeficiency characterized by a B cell deficiency.

False Signup and view all the answers

DiGeorge syndrome is an inherited familial disorder

False Signup and view all the answers

All people with DiGeorge syndrome have a deletion of a gene that maps to chromosome 22q11

False Signup and view all the answers

Low numbers of T lymphocytes in the blood and lymphoid tissues are characteristic of Severe Combined Immunodeficiency (SCID)

False Signup and view all the answers

Ig levels are always reduced in DiGeorge syndrome

False Signup and view all the answers

Severe Combined Immunodeficiency (SCID) only affects T-cell mediated immunity

False Signup and view all the answers

Infants with DiGeorge syndrome often present with oral candidiasis and failure to thrive

False Signup and view all the answers

DiGeorge syndrome is a type of Severe Combined Immunodeficiency (SCID)

False Signup and view all the answers

The T-cell zones of lymphoid organs are depleted in people with Severe Combined Immunodeficiency (SCID)

False Signup and view all the answers

In Anaphylaxis, IgG antibody is bound to mast cells at FcεRI receptor.

False Signup and view all the answers

Type II Hypersensitivity Reaction is an example of a cell-mediated immune response.

False Signup and view all the answers

Histamine is an inflammatory mediator that increases vascular permeability and causes smooth muscle contraction.

True Signup and view all the answers

Neutrophils and monocytes produce tissue damage by releasing oxidative enzymes in Type III Hypersensitivity Reaction.

False Signup and view all the answers

Autoimmune hemolytic anemia is an example of Type II Hypersensitivity Reaction.

False Signup and view all the answers

IgE antibody is involved in Type I Hypersensitivity Reactions.

True Signup and view all the answers

Transfusion reactions are an example of Type II Hypersensitivity Reaction.

False Signup and view all the answers

In Anaphylaxis, the treatment involves administration of epinephrine, saline, and a corticosteroid.

True Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding mechanisms:

Type I = IgE antibody bound to mast cells at FcεRI receptor Type II = Antibody mediated, cell surface-bound IgG Type III = IgG and IgM antibodies bind antigens in the circulation Anaphylaxis = IgE antibody bound to mast cells at FcεRI receptor Signup and view all the answers

Match the following inflammatory mediators with their effects:

Histamine = Increases vascular permeability and causes smooth muscle contraction Heparin = Increases vascular permeability and causes smooth muscle contraction TNFα = Causes activated macrophages to secrete NO and ROS that can destroy tissue Prostaglandins = Causes vasodilation and increased vascular permeability Signup and view all the answers

Match the following examples with their corresponding hypersensitivity reactions:

Transfusion reactions = Type II Hemolytic disease of the newborn = Type II Rheumatic fever = Type II Peanut butter reaction = Type I Signup and view all the answers

Match the following immune system components with their functions:

Mast cells = Release inflammatory mediators such as histamine and heparin IgE antibody = Bound to mast cells at FcεRI receptor IgG and IgM antibodies = Participate in Type II and Type III hypersensitivity reactions Neutrophils and monocytes = Produce tissue damage by releasing lysosomal enzymes Signup and view all the answers

Match the following diseases with their characteristics:

DiGeorge syndrome = Deletion of a gene that maps to chromosome 22q11 Rheumatic fever = Caused by streptococcal cell wall antigen and antibody cross-reacts with myocardial antigen Anaphylaxis = IgE antibody bound to mast cells at FcεRI receptor Pernicious anemia = Target antigen is intrinsic factor in stomach Signup and view all the answers

Match the following treatments with their uses:

Epinephrine = Treatment of anaphylaxis Corticosteroids = Suppression of immune responses in autoimmune diseases Benadryl = Treatment of anaphylaxis Antibiotics = Treatment of streptococcal infections Signup and view all the answers

Match the following immune responses with their characteristics:

