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23 General Pathology > 8/10 - Diseases of Immunity > Flashcards

8/10 - Diseases of Immunity Flashcards

1
Q

cause of systemic sclerosis

A

unkown

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2
Q

what does systemic sclerosis likely result from

A

interrelated processes:
1. autoimmne responses
2. vascular damage
3. collagen deposition

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3
Q

how does autoimmunity in systemic sclerosis work

A

CD4+ T cells respond to unknown antigen, accumulate in skin and release cytokines that activate inflammatory cells and fibroblasts

cytokines stimulate transcription of genes encoding collagen and other extracellular matrix proteins

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4
Q

describe vascular damage of pt with systemic sclerosis

A

microvascular disease could be present early on resulting from chronic inflammation

widespread narrowing of microvascular leads to ischemic injury and scarring

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5
Q

describe fibrosis in systemic sclerosis

A

fibroblasts of pt have intrinsic abnormality to produce excess collagen

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6
Q

sex and age of ppl with systemic sclerosis

A

women (3-5 times more likely than men)
50-60 years

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7
Q

distinctive feature of systemic sclerosis

A

striking skin changes
- insidious onset w/ cutaneous changes

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8
Q

clinical features of systemic sclerosis/scleroderma

A
  1. Raynaud phenomenon
  2. dysphagia
  3. respiratory difficulty
  4. myocardial fibrosis
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9
Q

what clinical feature of systemic sclerosis is seen in virtually all patients

A

Raynaud phenomenon

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10
Q

what is manifested as numbness and tingling of fibers and toes caused by episodic vasoconstriction of arteries and arterioles

A

Raynaud phenomenon

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11
Q

what is atrributed to esophageal fibrosis and its resultant hypomobility is present in more than 50% of pt w/ scleroderma

A

dysphagia

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12
Q

what does dysphagia in ssystemic sclerosis lead to

A

destruction of esophageal wall leading to atony and dilation

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13
Q

___ is caused by pulmonary fibrosis and may result in right-sided cardiac dysfunction of 40-50% of systemic sclerosis pt

A

respiratory difficulty

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14
Q

___ my cause cardiac failure in 40-50% of systemic scerosis pt

A

myocardial fibrosis

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15
Q

what is often the first sign of systemic scerosis

A

raynaud phenomenon

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16
Q

what triggers raynaud phenomenon

A

vasoconstrictive event:
1. emotional distress
2. exposure to cold

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17
Q

T/F: Raynaud phenomenon is specific to systemic sclerosis

A

FALSE! it is NOT SPECIFIC and can be seen in healthy individuals and other autoimmune disease

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18
Q

what is the color sequence of Raynaud phenomenon

A

white -> blue -> red

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19
Q

skin of systemic sclerosis

A

develops diffuse and hard texture and its surface is usually smooth

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20
Q

facial skin of systemic sclerosis

A

subcutaneous collagen deposition results in characteristic smooth, taut, masktlike facies; nasal alae becomes atrophied

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21
Q

hands of pt with systemic sclerosis

A

fingers fixed in claw-like position w/ resoprtion of terminal phalanges (acro osteolysis)

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22
Q

internal organ fibrosis of pt w/ systemic sclerosis

A

(and or vascular damage) involving lung, heart, GI tract, and or kidneys

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23
Q

what is present on the face of systemic sclerosis pt in the atrophic phase of disease

A

telangiectasias

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24
Q

what is present on the face of systemic sclerosis pt in later stage of disease

A

radial furrowing around the mouth

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25
Q

oral manifestation of pt with systemic sclerosis

A
  1. microstomia
  2. purse string mouth
  3. loss of attached gingival mucosa and gingival recession
  4. ! diffuse widerning of PDL
  5. resorption of posterior ramus of mandible, coronoid process, chin, and condyle
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26
Q

what develops as result of deposition of collagen in perioral tissues of 70% of patients in systemic sclerosis pt

A

microstomia

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27
Q

! diffuse widening of PDL space is often throughout dentition of what disease

A

systemic sclerosis

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28
Q

resorption of posterior ramus of mandible, coronoid process, chin, and condyle are detected in what percent of pt with systemic sclerosis

A

10-20%

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29
Q

limited cutaneous systemic sclerosis was previously referred to as what?

