Pathophysiology Exam 1: Immunodeficiency and Autoimmune Disease Practice Exam

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• What are the three key enzymes of HIV-1?
• Viremia refers to viruses present in which location?
• What is the resulting immune response of DiGeorge syndrome?
• The HIV-1 genome contains how many copies of ssRNA?
• Which hypersensitivity type is commonly involved in contact dermatitis and positive tuberculin skin tests?
• What does a CH50 test assess?
• Pemphigus vulgaris is caused by autoantibodies against which desmosomal protein?
• Which type of viruses are implicated in AIDS-related tumors?
• What is the time course of seroconversion in the early acute phase of HIV-1 infection?
• Which clinical finding is commonly seen in SLE due to synovitis?
• What is the difference between central and peripheral tolerance?
• What is the hallmark immunologic change in HIV infection?
• Which statement about treatment and prognosis is true for CVID (and often Bruton's)?
• Which intervention is used to remove circulating autoantibodies and immune complexes in autoimmune diseases?
• What are secondary neoplasms?
• Which statement about demographics of SLE is true?
• During which phase are many patients asymptomatic despite infection?
• Which test is more specific for diagnosing rheumatoid arthritis among common autoimmune serologies?
• What is the resulting immune response of CVID?
• Clonal deletion is a central tolerance mechanism. It occurs by what process?
• What are the two major components of the pathogenesis of Sjogren syndrome?
• What happens to viral load during weeks 6-12 of early HIV-1 infection?
• Which statement best describes the effect of HIV infection on macrophage functions relevant to antigen presentation?
• Terminal complement deficiencies increase susceptibility to which infections?
• If a patient is symptomatic during the middle chronic phase, which symptoms might they present with?
• What is the outcome of HIV-1 infection on NK cells?
• Hyper-IgM syndrome results from defective class-switch recombination due to deficiency of which molecule?
• During seroconversion in early HIV infection, what change occurs in CD4+ T cell count during weeks 1-6?
• Which of the following best describes common symptoms of clinical disease during the final crisis phase?
• Kaposi sarcoma is best described as which of the following in AIDS?
• Antiphospholipid syndrome is characterized by antibodies directed against which targets?
• What are the major structural features of HIV-1?
• Which of the following is NOT a major neurologic manifestation in HIV/AIDS?
• Which clinical finding is most characteristic of IgA deficiency?
• Anti-thyroid peroxidase antibodies are most commonly associated with which condition?
• Labs seen in DiGeorge syndrome?
• By approximately how many times is the risk of malignant lymphoma increased in Sjogren syndrome?
• CVID laboratory findings typically include which pattern?
• Which statement best describes the overall three major steps in SLE pathogenesis as per the provided material?
• Which of the following is a typical clinical finding in Bruton's disease?
• What is the concept of molecular mimicry in autoimmunity?
• In pemphigus vulgaris, autoantibodies preferentially target which molecule?
• What is the primary immunopathology of multiple sclerosis?
• In Bruton's disease, are T cells sufficient to provide defense against most viral and fungal infections?
• What is the outcome of HIV-1 infection on dendritic cells?
• What is the function of CD4+CD25+ FOXP3+ regulatory T cells?
• What are the two major causes of autoimmune diseases?
• What treatment may decrease morbidity/mortality in patients with HIV/AIDS?
• Which serologic markers are useful for monitoring SLE activity?
• Cryptococcosis (meningitis) in AIDS patients is characterized by which symptom constellation?
• Which statement best describes the gp120 interaction with host cells during entry?
• Which statement about CVID is accurate based on the provided material?
• AIDS dementia complex leads to which type of encephalopathy?
• What ANA specificity is found in scleroderma?
• What is the standard diagnostic test used to identify chronic granulomatous disease?
• What characterizes the Type III hypersensitivity reaction in SLE?
• How does HIV infection commonly affect the CD4:CD8 T cell ratio?
• Which statement best contrasts Bruton's disease with CVID?
• What is the pathophysiology of immune thrombocytopenia (ITP)?
• What renal pathology is associated with scleroderma renal crisis?
• Which autoantibodies are characteristic of Hashimoto thyroiditis?
• Which immunoglobulin pattern characterizes common variable immunodeficiency?
• What best describes the vascular lesion in diffuse systemic sclerosis?
• Non-Hodgkin's lymphoma in AIDS patients is characterized by which feature?
• Which autoantibody is highly specific for SLE and strongly linked to lupus nephritis?
• In pemphigus vulgaris, autoantibodies against desmoglein disrupt what cellular process?
• What is the role of CD8 cytotoxic T cells in the early acute phase of HIV-1 infection?
• Which description correctly characterizes the CCR5 receptor?
• Where does central tolerance normally occur for T cells and B cells?
• What is the outcome of HIV-1 infection on B cells?
• What is epitope spreading in autoimmune disease?
• In multiple sclerosis, which immune cell type is primarily involved in initiating demyelination?
• HIV-1 capsid protein is also known as which of the following?
• Which statement best describes the T cell status in Bruton's disease?
• What are considered specific autoantibody tests for Sjogren syndrome?