Type I Hypersensitivity Reaction = Localized immune response Type II Hypersensitivity Reaction = Antibody mediated, cell surface-bound IgG Type III Hypersensitivity Reaction = IgG and IgM antibodies bind antigens in the circulation Autoimmune disease = Impairment of immune tolerance Signup and view all the answers

Match the following hypersensitivity reactions with their effects:

Type I = Increased vascular permeability and smooth muscle contraction Type II = Phagocytosis or lysis of cells and tissue injury Type III = Tissue damage by neutrophils and monocytes Anaphylaxis = Rapid pulse, rapid breathing, and low blood pressure Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding mechanisms:

Type I = Production of IgE antibody → immediate release of vasoactive amines and other mediators from mast cells Type II = Production of IgG, IgM → hypersensitivity binds to antigen on target cell or tissue → phagocytosis or lysis of target cell by activated complement or Fc receptors Type III = Immune complex–mediated hypersensitivity → deposition of antigen-antibody complexes → recruitment of leukocytes by complement products and Fc receptors Type IV = Cell-mediated response → CD4+ TH1 cells (and sometimes CD8+ T cells) respond to tissue antigens by secreting cytokines that stimulate inflammation and activate phagocytes, leading to tissue injury Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding examples:

Type I = Anaphylaxis; allergies; bronchial asthma Type II = Autoimmune hemolytic anemia; Goodpasture syndrome Type III = Systemic lupus erythematosus; some forms of glomerulonephritis; serum sickness Type IV = Contact dermatitis; multiple sclerosis; type 1 diabetes; tuberculosis Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding histopathologic lesions:

Type I = Vascular dilation, edema, smooth muscle contraction Type II = Phagocytosis and lysis of cells; immune complex deposition Type III = Inflammation, necrotizing vasculitis (fibrinoid necrosis) Type IV = Perivascular cellular infiltrates; edema; granuloma formation Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding immune mechanisms:

Type I = Immediate hypersensitivity Type II = Antibody-mediated hypersensitivity Type III = Immune complex–mediated hypersensitivity Type IV = Cell-mediated hypersensitivity Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding prototypical disorders:

Type I = Anaphylaxis Type II = Autoimmune hemolytic anemia Type III = Systemic lupus erythematosus Type IV = Contact dermatitis Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding response times:

Type I = Immediate Type II = Minutes to hours Type III = Hours to days Type IV = Days to weeks Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding mediators:

Type I = Mast cells and IgE Type II = IgG and complement Type III = Immune complexes and neutrophils Type IV = CD4+ T cells and cytokines Signup and view all the answers

Match the following hypersensitivity reactions with their corresponding examples of antigen:

Type I = Peanut butter Type II = Thyroglobulin Type III = Penicillin Type IV = Pentadecacatechol (poison ivy) Signup and view all the answers

Match the following autoimmune diseases with their characteristic features:

Systemic Lupus Erythematosus = Circulating IgG antibodies specific for constituents of cell surfaces, cytoplasm and nucleus Rheumatoid Arthritis = Chronic, episodic inflammation of joints with symmetric involvement of multiple joints Autoimmune Hemolytic Anemia = Antibodies to erythrocytes causing anemia Hashimoto Disease = Patients become hypothyroid and eventually unable to make thyroid hormone Signup and view all the answers

Match the following hypersensitivity reactions with their characteristics:

Type II = Autoantibodies are directed against components of cell surfaces or the extracellular matrix Type III = Soluble immune complexes are deposited in tissues Type IV = Effector T cells cause tissue damage Anaphylaxis = A severe, systemic reaction to an antigen Signup and view all the answers

Match the following autoimmune diseases with their types:

Systemic Lupus Erythematosus = Systemic Rheumatoid Arthritis = Organ/Tissue Specific Hashimoto Disease = Organ/Tissue Specific Type 1 Diabetes Mellitus = Organ/Tissue Specific Signup and view all the answers

Match the following autoimmune diseases with their underlying mechanisms:

Systemic Lupus Erythematosus = Broken T cell tolerance to self-antigens Rheumatoid Arthritis = Chronic inflammation of synovium with activated T cells, macrophages, and plasma cells Type 1 Diabetes Mellitus = Selective autoimmune destruction of β cells of the pancreas Autoimmune Hemolytic Anemia = Antibodies to erythrocytes causing anemia Signup and view all the answers

Match the following laboratory findings with their corresponding autoimmune diseases:

ANA titer 1:1280 = Systemic Lupus Erythematosus Rheumatoid Factor (RF) = Rheumatoid Arthritis Anti-DNA Antibody = Systemic Lupus Erythematosus Low complement C3 = Systemic Lupus Erythematosus Signup and view all the answers

Match the following autoimmune diseases with their symptoms:

Systemic Lupus Erythematosus = Butterfly rash on face, stiff fingers and hips in the morning Rheumatoid Arthritis = Chronic, episodic inflammation of joints with symmetric involvement of multiple joints Hashimoto Disease = Patients become hypothyroid and eventually unable to make thyroid hormone Autoimmune Hemolytic Anemia = Anemia Signup and view all the answers

Match the following autoimmune diseases with their management:

Systemic Lupus Erythematosus = Corticosteroids to suppress immune responses Rheumatoid Arthritis = Corticosteroids to suppress immune responses Hashimoto Disease = Thyroid hormone replacement therapy Autoimmune Hemolytic Anemia = Corticosteroids to suppress immune responses Signup and view all the answers

Match the following autoimmune diseases with their prevalence:

Systemic Lupus Erythematosus = Female:Male ratio of 9 or 10:1 Rheumatoid Arthritis = Women:Men ratio of 3:1 Hashimoto Disease = More common in women than men Autoimmune Hemolytic Anemia = No specific prevalence ratio Signup and view all the answers

Match the following primary immunodeficiencies with their characteristics:

X-Linked Agammaglobulinemia = Mutation of the BTK gene, affects males, presents at 6 months of age Hyper-IgM Syndrome = Elevated levels of IgM, impaired antibody class switching DiGeorge Syndrome = Thymic hypoplasia, presents with oral candidiasis and failure to thrive Isolated IgA Deficiency = Deficiency of IgA antibodies, increased susceptibility to respiratory infections Signup and view all the answers

Match the following immune responses with their types of hypersensitivity reactions:

Type I = Immediate, IgE-mediated, anaphylaxis Type II = Antibody-mediated, cell surface antigens Type III = Immune complex-mediated, tissue damage Type IV = Cell-mediated, delayed type hypersensitivity Signup and view all the answers

Match the following autoimmune diseases with their characteristics:

Rheumatoid Arthritis = Inflammation of joints, infiltration of T cells and macrophages Systemic Lupus Erythematosus = Multi-system autoimmune disease, deposition of immune complexes Graves' Disease = Autoantibodies to thyroid-stimulating hormone receptor, hyperthyroidism Hashimoto Disease = Autoantibodies to thyroid peroxidase, hypothyroidism Signup and view all the answers

Match the following immunodeficiencies with their effects on the immune system:

Severe Combined Immunodeficiency = Defects in both humoral and cell-mediated immune responses Common Variable Immunodeficiency = Defects in antibody production, increased susceptibility to infections Hyper-IgM Syndrome = Defects in antibody class switching, impaired immune response DiGeorge Syndrome = Defects in T cell development, impaired cell-mediated immune response Signup and view all the answers

Match the following immunodeficiency disorders with their causes:

X-Linked Agammaglobulinemia = Mutation of the BTK gene DiGeorge Syndrome = Failure of development of the third and fourth pharyngeal pouches Common Variable Immunodeficiency = Defects in multiple genes, impaired immune response Severe Combined Immunodeficiency = Defects in multiple genes, impaired immune response Signup and view all the answers

Match the following immune responses with their mechanisms:

Type I Hypersensitivity Reaction = IgE-mediated, release of histamine and other mediators Type II Hypersensitivity Reaction = Antibody-mediated, complement activation and cell lysis Type III Hypersensitivity Reaction = Immune complex-mediated, tissue damage and inflammation Type IV Hypersensitivity Reaction = Cell-mediated, delayed type hypersensitivity and T cell activation Signup and view all the answers

Match the following immunodeficiency disorders with their symptoms:

DiGeorge Syndrome = Oral candidiasis, failure to thrive, and impaired T cell development Severe Combined Immunodeficiency = Recurrent infections, failure to thrive, and impaired immune response Common Variable Immunodeficiency = Recurrent infections, impaired antibody production, and increased susceptibility to infections Isolated IgA Deficiency = Recurrent respiratory infections, impaired IgA antibody production Signup and view all the answers

Match the following immunodeficiency disorders with their treatments:

X-Linked Agammaglobulinemia = Immunoglobulin replacement and hematopoietic stem cell transplantation Severe Combined Immunodeficiency = Hematopoietic stem cell transplantation and immunoglobulin replacement Common Variable Immunodeficiency = Immunoglobulin replacement and antibiotic therapy DiGeorge Syndrome = Thymus transplantation and immunoglobulin replacement Signup and view all the answers

Match the following immunodeficiency disorders with their characteristic symptoms:

X-linked Hyper-IgM Syndrome = Recurrent pyogenic bacterial infections of the upper and lower respiratory tract Common Variable Immunodeficiency = Abnormal accumulation of immune cells causes enlarged lymph nodes or an enlarged spleen Isolated IgA Deficiency = Respiratory, GI, and GU infections DeGeorge Syndrome = T-cell deficiency, tetany, and congenital defects of the heart and great vessels Signup and view all the answers

Match the following immunodeficiency disorders with their characteristic effects on antibody production:

X-linked Hyper-IgM Syndrome = Very low or no IgA, IgE, and IgG, but normal or elevated IgM Common Variable Immunodeficiency = Deficiency in all antibody classes, or just IgG deficiency Isolated IgA Deficiency = Low levels of serum and secretory IgA DeGeorge Syndrome = No effect on antibody production, as it is a T-cell deficiency Signup and view all the answers

Match the following immunodeficiency disorders with their genetic inheritance patterns:

X-linked Hyper-IgM Syndrome = 70% X-linked, 30% autosomal recessive Common Variable Immunodeficiency = Heterogeneous group of disorders, poorly defined Isolated IgA Deficiency = Familial and acquired forms DeGeorge Syndrome = Not an inherited disorder, results from failure of development of the third and fourth pharyngeal pouches Signup and view all the answers

Match the following immunodeficiency disorders with their effects on B cells:

X-linked Hyper-IgM Syndrome = B cells cannot switch to IgG, IgA, or IgE, and cannot expand in lymph nodes Common Variable Immunodeficiency = Normal numbers of circulating B cells, but not able to differentiate to plasma cells Isolated IgA Deficiency = Impaired differentiation of B lymphocytes to IgA-producing plasma cells DeGeorge Syndrome = No effect on B cells, as it is a T-cell deficiency Signup and view all the answers

Match the following immunodeficiency disorders with their age of onset:

X-linked Hyper-IgM Syndrome = Symptoms typically in the first or second year of life Common Variable Immunodeficiency = Onset in childhood and later Isolated IgA Deficiency = Can occur at any age, familial and acquired forms DeGeorge Syndrome = Congenital, presents at birth Signup and view all the answers

Match the following immunodeficiency disorders with their treatment options:

X-linked Hyper-IgM Syndrome = Treated with IV Ig Common Variable Immunodeficiency = No specific treatment, managed with antibiotics and IV Ig Isolated IgA Deficiency = No specific treatment, managed with antibiotics and IV Ig DeGeorge Syndrome = No specific treatment, managed with immune globulin and antibiotics Signup and view all the answers

Match the following immunodeficiency disorders with their effects on the immune system:

X-linked Hyper-IgM Syndrome = Deficiency in opsonizing IgG, leading to recurrent infections Common Variable Immunodeficiency = Abnormal accumulation of immune cells, leading to enlarged lymph nodes or an enlarged spleen Isolated IgA Deficiency = Impaired immune response to mucosal infections DeGeorge Syndrome = T-cell deficiency, leading to impaired cell-mediated immunity Signup and view all the answers

Match the following immunodeficiency disorders with their associated health risks:

X-linked Hyper-IgM Syndrome = Increased risk of autoimmune disease and lymphoid malignancies Common Variable Immunodeficiency = Increased risk of autoimmune disease and lymphoid malignancies Isolated IgA Deficiency = Increased risk of respiratory, GI, and GU infections DeGeorge Syndrome = Increased risk of heart defects and developmental abnormalities Signup and view all the answers

Immunopathology: Hypersensitivity and Autoimmune Diseases

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Questions and Answers

The main objective of the immune response is to ______ and remove the antigen/pathogen.

In hypersensitivity, the antigens are not destructive, but the responses to them cause ______ damage.

Hypersensitivity reactions are classified based on the mechanisms of ______ damage.

Type I Hypersensitivity Reactions involve ______ mediated TH2 cells and IgE antibodies.

Mast Cell Mediators include ______, which causes vasodilation and increased vascular permeability.

Type I Hypersensitivity Reactions can be ______ or systemic.

Anaphylaxis is a ______ reaction that follows injection or ingestion of an antigen in a susceptible individual.

Atopy is a predisposition to develop ______ immediate hypersensitivity reactions to inhaled and ingested antigens.

Alpha gal syndrome is an ______ food allergy caused by IgE-mediated hypersensitivity responses to the glycan galactose-alpha-1,3-galactose.

The example case of the 22-month-old baby boy involved an ______ reaction to peanut butter.

Autoimmune diseases occur when some aspect of ______ is lost and an adaptive immune response is directed toward normal components of the healthy human body.

A defining characteristic of autoimmune diseases is the presence of ______ and T cells specific for antigens expressed by the targeted tissue.

Type III autoimmune diseases involve the deposition of ______ immune complexes in tissues.

Systemic Lupus Erythematosus is a type of ______ autoimmune disease.

The binding of autoantibodies against cell - surface components initiates ______ reactions that cause cell and tissue destruction.

Rheumatoid Arthritis is characterized by the presence of ______ Factor (RF) in 80% of patients.

The thyroid gland regulates the basal ______ rate of the body.

Rheumatoid Arthritis involves the infiltration of ______ blood cells into the joint synovium.

Autoimmune Diseases often involve the impairment of ______ cells within the endocrine gland.

Thyroid cells take up ______ used to iodinate and crosslink the tyrosine residues of stored thyroglobulin.

The epithelial cells of the thyroid make a large ______ called thyroglobulin stored within follicles.

Tissue erosion in Rheumatoid Arthritis is increased by the action of ______ and collagenases produced by inflammatory cells.

DiGeorge syndrome is not an inherited/______ disorder

More than 50% of people with DiGeorge syndrome have a deletion of a gene that maps to chromosome ______q11

People with DiGeorge syndrome often have low numbers of ______ lymphocytes in the blood and lymphoid tissues

The T-cell zones of ______ organs are depleted in people with DiGeorge syndrome

Severe Combined Immunodeficiency (SCID) results in defects in both ______ and cell-mediated immune responses

Infants with Severe Combined Immunodeficiency (SCID) often present with ______ candidiasis and failure to thrive

Phagocytes lack functional ______ – unable to migrate in Leukocyte Adhesion Deficiency.

Phagocytes cannot produce ______ radicals in Chronic Granulomatous Disease.

Infections caused by ______ virulent or opportunistic organisms may indicate primary immunodeficiencies.

A patient with a neutrophil count of less than 1500/μl is said to have ______.

Early diagnosis of primary immunodeficiencies results in improved ______ outcomes.

