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

8/10 - Diseases of Immunity Flashcards

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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
oral manifestation of pt with systemic sclerosis
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
26
what develops as result of deposition of collagen in perioral tissues of 70% of patients in systemic sclerosis pt
microstomia
27
! diffuse widening of PDL space is often throughout dentition of what disease
systemic sclerosis
28
resorption of posterior ramus of mandible, coronoid process, chin, and condyle are detected in what percent of pt with systemic sclerosis
10-20%
29
limited cutaneous systemic sclerosis was previously referred to as what?
CREST syndrome
30
what does CREST stand for
C = calcinosis R = raynaud phenomenon E = esophageal dysmotility S = sclerodactyly T = telangiectasis
31
T/F: virtually all patients react to variety of nuclear antigens (ANAs)
trUE
32
2 ANA strongly associated w/ systemic sclerosis? what percent of patients?
1. anti-scl 70 - directed against topoisomerase I - 10-20% 2. anticentromere antibody - 20-30%
33
treatment for systemic sclerosis pt
difficult 1. penicillamine (systemic med) - inhibits collagen production 2. controlling symptoms 3. dental strategies = collapsible dentures
34
how can immunodeficiency diseases be divided
1. primary/congenital 2. secondary/acquired
35
what immunodeficiency is genetically determined
primary/congenital
36
what immunodeficiency may arise as complications of cancers, infections, malnutrition, or side effects of immunosuppression, irradiation, or chemo for cancer and other diseases
secondary/acquired
37
clinical manifestations of immunodeficiency diseases
increased infections
38
primary immunodeficiency diseases are caused by genetic defects that affect what
1. innate immunity (phagocytes, NK cells, or complement) 2. acquired immunity (B and T cells)
39
when are most primary immunodeficiency diseases detected
infancy between 6 months and 2 years of life
40
what disease: characterized by failure of B cell precursors to develop into mature B cells
X-linked (bruno) agammaglobulinemia
41
what inheritance is bruno agammaglobulinemia
x-linked
42
X-linked (bruno) agammaglobulinemia is almost always seen in what sex? what age is it apparent
seen in males; apparent at about 6 months
43
what is not synthesized in X-linked (bruno) agammaglobulinemia resulting in absence of complete immunoglobulins
light chains
44
what are recurrent bacterial infections in X-linked (bruno) agammaglobulinemia
- H. influenzae - S. pneumoniae - S. aureus
45
what is a common immunodeficiency caused by impaired differentiation of naive B cell to IgA-producing plasma cells
isolated IgA deficiency
46
what pt have extremely low levels of both serum and secretory IgA and most are asymptomatic
isolated IgA deficiency
47
B) IgA is a major antibody where
mucosal secretion
48
B) mucosal defenses are weakened and infections occur in the respiratory, GI, and urogenital tracts in what disease
isolated IgA deficiency
49
symptomatic patients present w/ recurrent sinopulmonary infections and diarrhea
isolated IgA deficiency
50
thymic hypoplasia is also called what
digeorge syndrom
51
what is a T-cell deficiency that results from failure of development of thymus
thymic hypoplasia/digeorge syndrome
52
what disease has low number of T cells in blood and lymphoid tissue and poor defense against certain viral and fungal infections
thymic hypoplasia/digeorge syndrome
53
90% of cases of ___ caused by small germline deletion that maps to chromosome 22q11
thymic hypoplasia/digeorge syndrome
54
what is CATCH 22 syndrome
Cardiac defects Abnormal facial features Thymic hypoplasia/ T cell abnormality Cleft palate Hypocalcemia
55
thymic hypoplasia is part of what syndrome
CATCH 22
56
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
secondary immunodeficiencies
57
what is a virus that attacks the body's immune system; if HIV is not treated, it can lead to AIDS (acquired immunodeficiency syndrome)
HIV infection/ AIDS
58
___ is the late stage of ___ infection that occurs when the body's immune system is badly damaged because of the virus
AIDS; HIV
59
B) when does a person with HIV considered to have progressed to AIDS
1. CD4 cells fall below 200 cells per cubic mm of blood 2. develop one or more opportunistic infections regardless of CD4 count
60
what is the CD4 counts of someone with healthy immune system
between 500 and 1,600 cells per cubic mm
61
HIV infection/AIDS is characterized by ___ and is characterized by ___
retrovirus human immunodeficiency virus; profound immunosuppression
62
profound immunocuppression in HIV/AIDS leads to what
1. opportunistic infections 2. secondary neoplasms 3. neurological (CNS) manifestations
63
what is the 2nd leading cause of death in men between 25-44 years
HIV infection/AIDS
64
what is the 3rd leading cause of death in women between 25-44 years
HIV infection/AIDS
65
B) what enzymes does a retrovirus use to convert its RNA into DNA
reverse transcriptase
66
what allows the retrovirus to replicate
retrovirus integrates its viral DNA into the DNA of the host cell
67
what groups of adults are high risk of developing AIDS
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
three major routes of HIV transmission
1. sexual contact 2. parenteral inoculation (inject into oneself) 3. passage of virus from infected mother to newborn
69
HIV human retrovirus belongs to what virus family
lentivirus
70
which HIV is most common in USA, Europe, and Central Africa? which was is principally in West Africa and India?
HIV-1; HIV-2
71
what is the structure of HIV-1 virion
1. spherical in shape 2. electron dense cone shaped core containing RNA 3. surrounded by lipid
72
how does HIV/AIDS enter into cells
requires CD4 and coreceptors; main cellular targets are CD4+ helper cells, macrophages, and dendritic cells
73
how does viral replication occur in HIV/AIDS
virus genome integrates into host cell DNA
74
mechanisms of immune deficiency in HIV/AIDS
1. loss of CD4+T cells 2. defective macrophage and dentritic cell functions 3. destruction of architecture of lymphoid tissue
75
are patients with AIDS able to mount an antibody response to a new antigen
NO
76
how does HIV/AIDS progress
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
what are the different stages of untreated HIV infection
acute phase -> asymptotic phase -> AIDS
78
clinical manifestations of HIV
1. opportunistic infections 2. tumors (B cell lymphoma) 3. CNS abnormalities
79
what is a disorder characterized by extracellular deposition of misfolded proteins that aggregate in form of insoluble fibrils
amyloidosis
80
deposition of proteins in amyloidosis may result from what
1. excessive production of proteins that are prone to misfold and aggregate 2. mutations 3. defective or incomplete proteolytic degradation
81
is amyloidosis localized or systemic
both
82
amyloidosis is seen with a variety of what primary disorders
1. monoclonal B cell proliferation (multiple myeloma) 2. chronic inflammatory diseases (RA) 3. alzheimers 4. familial conditions 5. amyloid associated w/ dialysis
83
amyloid deposits can cause ___ and ___ by causing pressure on cells and tissues
tissue injury, impair normal function
84
does amyloidosis invoke an inflammatory response
NO
85
where does oral amyloidosis occur
macroglossia = in tongue

23 General Pathology (24 decks)

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