• Renal crisis features in diffuse scleroderma?
• What is the hallmark cellular defect in Bruton disease?
• Complement deficiencies associated with severe infections and autoimmune phenomena commonly involve which components?
• Chronic granulomatous disease is caused by a defect in which enzyme system?
• Sjogren syndrome is associated with other autoimmune diseases in approximately what percentage?
• Which mechanism best describes Graves disease pathogenesis?
• The final crisis phase of HIV infection is defined by progression to what?
• What is the function of reverse transcriptase in HIV replication?
• How should ANA results be interpreted clinically?
• What immunologic change is typically observed after starting antiretroviral therapy in HIV infection?
• What overall description best summarizes diffuse systemic sclerosis pathophysiology?
• Self-tolerance is defined as the lack of immune response to an individual's own tissue antigens.
• SLE tissue injury is largely caused by deposition of immune complexes that trigger which hypersensitivity reactions?
• Which laboratory profile is characteristic of Bruton's disease?
• What happens after gp120 binds to the CD4 receptor on a T helper cell?
• In SLE, which immune components are primarily involved in its pathogenesis?
• Which immune cell type is primarily involved in gland infiltration in Sjogren syndrome?
• Describe finger injury typical of scleroderma.
• Which mechanism is described as a way the body develops self-tolerance?
• Which labs would you expect in IgA deficiency?
• What is the mechanism by which TNF inhibitors treat autoimmune diseases?
• Which statement about ANA testing is correct?
• What is the syndrome of primary HIV infection?
• Which autoantibody is most commonly associated with limited systemic sclerosis (CREST)?
• What is the principal patient population affected by SLE?
• What is the consequence of CCR5 gene deletion?
• What is the major cause of fatality in patients with HIV/AIDS?
• TNF inhibitors in autoimmune disease primarily exert their effect by:
• What is the function of reverse transcriptase?
• Which autoantibody is highly specific for rheumatoid arthritis and predictive of its severity?
• Where does central tolerance occur for B cells?
• What type of infections are infants with DiGeorge syndrome particularly susceptible to due to T cell deficiency?
• In Bruton agammaglobulinemia, which of the following is characteristically decreased?
• Which cells are infected by HIV-1?
• What sequence describes the events after activation of HIV-1 infected T cells?
• Which statement best describes the effect of protease after translation of viral polyproteins?
• Which of the following best describes the typical duration for the middle chronic phase in many patients?
• Diffuse systemic sclerosis commonly involves which of the following?
• Why are asplenic patients particularly at risk for severe infections?
• In common variable immunodeficiency, B cell numbers are typically:
• Which sequence best describes the three major steps in the pathogenesis of SLE?
• CD40 ligand is expressed on which cell type?
• In clonal anergy (peripheral tolerance), what best describes the fate of a naïve CD8 T cell?
• Which statement about IgA deficiency treatment is true?
• What is the defect in IgA deficiency?
• Which autoimmune disease is systemic and involves circulating autoantibodies?
• Which humoral immunodeficiency is most commonly observed as a primary humoral immunodeficiency?
• Which condition is most consistent with increased susceptibility to bacterial infections due to defective phagocyte respiratory burst?
• What are the events of latent infection of lymphoid tissues in the early acute phase of HIV-1 infection?
• Graves disease is driven by autoantibodies that target which component of the thyroid?
• What is the average duration of the middle chronic phase?
• What is the resulting immune response of IgA deficiency?
• Which primary immunodeficiency is associated with thymic aplasia and T cell deficiency?
• In common variable immunodeficiency (CVID), what is the primary B cell defect as described in the material?
• Central tolerance is primarily established during lymphocyte development in which locations?
• Which enzyme is responsible for integrating the viral DNA into the host chromosomal DNA?
• In DiGeorge syndrome, what are the typical immunoglobulin levels?
• Why is there a gradual decline in CD4 T cell count after several years in the middle chronic phase?
• Which organ is primarily responsible for destruction of antibody-coated platelets in ITP?
• Rheumatoid factor, ANAs, and ESR are best described as which type of tests for Sjogren syndrome?
• Bruton Tyrosine Kinase (BTK) mutation causes Bruton's disease. Which protein is mutated?
• Goodpasture syndrome involves autoantibodies against which basement membrane component?
• Which term describes end-stage lung changes in diffuse sclerosis?
• What is the role of gp41 in HIV entry?
• Which statement best describes general properties of HIV-1?
• How are organ-specific autoimmune diseases different from systemic autoimmune diseases?
• Which enzyme is NOT among the three key enzymes of HIV-1?
• What is IVIG and what is its therapeutic use?
• Which set of defects are immunologic defects seen in HIV-1 infection?
• Autoantibodies against which receptor cause myasthenia gravis?
• What is a distinguishing facial feature of scleroderma?
• Autoantibodies found in scleroderma target which components?
• Which description best fits Type IV hypersensitivity?
• What is the immunologic consequence of the BTK mutation in Bruton's disease?
• Which of the following are major functions of the complement system?
• Candidiasis in AIDS patients commonly presents as which of the following?