A

CREST syndrome

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30
Q

what does CREST stand for

A

C = calcinosis
R = raynaud phenomenon
E = esophageal dysmotility
S = sclerodactyly
T = telangiectasis

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31
Q

T/F: virtually all patients react to variety of nuclear antigens (ANAs)

A

trUE

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32
Q

2 ANA strongly associated w/ systemic sclerosis? what percent of patients?

A
  1. anti-scl 70 - directed against topoisomerase I - 10-20%
  2. anticentromere antibody - 20-30%
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33
Q

treatment for systemic sclerosis pt

A

difficult
1. penicillamine (systemic med) - inhibits collagen production
2. controlling symptoms
3. dental strategies = collapsible dentures

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34
Q

how can immunodeficiency diseases be divided

A
  1. primary/congenital
  2. secondary/acquired
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35
Q

what immunodeficiency is genetically determined

A

primary/congenital

36
Q

what immunodeficiency may arise as complications of cancers, infections, malnutrition, or side effects of immunosuppression, irradiation, or chemo for cancer and other diseases

A

secondary/acquired

37
Q

clinical manifestations of immunodeficiency diseases

A

increased infections

38
Q

primary immunodeficiency diseases are caused by genetic defects that affect what

A
  1. innate immunity (phagocytes, NK cells, or complement)
  2. acquired immunity (B and T cells)
39
Q

when are most primary immunodeficiency diseases detected

A

infancy between 6 months and 2 years of life

40
Q

what disease:

characterized by failure of B cell precursors to develop into mature B cells

A

X-linked (bruno) agammaglobulinemia

41
Q

what inheritance is bruno agammaglobulinemia

42
Q

X-linked (bruno) agammaglobulinemia is almost always seen in what sex? what age is it apparent

A

seen in males; apparent at about 6 months

43
Q

what is not synthesized in X-linked (bruno) agammaglobulinemia resulting in absence of complete immunoglobulins

A

light chains

44
Q

what are recurrent bacterial infections in X-linked (bruno) agammaglobulinemia

A
  • H. influenzae
  • S. pneumoniae
  • S. aureus
45
Q

what is a common immunodeficiency caused by impaired differentiation of naive B cell to IgA-producing plasma cells

A

isolated IgA deficiency

46
Q

what pt have extremely low levels of both serum and secretory IgA and most are asymptomatic

A

isolated IgA deficiency

47
Q

B) IgA is a major antibody where

A

mucosal secretion

48
Q

B) mucosal defenses are weakened and infections occur in the respiratory, GI, and urogenital tracts in what disease

A

isolated IgA deficiency

49
Q

symptomatic patients present w/ recurrent sinopulmonary infections and diarrhea

A

isolated IgA deficiency

50
Q

thymic hypoplasia is also called what

A

digeorge syndrom

51
Q

what is a T-cell deficiency that results from failure of development of thymus

A

thymic hypoplasia/digeorge syndrome

52
Q

what disease has low number of T cells in blood and lymphoid tissue and poor defense against certain viral and fungal infections

A

thymic hypoplasia/digeorge syndrome

53
Q

90% of cases of ___ caused by small germline deletion that maps to chromosome 22q11

A

thymic hypoplasia/digeorge syndrome

54
Q

what is CATCH 22 syndrome

A

Cardiac defects
Abnormal facial features
Thymic hypoplasia/ T cell abnormality
Cleft palate
Hypocalcemia

55
Q

thymic hypoplasia is part of what syndrome

56
Q

what is encountered in individuals with cancer, diabetes and other metabolic diseases, malnutrition, chronic infections, and in persons receiving chemotherapy or radiation therapy for cancer, or immunosuppressive drugs to prevent GVHD or to treat autoimmune diseases

A

secondary immunodeficiencies

57
Q

what is a virus that attacks the body’s immune system;
if HIV is not treated, it can lead to AIDS (acquired
immunodeficiency syndrome)

