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Late semester 2 exams > Immunodeficiencies / Autoimmune processes > Flashcards

Immunodeficiencies / Autoimmune processes Flashcards

1
Q

Asplenia

A

Encapsulated extracellular bacteria (not removed from blood)

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2
Q

C3 deficiency

A

Recurrent G-, encapsulated bacterial infection (for all alternative complement pathway disruptions)

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3
Q

Asplenia

A

Encapsulated extracellular bacteria

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4
Q

C3 deficiency

A

Recurrent G- bacterial infection

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5
Q

Factor I deficiency

A

Absence of factor I –> depletion of C3

Encapsulated bacteria

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6
Q

Hereditary angioneurotic edema

A

Deficiency of C1 inhibitors –> depletion of C4, C2

Recurrent episodes of edema skin, larynx, GI, & UG tracts

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7
Q

Paroxysmal nocturnal hemoglobinuria

A

Deficient expression of DAF & CD59 –> intravascular hemolysis
(X-linked)

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8
Q

Autoimmune hemolytic anemia

A

Rh blood group antigens, I antigen; Destruction of RBCs via complement, phagocytosis –> anemia (2)

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9
Q

Autoimmune thrombocytopenia purpura

A

Platelet integrins gpIIb:IIIa –> abnormal bleeding (2)

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10
Q

Goodpasture’s syndrome

A

Basement membrane, collagen type IV –> Glomerulonephritis, pulmonary hemorrhage (2)

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11
Q

Pemphigus vulgaris

A

Epidermal cadherin –> Skin blisters (2)

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12
Q

Pemphigus foliaceus

A

Desmoglein –> mild skin blistering (2)

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13
Q

Acute rheumatic fever

A

Group A Streptococcal cell wall antigens (Abs cross react with endocardium) –> Arthritis, myocarditis, heart valve scarring (2)

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14
Q

Grave’s disease

A

TSH receptor (agonist) –> hyperthyroidism (2); IgGs may be transmitted to fetus through placenta

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15
Q

Myasthenia gravis

A

ACh receptors –> Progressive weakness (2)

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16
Q

T2 diabetes

A

Insulin receptor (antagonist) –> hyperglycemia, ketoacidosis (2)

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17
Q

Hypoglycemia

A

Insulin receptor (agonist) –> Hypoglycemia (2)

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18
Q

Subacute bacterial endocarditis

A

Bacterial antigens –> soluble immune complexes –> glomerulonephritis (3)

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19
Q

Cryoglobulinemia

A

RF IgG complexes –> Vasculitis (3)

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20
Q

Systemic lupus erythemaosus

A

DNA, histones, ribosomes –> Glomerulonephritis, vasculitis, arthritis (3)

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21
Q

T1 diabetes

A

Pancreatic beta-cell Ag –> beta-cell destruction (4)

Coxsackie A&B virus may trigger

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22
Q

RA

A

Unknown synovial joint Ag –> Joint inflammation, destruction (4)

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23
Q

MS

A

Myelin basic protein –> Brain degeneration, paralysis (4)

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24
Q

APECED (Autoimmune PolyEndocrinology Candidiasis Ectodermal Dystrophy)

A

Mutations in AIRE (AutoImmune REgulator) –> defective thymic deletion of autoreactive T cells (TECs can’t express peripheral Ags) –> wide ranging autoimmune rxns

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25
Q

IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked syndrome)

A

Mutations in transcription regulator of FOXP3 –> Treg cells aren’t produced –> High, autoreactive B, T IRs

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26
Q

HLA allotypes:

B27, A29, DQ6

A

Associated w/:
B27 - Ankylosing spondylitis
A29 - Birdshot chorioretinopathy
DQ6 - Narcolepsy, MS, (diminished risk of T1 diabetes)

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27
Q

Ciliac’s

A

Wheat flour gluten modifies tissue transglutaminase –> Anti-transglutaminase CD4+/B cells

28
Q

Borrelia burgdorferi

A

Associated with chronic autoimmune arthritis

29
Q

Campylobacter jejuni

A

Associated with reactive arthritis, GBS

30
Q

Chlamydia trachomatis

A

Associated with Reiter’s syndrome (arthritis)

31
Q

Hashimoto’s thyroiditis

A

Hypothyroidism, autoimmune destruction, antagonists of TSH receptors

32
Q

Crohn’s disease

A

Abnormal mucosal T cell regulation –> Granulomatous rxn

33
Q

Pernicious anemia

A

Anti-parietal cell/anti-intrinsic factor Abs –> impaired vit B12 absorption from lack of intrinsic factor in gastric secretions