• Mycobacterium avium complex infection in AIDS patients is commonly diagnosed late and may be asymptomatic; when symptomatic, which combination of symptoms is typical?
• Which type of hypersensitivity is primarily associated with SLE?
• Which autoimmune disease features autoimmune destruction of pancreatic beta cells leading to insulin deficiency?
• Which of the following is a clinical finding in DiGeorge syndrome?
• Which of the following best describes clinical findings associated with CVID?
• Which statement accurately defines primary immunodeficiencies?
• Which of the following are self-antigens implicated in lupus?
• Which autoantibody is highly specific for SLE but less sensitive?
• What is the outcome of HIV-1 infection on macrophages?
• During latent HIV infection, where is HIV cDNA typically found?
• Which treatment is used for DiGeorge syndrome?
• During latent infection of lymphoid tissues, which cell type is not a primary reservoir for HIV?
• What is the primary connective tissue abnormality in diffuse systemic sclerosis?
• Which statement describes skin changes in diffuse systemic sclerosis?
• Which mechanism describes the emergence of sequestered antigens in response to trauma as part of self-tolerance?
• Which function describes HIV integrase?
• What is the fundamental lesion of HIV-1 infection?
• In SLE, deposition of immune complexes in tissues leads to which combination of tissue changes?
• In the middle chronic phase of HIV-1 infection, patients may present as which of the following states?
• What occurs during the final crisis phase?
• What are the major therapeutic approaches used to treat autoimmune diseases?
• Which combination of strategies is commonly used to reduce HIV-related morbidity and mortality?
• Which best describes the effect of antiretroviral therapy on HIV infection?
• Kaposi sarcoma in AIDS patients is best described as which of the following?
• Which enzyme is responsible for processing viral polyproteins during maturation?
• Describe the microvascular injury sequence in scleroderma.
• Which sequence best describes gp120 leading to entry after CD4 binding?
• What is the role of Th17 cells in autoimmunity?
• What is a standard treatment for CVID?
• Which statement correctly describes drugs and hormones that may induce SLE?
• Which standard diagnostic test is used to identify chronic granulomatous disease?
• Infected monocytes are vesicles for transport of the virus to other tissues. How does this affect systemic infection?
• Antiphospholipid syndrome often exhibits lupus anticoagulant activity; what is a typical in vitro and in vivo effect of these antibodies?
• Autoimmunity is defined as which of the following?
• What is the standard treatment for Bruton's disease?
• Which markers define regulatory T cells (for peripheral tolerance)?
• Which encapsulated bacteria are particularly dangerous in asplenia due to impaired opsonization?
• Which of the following is the first major step in the pathogenesis of SLE?
• What are the two surface glycoproteins on HIV-1?
• What immunodeficiency is associated with chronic mucocutaneous candidiasis due to IL-17 pathway defects?
• Which autoantibody is highly specific for rheumatoid arthritis and used diagnostically?
• What screening test is commonly used as an initial screen for autoimmune diseases, including SLE?
• In Sjogren syndrome, which gland is characteristically enlarged due to lymphocytic infiltration?
• Which neoplasm is the second most common cutaneous lesion and neoplasm in AIDS after Kaposi sarcoma?
• Which infection is associated with CVID due to gastrointestinal involvement?
• Which finding is NOT typical of Bruton's disease?
• In IgA deficiency, which of the following is a common lab finding?
• Which statement best describes the function of HIV protease?
• HIV-1 capsid protein is also known as what?
• Cryptococcosis in AIDS patients is described as which of the following?
• What is the defect in DiGeorge syndrome?
• Which therapeutic class includes TNF inhibitors and IL inhibitors used to treat autoimmune diseases?
• Which statement about HIV-1 infection is true?
• What is the role of regulatory T cells (FOXP3+) in peripheral tolerance?
• Which primary immunodeficiency presents with eczema, small platelets, and increased susceptibility to infections?
• Which of the following best describes immunoglobulin levels in DiGeorge syndrome?
• Which complement components are typically measured to assess SLE activity?
• Systemic lupus erythematosus is classically associated with what hypersensitivity mechanism?
• In addition to acetylcholine receptor antibodies, which autoantibody is associated with a subset of myasthenia gravis?
• What effect does UV light have on SLE?
• Which statement about the HIV-1 genome is accurate?
• Clonal deletion (central tolerance) occurs in which location and by what mechanism?
• In rheumatoid arthritis, which autoantibodies are commonly detected and clinically informative?
• What symptoms might present in a patient infected with HIV-1 in the early acute phase?
• In diffuse systemic sclerosis, which immune features are true?
• What are the two principal clinical features of Sjogren syndrome?
• Which organ can be affected in SLE, with examples including pericarditis and valvular endocarditis?
• Which receptor is used for HIV entry into macrophages?
• Which of the following are common environmental triggers for systemic lupus erythematosus (SLE)?
• After CD4 engagement, which coreceptors can gp120 use to enter the cell?
• Lung involvement in diffuse scleroderma includes which features?
• Which organ is commonly affected in SLE, often showing glomerulonephritis?
• How do Th1 and Th17 cytokines contribute to autoimmune tissue injury?
• Which fungal infection is the second most common in AIDS patients?