Severe combined immunodeficiency is associated with a ______ of T cells.

The patient was given epinephrine, saline, ______, and a corticosteroid at the emergency room.

In Anaphylaxis, ______ antibody is bound to mast cells at FcεRI receptor.

Inflammatory mediators released by mast cells include ______, which increases vascular permeability.

Type II Hypersensitivity Reaction involves ______ mediated injury to cells.

Transfusion reactions are an example of ______ Hypersensitivity Reaction.

In Type III Hypersensitivity Reaction, ______ and ______ antibodies bind antigens in the circulation.

Neutrophils and monocytes produce tissue damage by releasing ______ enzymes in Type III Hypersensitivity Reaction.

Autoimmune hemolytic anemia is an example of ______ Hypersensitivity Reaction.

Rheumatoid Arthritis involves the infiltration of ______ cells into the joint synovium.

Prostaglandins and leukotrienes produced by inflammatory cells mediate ______ in Rheumatoid Arthritis.

In autoimmune diseases, each disease is due to impairment of a single type of ______ cell within the endocrine gland.

The thyroid gland regulates the basal ______ rate of the body by secreting tri-iodothyronine (T3) and tetra iodothyronine (thyroxine or T4).

Autoimmune CD4 T cells are activated by ______ cells and these activate macrophages.

Thyroid cells are uniquely specialized to take up ______ used to iodinate and crosslink the tyrosine residues of stored thyroglobulin.

Rheumatoid factor is produced by ______ cells in Rheumatoid Arthritis.

In Rheumatoid Arthritis, tissue erosion is increased by the action of ______ and collagenases produced by inflammatory cells.

Antibodies react with epitopes present on human ______ tissue

Rheumatic fever is a transient ______ disease

The incidence of rheumatic fever has greatly ______ since streptococcal infections began to be treated with antibiotics

Primary immunodeficiencies are often ______ and may present initially in children and adults

DiGeorge syndrome is a primary immunodeficiency characterized by ______ hypoplasia

More than 150 primary ______ have been identified

The most common primary immunodeficiency is ______ deficiency

When outside temperature drops, the nervous system triggers the pituitary to secrete ______ hormone (TSH)

In Graves' Disease, symptoms are caused by autoantibodies that bind to the ______ receptor

Patients with primary immunodeficiencies have an increased susceptibility to ______ and autoimmune diseases

In Hashimoto Disease, patients become ______ and eventually unable to make thyroid hormone

Type 1 Diabetes Mellitus involves selective autoimmune destruction of the ______ of Langerhans

In Type 1 Diabetes Mellitus, CD8 T cells specific for peptide antigens unique to ______ cells mediate β – cell destruction

During infections, antigen presenting cells may present self-antigens and express ______ receptors to activate T cells

Microbial antigens may mimic ______ - antigens (molecular mimicry)

Rheumatic Fever is triggered by an initial infection with ______ pyogenes

Examples of phagocyte deficiencies include ______ and Leukocyte Adhesion Deficiency.

In Leukocyte Adhesion Deficiency, phagocytes lack functional ______ – unable to migrate.

In Chronic Granulomatous Disease, phagocytes cannot produce ______ radicals.

Examples of primary immunodeficiencies include Severe Combined Immunodeficiency (SCID), Wiskott–Aldrich syndrome, and ______.

Infections caused by poorly ______ or opportunistic organisms may indicate primary immunodeficiencies.

Bone marrow transplant has up to ______% survival in treating primary immunodeficiencies.

In oral cavity, Leukocyte Adhesion Deficiency (LAD) can cause ______/periodontal infection.

Common variable immunodeficiency (CVID) is an example of primary immunodeficiency that presents with ______ sino-pulmonary infections.

Systemic Lupus Erythematosus is a type of organ-specific autoimmune disease.

Rheumatoid Arthritis involves the infiltration of T cells into the joint synovium.

In Type III Hypersensitivity Reaction, and antibodies bind antigens in the circulation.