Epidemiology of Primary Immunodeficiency Disorders
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Questions and Answers

What type of immune response is involved in Serum Sickness?

  • Type II
  • Type IV
  • Type III (correct)
  • Type I
  • Which of the following is NOT a characteristic of Arthus Reaction?

  • Mediated by IgG antibodies
  • Involves T-cell mediated response (correct)
  • Occurs due to repeated exposure to an antigen
  • Localized immune complex deposition
  • Inflammatory response in Type IV hypersensitivity reactions is primarily driven by which of the following cell types?

  • B cells
  • T cells (correct)
  • Macrophages
  • Mast cells
  • Which type of hypersensitivity reaction is also known as delayed-type hypersensitivity?

    <p>Type IV</p> Signup and view all the answers

    What is the primary immunoglobulin involved in Hypersensitivity Type I reactions?

    <p>IgE</p> Signup and view all the answers

    Which of the following reactions is characterized by vasculitis near the injection site?

    <p>Arthus Reaction</p> Signup and view all the answers

    What type of reaction is mediated by T-cells sensitized by antigens presented by antigen-presenting cells (APCs)?

    <p>Hypersensitivity Type IV</p> Signup and view all the answers

    Which of the following reactions can lead to tissue damage through the release of cytokines and chemokines?

    <p>Hypersensitivity Type IV</p> Signup and view all the answers

    Which of the following hypersensitivity reactions plays a role in transplant rejection?

    <p>Hypersensitivity Type IV</p> Signup and view all the answers

    Why is the Arthus Reaction considered a Type III hypersensitivity reaction?

    <p>Deposition of immune complexes</p> Signup and view all the answers

    Which of the following is NOT a characteristic of the acute phase reaction caused by hypersensitivity Type I?

    <p>Tissue infiltration and destruction</p> Signup and view all the answers

    In hypersensitivity Type II reactions, what type of immune response is involved?

    <p>IgG or IgM mediated cytotoxic response</p> Signup and view all the answers

    Which of the following allergic reactions leads to life-threatening respiratory failure most commonly?

    <p>Anaphylaxis</p> Signup and view all the answers

    Which type of hypersensitivity reaction involves a complex series of reactions that cause serum sickness?

    <p>Hypersensitivity Type III</p> Signup and view all the answers

    What type of inflammatory response is characterized by intense tissue infiltration and destruction in hypersensitivity Type I reactions?

    <p>Arthus reaction</p> Signup and view all the answers

    Which type of reaction is typically seen after vaccination, presenting at the site of injection as swelling after 24 hours?

    <p>Arthus Reaction</p> Signup and view all the answers

    What type of immune response is triggered by drugs containing proteins of other species, leading to Serum Sickness?

    <p>Type III Immune Complex-Mediated Hypersensitivity</p> Signup and view all the answers

    How does the accumulation of antigen-antibody complexes in multiple tissue locations lead to tissue damage?

    <p>Activation of complement leading to inflammatory cell recruitment and release of damaging molecules</p> Signup and view all the answers

    What are the non-specific symptoms that may be observed approximately 10 days after exposure to immune complexes in Serum Sickness?

    <p>Fever, urticaria, arthralgias, proteinuria, adenopathy</p> Signup and view all the answers

    Which hypersensitivity reaction involves recruitment of macrophages and neutrophils causing inflammatory damage to tissues after complement activation?

    <p>Hypersensitivity Type III</p> Signup and view all the answers

    Which primary immunodeficiency disorder is characterized by a breakdown in tolerance leading to activation of autoimmunity?

    <p>Selective IgA deficiency</p> Signup and view all the answers

    What demographic group has the highest incidence of Selective IgA deficiency?

    <p>African Americans</p> Signup and view all the answers

    Which autoimmune disorder presents with severe dryness due to inflammation and damage to lacrimal and salivary glands?

    <p>Sjogren's syndrome</p> Signup and view all the answers

    What is the hallmark manifestation of systemic lupus erythematosus involving the skin?

    <p>Malar rash</p> Signup and view all the answers

    Which laboratory finding is typically positive in systemic lupus erythematosus?

    <p>Antinuclear antibodies (ANA)</p> Signup and view all the answers

    What is the mechanism of action of antibody-dependent cellular toxicity in type II hypersensitivity reactions?

    <p>Antibodies bind to the target cell's surface and to macrophages</p> Signup and view all the answers

    Which common cause of type II hypersensitivity reactions involves antibodies binding to self-antigens and activating the complement pathway?

    <p>Autoimmune thrombocytopenia</p> Signup and view all the answers

    How do antibodies in type II hypersensitivity reactions cause cellular dysfunction without inflammation or destruction?

    <p>Autoantibodies bind to the receptors on target cells causing dysfunction without inflammation or destruction</p> Signup and view all the answers

    In type II hypersensitivity reactions, what do antibodies (IgG) binding to the target cell's surface and activating the complement pathway create?

