Immunodeficiency & Autoimmunity

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What percentage of immunodeficiency cases are primary?

50%

10% (correct)

5%

20%

What type of immunodeficiency is characterized by impaired thymus development and very low circulating lymphocytes?

T-cell immunodeficiency disorder (correct)

Severe combined immunodeficiency (SCID)

B-cell immunodeficiency disorder

Autoimmune disorder

What is the primary cause of secondary immunodeficiency?

Genetic defects

Malnutrition

Exposure to various agents (correct)

Lymphoid malignancy

What is the typical age of manifestation for primary immunodeficiency?

Manifest early in life Signup and view all the answers

What is the characteristic of severe combined immunodeficiency (SCID)?

Complete absence of mature recirculating B-cells Signup and view all the answers

What is the result of genetic defects in the development of lymphoid cells?

Severe combined immunodeficiency (SCID) Signup and view all the answers

What type of infections are individuals with severe combined immunodeficiency (SCID) more susceptible to?

Extracellular bacterial infections Signup and view all the answers

What is the characteristic of T-cell immunodeficiency disorders?

Normal circulating RBC number Signup and view all the answers

What is the primary defect in Bruton's agammaglobulinemia?

Defect in B-cell signal transduction due to a defect in Bruton's tyrosine kinase (Btk) Signup and view all the answers

What is the characteristic feature of X-linked agammaglobulinemia in terms of antibody production?

Extremely low IgG levels and absence of other Igs Signup and view all the answers

What is the reason for the decreased response to bacterial infections in X-linked agammaglobulinemia?

Defect in B-cell signal transduction Signup and view all the answers

What is the X-linked inheritance pattern of X-linked agammaglobulinemia due to?

Mutations in the Bruton's tyrosine kinase (Btk) gene Signup and view all the answers

What is the characteristic feature of X-linked hyper-IgM syndrome?

Elevated IgM levels and decreased levels of other Igs Signup and view all the answers

What is the primary function of CD40-CD40L interaction?

Class switch and formation of memory B-cells Signup and view all the answers

What is the defect in X-linked hyper-IgM syndrome?

Defect in CD40 ligand expression Signup and view all the answers

At what age do symptoms of X-linked agammaglobulinemia typically appear?

At 6 months of age Signup and view all the answers

Which type of adhesion does impaired leukocyte rolling lead to?

Type II Signup and view all the answers

What is the primary cause of death in patients with impaired leukocyte rolling and adhesion?

Infections Signup and view all the answers

Which autoimmune disorder is characterized by the generation of auto-antibodies and sensitized Th1 cells specific for thyroid antigens?

Hashimoto's thyroiditis Signup and view all the answers

What is the primary effect of autoantibodies in autoimmune hemolytic anemia?

Both A and B Signup and view all the answers

Which cytokines are involved in the cell-mediated DTH response in insulin-dependent DM?

IL-1, TNFα, and IFNγ Signup and view all the answers

What is the outcome of the destruction of beta cells in insulin-dependent DM?

Decreased insulin production Signup and view all the answers

Which type of autoimmune disorder is characterized by the formation of autoantibodies targeting the intrinsic factor?

Pernicious anemia Signup and view all the answers

What is the primary effect of CTLs in insulin-dependent DM?

All of the above Signup and view all the answers

What is the mechanism of drug-induced anemia?

Complement mediated RBC lysis Signup and view all the answers

What is the effect of autoantibodies in Grave's disease?

Mimicry of TSH action Signup and view all the answers

What is the target of autoantibodies in Goodpasture's syndrome?

Basement membrane antigens of the kidney and lung Signup and view all the answers

What is the characteristic symptom of myasthenia gravis?

Progressive weakness of the skeletal muscle Signup and view all the answers

What is the demographic characteristic of systemic lupus erythematosus (SLE)?

Typically appears in young women Signup and view all the answers

What is the mechanism of tissue destruction in Goodpasture's syndrome?

Complement activation Signup and view all the answers

What is the effect of autoantibodies on acetylcholine receptors in myasthenia gravis?

Blocking of the normal binding of Ach Signup and view all the answers

What is the common characteristic of autoimmune disorders?

Production of antibodies against self-antigens Signup and view all the answers

What is the result of autoantibodies against various tissue antigens?

Hemolytic anemia, thrombocytopenia, and kidney dysfunction Signup and view all the answers

What is the role of auto-reactive T-cells in multiple sclerosis?

Participating in the formation of inflammatory response Signup and view all the answers

What are rheumatoid factors?

Autoantibodies against IgG Signup and view all the answers

What is the result of IgM-IgG complex deposition in the joints?

Complement activation and chronic inflammation Signup and view all the answers

What are the common symptoms of multiple sclerosis?

Numbness in the limbs or loss of vision Signup and view all the answers

What is the mechanism of autoantibody-mediated disease?

Production of autoantibodies against tissue antigens Signup and view all the answers

What is the result of complement activation in autoimmune diseases?

Chronic inflammation and tissue damage Signup and view all the answers

What is the characteristic of rheumatoid arthritis?

Formation of immune complexes in the joints Signup and view all the answers

What is the result of demyelination of nerve fibers?

Neurological dysfunction and paralysis Signup and view all the answers