A

HIV infection/ AIDS

58
Q

___ is the late stage of ___ infection that
occurs when the body’s immune system is badly
damaged because of the virus

59
Q

B) when does a person with HIV considered to have progressed to AIDS

A
  1. CD4 cells fall below 200 cells per cubic mm of blood
  2. develop one or more opportunistic infections regardless of CD4 count
60
Q

what is the CD4 counts of someone with healthy immune system

A

between 500 and 1,600 cells per cubic mm

61
Q

HIV infection/AIDS is characterized by ___ and is characterized by ___

A

retrovirus human immunodeficiency virus; profound immunosuppression

62
Q

profound immunocuppression in HIV/AIDS leads to what

A
  1. opportunistic infections
  2. secondary neoplasms
  3. neurological (CNS) manifestations
63
Q

what is the 2nd leading cause of death in men between 25-44 years

A

HIV infection/AIDS

64
Q

what is the 3rd leading cause of death in women between 25-44 years

A

HIV infection/AIDS

65
Q

B) what enzymes does a retrovirus use to convert its RNA into DNA

A

reverse transcriptase

66
Q

what allows the retrovirus to replicate

A

retrovirus integrates its viral DNA into the DNA of the host cell

67
Q

what groups of adults are high risk of developing AIDS

A
  1. men who have sex with men
  2. heterosexual transmission
  3. IV drug users
  4. hemophiliacs
  5. recipients of blood and blood components infected with HIV
68
Q

three major routes of HIV transmission

A
  1. sexual contact
  2. parenteral inoculation (inject into oneself)
  3. passage of virus from infected mother to newborn
69
Q

HIV human retrovirus belongs to what virus family

A

lentivirus

70
Q

which HIV is most common in USA, Europe, and Central Africa? which was is principally in West Africa and India?

A

HIV-1; HIV-2

71
Q

what is the structure of HIV-1 virion

A
  1. spherical in shape
  2. electron dense cone shaped core containing RNA
  3. surrounded by lipid
72
Q

how does HIV/AIDS enter into cells

A

requires CD4 and coreceptors; main cellular targets are CD4+ helper cells, macrophages, and dendritic cells

73
Q

how does viral replication occur in HIV/AIDS

A

virus genome integrates into host cell DNA

74
Q

mechanisms of immune deficiency in HIV/AIDS

A
  1. loss of CD4+T cells
  2. defective macrophage and dentritic cell functions
  3. destruction of architecture of lymphoid tissue
75
Q

are patients with AIDS able to mount an antibody response to a new antigen

76
Q

how does HIV/AIDS progress

A
  1. acute infection of mucosal T cells and dendritic cells
  2. viremia and dissemination of virus
  3. latent infection of cells in lymphoid tissue
  4. continuing viral replication and progressive loss of CD4+ T cells
77
Q

what are the different stages of untreated HIV infection

A

acute phase -> asymptotic phase -> AIDS

78
Q

clinical manifestations of HIV

A
  1. opportunistic infections
  2. tumors (B cell lymphoma)
  3. CNS abnormalities
79
Q

what is a disorder characterized by extracellular deposition of misfolded proteins that aggregate in form of insoluble fibrils

A

amyloidosis

80
Q

deposition of proteins in amyloidosis may result from what

A
  1. excessive production of proteins that are prone to misfold and aggregate
  2. mutations
  3. defective or incomplete proteolytic degradation
81
Q

is amyloidosis localized or systemic

82
Q

amyloidosis is seen with a variety of what primary disorders

A
  1. monoclonal B cell proliferation (multiple myeloma)
  2. chronic inflammatory diseases (RA)
  3. alzheimers
  4. familial conditions
  5. amyloid associated w/ dialysis
83
Q

amyloid deposits can cause ___ and ___ by causing pressure on cells and tissues

A

tissue injury, impair normal function

84
Q

does amyloidosis invoke an inflammatory response

85
Q

where does oral amyloidosis occur

A

macroglossia = in tongue

23 General Pathology (24 decks)

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