    <p>Channel to induce lysis</p> Signup and view all the answers

    Which mechanism in type II hypersensitivity reactions acts as an opsonin, targeting cells for phagocytosis?

    <p>Antibodies binding to target cells and macrophages</p> Signup and view all the answers

    Which autoimmune disease primarily affects the thyroid gland but can also involve the eyes and skin?

    <p>Grave's Disease</p> Signup and view all the answers

    What is the most common cause of hyperthyroidism?

    <p>Thyroid stimulating immunoglobulin (TSI)</p> Signup and view all the answers

    Which autoimmune disease involves inhibition of intrinsic factor that leads to vitamin B12 deficiency?

    <p>Pernicious Anemia</p> Signup and view all the answers

    What is the mechanism by which Grave's Disease primarily affects the thyroid gland?

    <p>Thyroid stimulating immunoglobulin (TSI) binding to TSH receptor</p> Signup and view all the answers

    Which autoimmune disease is rare and primarily affects vitamin B12 absorption leading to megaloblastic anemia?

    <p>Pernicious Anemia</p> Signup and view all the answers

    Which autoimmune disease is characterized by inflammation and cellular proliferation that affects large and medium arteries?

    <p>Takayasu Arteritis</p> Signup and view all the answers

    'Heat intolerance, sweating, fatigue, weight loss' are symptoms associated with which autoimmune disease?

    <p>Grave's Disease</p> Signup and view all the answers

    'Jaw claudication, ophthalmic manifestations, headache' are symptoms associated with which autoimmune disease?

    <p>Grave's Disease</p> Signup and view all the answers

    'Unilateral or bilateral enlargement of the temporal artery, new-onset headache' are features of which autoimmune condition?

    <p>Giant Cell Arteritis</p> Signup and view all the answers

    'Weight loss, malaise, fever' are considered constitutional symptoms associated with which autoimmune condition?

    <p>Takayasu Arteritis</p> Signup and view all the answers

    Which of the following autoimmune diseases is characterized by mucocutaneous manifestations such as a malar or butterfly rash?

    <p>HIV/AIDS</p> Signup and view all the answers

    What primary immunodeficiency disorder has a higher propensity for conditions like asthma, rheumatoid arthritis, gluten intolerance, and IBS?

    <p>Wiskott-Aldrich Syndrome</p> Signup and view all the answers

    Which autoimmune disease presents with anorexia, weight loss, and fatigue as constitutional symptoms?

    <p>Wiskott-Aldrich Syndrome</p> Signup and view all the answers

    In which disorder is there a deficiency of IgA, with a higher incidence in African Americans and a younger presentation?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    Which autoimmune disorder involves severe dryness (Sicca) due to inflammation and damage to lacrimal and salivary glands?

    <p>HIV/AIDS</p> Signup and view all the answers

    Which of the following diseases is characterized by a deficiency in IgA?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    What is the hallmark feature of Wiskott-Aldrich Syndrome?

    <p>Deficiency in platelet function</p> Signup and view all the answers

    In HIV/AIDS, what defines Severe/Advanced stage of the disease?

    <p>CD4 cell count less than 200 cells/microL</p> Signup and view all the answers

    Which test is used for diagnosing HIV infection with results available within 1 hour?

    <p>ELISA test</p> Signup and view all the answers

    Which autoimmune disease primarily affects the eyes and skin in addition to the thyroid gland?

    <p>Grave's Disease</p> Signup and view all the answers

    Which genetic condition is characterized by a deletion on 22q11.2, absence or hypoplasia of the thymus, and decreased T-cells for cellular immunity?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    Which condition is caused by mutations in the WAS gene, leading to immune deficiency, recurrent infections, and an increased risk of lymphoma?

    <p>Wiskott-Aldrich Syndrome</p> Signup and view all the answers

    Which syndrome is known to be caused by a deletion on chromosome 22q11.2 and results in underdeveloped parathyroid glands and congenital heart disease?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    Which medical condition is managed by Highly Active Antiretroviral Therapy (HAART) to prevent DNA changes and production of viral proteins?

    <p>HIV/AIDS</p> Signup and view all the answers

    Which condition is characterized by a genetic defect on chromosome 22q11.2, underdeveloped parathyroid glands, and a decreased immune response leading to increased susceptibility to infections?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    What is the estimated prevalence of Secondary Sjogren's Syndrome in the population?

    <p>0.5-1.0%</p> Signup and view all the answers

    Which autoimmune condition is commonly associated with Sjogren's Syndrome?

    <p>Systemic Lupus Erythematosus</p> Signup and view all the answers

    What is a common manifestation of Sjogren's Syndrome that might lead to the need for lubricant drops and punctal plugs?

    <p>Dry eye</p> Signup and view all the answers

    Which autoimmune condition can present with constitutional symptoms like weight loss, malaise, and fever, similar to those in Takayasu Arteritis?

    <p>Rheumatoid Arthritis</p> Signup and view all the answers

    What type of laboratory test is typically used to confirm a diagnosis of Systemic Lupus Erythematosus?

