8/8 - Diseases of Immunity Flashcards

1
Q

what disease has swan-neck and boutonniere deformities

A

rheumatoid arthritis

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2
Q

what is a chronic autoimmune disorder that principally attacks the joints, producing nonsuppurative prolierative and inflammatory synovitis

A

rhematoid arthritis

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3
Q

RA often progresses to ___ and in some cases ___

A

articular cartilage destruction; joint fusion (ankylosis)

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4
Q

extraarticulator lesions of ___ may involve skin, BV, lungs and heart leading to clinical manifestations that overlap w/ other autoimmune disorders including SLE and scleroderma

A

rhematoid artheritis

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5
Q

prevalence of RA in US

A

1% in US population

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6
Q

age of people affected by RA

A

2nd to 4th decades

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7
Q

what sex predominantly affects RA

A

women

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8
Q

what disease principally attacks joints; extraarticular lesions may involve skin, heart, BV and lungs

A

RA

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9
Q

what contributes to development, progression, and persistence of disease of RA

A
  1. genetic predisposition
  2. environmental factors
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10
Q

autoimmune response of RA is initiated by what

A

CD4+ helper T cells

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11
Q

pathologic changes of RA are mediated by what

A

antibodies against self antibodies against self antigens and inflammation induces cytokines, predoimnantly induced CD4+ t cells

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12
Q

activated ___ release inflammatory mediators that stimulate inflammatory cells leading to tissue injury in RA

A

CD4+ T cells

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13
Q

___ and ___ from ___ stimulate resident synovial cells to secrete proteases and destroy hyaline cartilage

A

TNF, IL-1, macrophages

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14
Q

RA begins with what symptoms

A

malaise, fatigue, and generalized musculoskeletal pain

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15
Q

joint involvement of RA begins when

A

after weeks to months - general symmetrical and effects small joints before larger ones

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16
Q

described joints involved in RA

A

swollen, warm, and painful

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17
Q

B)

which arthritis is better with movement? which arthritis doesn’t get better with movement?

A

osteoarthritis = movement is better
rheumatoid arthritis = movement doesn’t get better

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18
Q

what produces radial deviation of the wrist, ulnar deviation of fingers and flexion-hyperextension of the fingers

A

RA

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19
Q

in RA, what becomes edematous, thickened, and hyperplastic transforming its smooth contour to one covered by delicate and bulbous villi

A

synovium

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20
Q

characteristic features of RA tissue injury

A
  1. synovial cell: hyperplasia and proliferation
  2. dense inflammatory infiltrate
  3. increased vascularity
  4. fibrinopurulent exudate
  5. osteoclastic activity in subchondral bone
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21
Q

buzz word PANNUS = ___

A

RA

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22
Q

how does pannus cause RA

A

grows over synovium an causes erosion of articular cartilage. pannus then bridges opposing bones to form fibrous ankylosis which ossifies, resulting in bone fusion or bony ankylosis)

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23
Q

80% of patients have which antibodies in RA? what are these autoantibodies called?

A

IgM or IgA reacting against self-IgG = called rheumatoid factor (RF)

24
Q

! what are diagnostic markers that can be detected in serum of up to 70% of RA patients

A

anti-citrullinated peptide antibodies (ACPAs)

25
Q

RA tx

A

aimed at relieving pain and inflammation and slowing and arresting joint destruction

26
Q

RA therapies

A
  1. corticosteroids
  2. immunosuppressants (methotrexate, TNF antagnoists)
27
Q

what disease:
- dry eyes damage to eye surface
- dry mouth causes increased tooth decay

A

sjogren syndrome

28
Q

what type is sjogren syndrome

A

type II

29
Q

what is a secondary form of sjogren syndrome

A

dry eyes and mouth PLUS another autoimmune disease

30
Q

the isolated disorder/primary form of Sjogren Syndrome is also called what

A

sicca syndrome

31
Q

what diseases is sjogren syndrome most commonly associated with? what do other patients have?

A

most common: rheumatoid arthritis

other: SLE, scleroderma, vasculitis, and thyroiditis

32
Q

characteristic decrease in tears and saliva in sjogren syndrome results in what

A

lymphocytic infiltration and fibrosis of lacrimal and salivary gladns

33
Q

infiltrate in sjogren syndrome contains what

A

activated CD4+ helper T cells , some B cells including plasma cells

34
Q

what is the initiating trigger of sjogrens syndrome

A

viral infection of salivary glands, which cause local cell death and release of tissue self antigens

35
Q

___% of patients with sjogrens have rhematoid factor whether coexisting RA is present or not

A

75%

36
Q

pathogenesis of sjogrens

A
  1. decreased saliva and tear from inflammatory destruction of exocrine glands
  2. lymphocytic infiltration and fibrosis of lacrimal and salivary glands
37
Q

who does sjogrens most commonly occur in

A

women between 50 and 60, and rarely in chilcdren

38
Q

what produces blurring of vision, burning and itching, and thick scretions

A

keratoconjuncitivitis associated w sjogrens

39
Q

what results in difficulty in swallowing solid food, a decrease in in ability to taste, cracks and fissures in mouth, and dryness of oral mucosa

A

xerostomia associated w sjogrens

40
Q

parotid gland enlargement is seen in what percent of sjogrens patients

A

50%

41
Q

how does saliva appear in sjogrens

A

frothy, with lack of usual pooling saliva in floor of mouth

42
Q

how does tongue appear in sjogren

A

fissured and exhibits atrophy of papillae

43
Q

how does oral mucosa appear in sjogrens

A

red and tender and usually results in secondary candidiasis

44
Q

buzz word “fruit-laden, branchless tree” = ___

A

sjogrens syndrome

45
Q

what disease:

sialo graphic examination often reveals punctate sialectasis and lack of arborization of ductal system

A

sjogrens

46
Q

presence of ___ can be helpful to clue in diagnosis

A

autoantibodies

47
Q

positive rheumatoid factor found in ___ of sjogrens patients, regardless whether the patient has rheumatoid arthritis

A

60%

48
Q

antinuclear antibodies (ANA) are present in ___% of sjogrens patients

A

75-80%

49
Q

antibody SS-A (anti-Ro) and anti SS-B (anti-La) may be found in what patients

A

sjogrens syndrome

50
Q

sjogren syndrome tx

A

most supportive:
- artifical tears
- artificial saliva

51
Q

B)

about 5% of pt with sjogren syndrome develop ___ and incident that is 40-fold greater than normal

A

lymphoma

52
Q

what type is systemic sclerosis/scleroderma

A

type II

53
Q

systemic sclerosis is also called what

A

scleroderma

54
Q

systemic sclerosis is characterized by what

A
  1. chronic inflammation
  2. widespread damage to small BV
  3. progressive interstitial and perivascular fibrosis in skin and multiple organs
55
Q

what is most affected in systemic sclerosis? what else can be affected?

A

most affected: skin
other: GI tract, kidneys, heart, muscle, lungs