34
Q

Autoimmune chronic active hepatitis

A

Liver cells express MHC II –> Anti-liver cell Abs

35
Q

Deficiencies of any of C5-9

A

Lack of complement-mediated lysis –> Neisseria susceptibility

36
Q

NEMO deficiency

A

Impaired NFkB activation –> Chronic bacterial/viral infections

37
Q

Chronic granulomatous disease

A

Defective NADPH oxidase in neutrophils –> Chronic bacterial/fungal infections

38
Q

MBL (mannose-binding lectin) deficiency

A

Neisseria meningitidis –> Meningitis

39
Q

NK-cell deficiency

A

GATA2 gene impaired –> No NK cells –> Severe herpesvirus infections

40
Q

Hyper-IgM deficiency

A

CD40/CD40L impaired –> No isotype switching/somatic hypermutation in B cells –> Extracellular bacterial/fungal infections

41
Q

IgG2 deficiency

A

Susceptible to encapsulated bacteria

42
Q

SCID

A

Various genes affected (ADA, PNP, common gamma chain in IL receptors, repair mechs.) –> B & T cell fxn absent –> All infxns

43
Q

Omenn syndrome

A

RAG1/2 –> Impaired VDJ recombination –> Can’t form TCRs/BCRs properly –> All infxns

44
Q

X-linked agammaglobulinemia

A

BTK doesn’t signal –> B cells blocked at pro-B stage –> Recurrent bacterial infxns

45
Q

Complete DiGeorge syndrome

A

Absence of thymus, T cells –> All infxns

46
Q

ZAP-70 deficiency

A

T cells cannot signal through TCRs –> All infxns

47
Q

Selective IgA deficiency

A

No major susceptibility, most common immunodeficiency

48
Q

DiGeorge’s syndrome

A

Thymic aplasia –> General infection susceptibility

49
Q

MHC I deficiency

A

No CD8+ T cells –> Intracellular infection susceptibility

50
Q

MHC II deficiency

A

No CD4+ T cells –> SCID

51
Q

Graft-Vs-Host disease

A

Donor T cells in bone marrow grafts attack tissues of IC’d hosts –> allogenic IR, inflammatory state; Immunosuppressive drugs, deplete grafts of T cells

52
Q

Interferon-G deficiency

A

Macrophages do not activate –> No granulomas, susceptible to intracellular pathogens

53
Q

Serum sickness

A

Introduced proteins or drugs induce formation of Abs targeting them, esp. w/multiple exposures –> immune complexes form (Arthus rxn)

54
Q

Atopic dermatitis

A

Eczema associated w/Type I hypersensitivity, common in young patients

55
Q

Adenosine deaminase deficiency

A

Decreased DNA synthesis –> Low T & B cell counts –> SCID

56
Q

Selective IgA deficiency

A

Slight susceptibility to mucosal pathogens, most common immunodeficiency

57
Q

Adenosine deaminase deficiency

A

Decreased DNA synthesis –> Low T & B cell counts –> SCID; Need bone marrow transplant

58
Q

Leukocyte adhesion deficiency

A

Deficient integrin B2/CD18/LFA-1/CR3 in neutrophils –> Defective migration to inflammation sites –> Chronic bacterial infxns

59
Q

Toxic shock syndrome

A

Superantigen TSST from Staph, Strep –> Polyclonal CD4+ activation (~20% of total) –> Cytokine storm –> Organ failure

60
Q

Leukocyte adhesion deficiency

A

Deficient integrin B2/CD18/LFA-1/CR3 in neutrophils/macrophages –> Defective migration to inflammation sites –> Chronic bacterial infxns

61
Q

Toxic shock syndrome

A

Superantigen TSST from Staph, Strep –> Polyclonal CD4+ activation (~20% of total) –> Cytokine storm –> Organ failure

62
Q

G6PD & Myeloperoxidase deficiency

A

Defective phagocytes can’t kill pathogens –> Chronic bacterial/fungal infxns

63
Q

Chediak-Higashi syndrome

A

Lysosoal trafficking regulator protein defective –> Defective phagocytosis/phagolysosome formation –> Persistent bacterial infxns, granulomas

64
Q

HIV

A

Depletion of CD4+ T cells –> IC

Opp. infxns include Toxoplasma, Mycobacterium, Cryptococcus, Pneumocystis, Candida, Herpesviruses

65
Q

Radiation, chemotherapy, & metastases to bone marrow

A

Inhibition of leukocyte developmet –> IC

Late semester 2 exams (2 decks)

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