    <p>ANA, RF, SS-A, SSTear</p> Signup and view all the answers

    Which autoimmune disease is characterized by the hallmark feature of 'butterfly rash' on the face?

    <p>Systemic Lupus Erythematosus</p> Signup and view all the answers

    Which condition presents with inflammation and damage to lacrimal and salivary glands, leading to dryness of the eyes and mouth?

    <p>Sjogren's Syndrome</p> Signup and view all the answers

    Which genetic condition results in underdeveloped parathyroid glands, congenital heart disease, and decreased T-cells for cellular immunity?

    <p>DiGeorge Syndrome</p> Signup and view all the answers

    Which autoimmune disease involves inhibition of intrinsic factor causing vitamin B12 deficiency and megaloblastic anemia?

    <p>Sjogren's Syndrome</p> Signup and view all the answers

    Which condition is characterized by a deficiency in IgA, typically presenting with a higher incidence in African Americans and a younger age of onset?

    <p>Sjogren's Syndrome</p> Signup and view all the answers

    What is the primary mechanism leading to the development of Systemic Lupus Erythematosus?

    <p>Breakdown in tolerance in genetically susceptible individuals</p> Signup and view all the answers

    Which demographic group has the highest incidence of Systemic Lupus Erythematosus?

    <p>African Americans</p> Signup and view all the answers

    What is the most common autoimmune disorder associated with a higher propensity in individuals affected by asthma, rheumatoid arthritis, gluten intolerance, and IBS?

    <p>Systemic Lupus Erythematosus</p> Signup and view all the answers

    What is the characteristic symptom that defines Sjogren's Syndrome?

    <p>Severe dryness due to inflammation and damage to lacrimal and salivary glands</p> Signup and view all the answers

    Which autoimmune disorder involves extra-glandular involvement of the joints, skin, lungs, GI, nervous system, and/or kidneys in approximately one-third of patients?

    <p>Sjogren's Syndrome</p> Signup and view all the answers

    In Hypersensitivity Type III reactions, the accumulation of antigen-antibody complexes can lead to tissue damage through the release of cytokines and chemokines.

    <p>True</p> Signup and view all the answers

    In Hypersensitivity Type II reactions, immune complexes are small/intermediate in size and are easily removed from circulation by mononuclear cells.

    <p>False</p> Signup and view all the answers

    IgG or IgM mediated cytotoxic reactions primarily involve the activation of complement proteins C3a and C5a.

    <p>True</p> Signup and view all the answers

    Serum Sickness Type is primarily caused by a localized inflammatory response typically seen after vaccination.

    <p>False</p> Signup and view all the answers

    Arthus Reaction is an example of Hypersensitivity Type I reaction involving heat intolerance, sweating, fatigue, and weight loss.

    <p>False</p> Signup and view all the answers

    Which immunoglobulin is present in the smallest quantity in the blood?

    <p>IgD</p> Signup and view all the answers

    Which immunoglobulin is primarily involved in immunity through enhancing phagocytosis and neutralizing toxins?

    <p>IgG</p> Signup and view all the answers

    Which immunoglobulin is located on mast cells and basophils and plays a role in allergic reactions?

    <p>IgE</p> Signup and view all the answers

    Which immunoglobulin is present in all body fluids and provides memory immunity?

    <p>IgG</p> Signup and view all the answers

    Which immunoglobulin is involved in the induction of other immune system cells to destroy foreign substances?

    <p>IgM</p> Signup and view all the answers

    Which immunoglobulin is primarily responsible for providing localized protection of mucous membranes against bacteria and viruses?

    <p>IgA</p> Signup and view all the answers

    Which part of the antibody functions as a lock and key, specifically binding to the epitope on the antigen?

    <p>Variable regions</p> Signup and view all the answers

    Which characteristic distinguishes IgA, IgE, IgG, IgM, and IgD from each other?

    <p>Constant region sequence</p> Signup and view all the answers

    Which antibody type has a primary function of providing ab to newborns through breast milk?

    <p>IgA</p> Signup and view all the answers

    Which type of chain makes up the majority of the structure of an antibody?

    <p>Heavy chains</p> Signup and view all the answers

    In Hypersensitivity Type I reactions, what is the primary immunoglobulin involved in the mechanism of anaphylaxis?

    <p>IgE</p> Signup and view all the answers

    Which type of immunity is associated with the transfer of IgG across the placenta to provide protection to the fetus?

    <p>Naturally Acquired Passive Immunity</p> Signup and view all the answers

    Which immunoglobulin is primarily involved in defending against parasites and providing protection in allergic reactions?

    <p>IgA</p> Signup and view all the answers

    Which type of immunity is induced through vaccination but does not involve pathogenic antigens?

    <p>Artificially Acquired Active Immunity</p> Signup and view all the answers

    Which immunoglobulin mediates the release of histamine and inflammatory mediators in Hypersensitivity Type I reactions?

    <p>IgE</p> Signup and view all the answers

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