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 (C)</p> Signup and view all the answers

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

<p>IgE (D)</p> Signup and view all the answers

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

<p>Arthus Reaction (C)</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 (B)</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 (B)</p> Signup and view all the answers

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

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

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

<p>Deposition of immune complexes (B)</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 (A)</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 (A)</p> Signup and view all the answers

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

<p>Anaphylaxis (C)</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 (C)</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 (C)</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 (A)</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 (D)</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 (C)</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 (D)</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 (A)</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 (C)</p> Signup and view all the answers

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

<p>African Americans (B)</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 (D)</p> Signup and view all the answers

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

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

Which laboratory finding is typically positive in systemic lupus erythematosus?

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

What is the most common cause of hyperthyroidism?

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

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

<p>Pernicious Anemia (D)</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 (A)</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 (C)</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 (D)</p> Signup and view all the answers

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

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

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

<p>Grave's Disease (B)</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 (C)</p> Signup and view all the answers

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

<p>Takayasu Arteritis (D)</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 (B)</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 (A)</p> Signup and view all the answers

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

<p>Wiskott-Aldrich Syndrome (D)</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 (C)</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 (B)</p> Signup and view all the answers

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

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

What is the hallmark feature of Wiskott-Aldrich Syndrome?

<p>Deficiency in platelet function (A)</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 (D)</p> Signup and view all the answers

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

<p>ELISA test (D)</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 (A)</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 (B)</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 (A)</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 (A)</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 (B)</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 (B)</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% (C)</p> Signup and view all the answers

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

<p>Systemic Lupus Erythematosus (D)</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 (A)</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 (D)</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 (B)</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 (B)</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 (D)</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 (C)</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 (C)</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 (D)</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 (B)</p> Signup and view all the answers

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

<p>African Americans (C)</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 (B)</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 (D)</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 (B)</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 (A)</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 (B)</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 (A)</p> Signup and view all the answers

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

<p>False (B)</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 (B)</p> Signup and view all the answers

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

<p>IgD (A)</p> Signup and view all the answers

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

<p>IgG (A)</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 (C)</p> Signup and view all the answers

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

<p>IgG (C)</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 (C)</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 (A)</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 (A)</p> Signup and view all the answers

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

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

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

<p>IgA (C)</p> Signup and view all the answers

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

<p>Heavy chains (D)</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 (C)</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 (C)</p> Signup and view all the answers

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

<p>IgA (B)</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 (D)</p> Signup and view all the answers

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

<p>IgE (B)</p> Signup and view all the answers

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