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MF2 Final study > Immune disorders > Flashcards

Immune disorders Flashcards

1
Q

Crystallized eosinophil granules

A

Charcot-Leyden crystals

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2
Q

This can occur when immune complexes deposit in vessels in type III hypersensitivity

A

Vasculitis
Often produces fibrinoid necrosis of vascular wall (fibrin-like pink material)

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3
Q

This method can be used to see “granular” deposits of immune complexes with the use of labeled antisera against IgG, IgA, IgM, and complement components

A

Direct immunofluorescence

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4
Q

Granuloma formation and apoptotic cells may be seen in this type of hypersensitivity

A

Type IV
from macrophage activation and CTL response

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5
Q

Low levels of this product that is made in the liver cause edema

A

Albumin

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6
Q

Immunoglobulin which precipitates in cold temperatures

A

Cryoglobulin

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7
Q

Cryoglobulinemia vasculitis is highly associated with this

A

Hep C infection

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8
Q

This immune-complex mediated condition is highly associated with Hep C infection

A

Cryoglobulinemia vasculitis

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9
Q

Mixed cryoglobulinemia has both of these types of Ig
These precipitate in tissues and cause damage

A

Polyclonal IgG (against Hep C)
Monoclonal IgM (with Rheumatoid Factor activity)

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10
Q

Macrophages with some giant cells and “loose” granulomas are characteristic of this type of hypersensitivity

A

Type IV

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11
Q

Antibodies directly on cell causing damage, such as those against ABO blood groups, is this type of hypersensitivity

A

Type II Hypersensitivity

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12
Q

Autoimmune disorders occur due to defective this

A

Self tolerance

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13
Q

2 characteristics of autoimmune disorders

A

Inherent susceptibility (family hx, HLA, female more common)
Environmental triggers (molecular mimicry, polyclonal activation, tissue damage)

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14
Q

Tissue damage can lead to exposure of these, which can cause autoimmunity

A

Autoantigens

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15
Q

Inflammation can alter these, leading to autoimmunity

A

Self antigens

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16
Q

Antibodies against various (nonspecific) nuclear antigens
Often used as initial screen test for autoimmune diseases

A

Antinuclear antibodies

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17
Q

Antinuclear antibodies are originally detected on cultured cells (HEp2 usually) by this

A

Indirect immunofluorescence

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18
Q

Antinuclear antibodies are more commonly detected with this testing

A

ELISA/EIA

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19
Q

Antibody (usually IgM) against IgG Fc receptor
May be monoclonal or polyclonal
May result from immune activation of various causes; seen in healthy individuals (greater chance with age) and in many autoimmune diseases

A

Rheumatoid Factor

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20
Q

What is Rheumatoid Factor?

A

Antibody (usually IgM) against IgG Fc receptor

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21
Q

Is Rheumatoid Factor specific?

A

No
Screening for autoimmune disorders

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22
Q

Is Rheumatoid Factor monoclonal or polyclonal?

A

May be either

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23
Q

Is Rheumatoid Factor seen in healthy individuals?

A

Yes
Higher chance with age

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24
Q

High titer Rheumatoid Factor usually seen in this two conditions

A

Rheumatoid arthritis and Systemic Sclerosis

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25
Q

Typical onset of Systemic Lupus Erythematosus

A

Mostly young adults 20-30’s, but any age

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26
Q

2 very specific autoantibodies associated with Systemic Lupus Erythematosus

A

Anti-Smith
Anti-dsDNA

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27
Q

What is Systemic Lupus Erythematosus mediated by?

A

Immune complexes and direct cell destruction

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28
Q

What organs/tissues does Systemic Lupus Erythematosus affect?

A

“Any”
Lesions of kidney, serosal membranes, skin, joints most common

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29
Q

4 effects of renal lesions in Systemic Lupus Erythematosus

A

Hematuria
Proteinuria
Increased creatinine (indicates renal failure)
Decreased albumin (causes edema)

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30
Q

Basement membrane is normally thin and uniform, however one that is thick with big dark deposits of Ig complexes is characteristic of this condition

A

Systemic Lupus Erythematosus

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31
Q

Malar rash is characteristic of this condition

A

Systemic Lupus Erythematosus

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32
Q

This type of rash is characteristic of Systemic Lupus Erythematosus

A

Malar rash

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33
Q

Rash, often after sun exposure, occurs in this condition

A

Systemic Lupus Erythematosus

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34
Q

Subacute cutaneous lupus erythematosus or Discoid lupus:
Annular photosensitive rash
Scaly with erythematous rim

A

Subacute cutaneous lupus erythematosus

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35
Q

Subacute cutaneous lupus erythematosus or Discoid lupus:
Tend to be transient with no scarring

A

Subacute cutaneous lupus erythematosus

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36
Q

Subacute cutaneous lupus erythematosus or Discoid lupus:
Typically oval rash with raised plaque and erythematous rim

A

Discoid lupus

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37
Q

Subacute cutaneous lupus erythematosus or Discoid lupus:
Deep inflammation

A

Discoid lupus

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38
Q

Subacute cutaneous lupus erythematosus or Discoid lupus:
Produces atrophy, pigment loss, hair loss, scarring

A

Discoid lupus

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39
Q

Condition involving:
Hemolytic anemia (due to autoimmune hemolysis)
Anemia of chronic disease (due to chronic inflammation)
Leukopenia
Immune thrombocytopenia

A

Systemic Lupus Erythematosus

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40
Q

Level of creatinine in Systemic Lupus Erythematosus

A

High

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41
Q

Result of Coomb’s test in Systemic Lupus Erythematosus

A

Positive
Autoimmune hemolysis common

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42
Q

Renal lesions in Systemic Lupus Erythematosus are mostly this type

A

Glomerular
But also affects tubules, vessels

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43
Q

Anti-cardiolipin antibodies indicate this condition

A

Systemic Lupus Erythematosus

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44
Q

Condition that most commonly affects kidneys, serosal membranes, skin, joints, lungs and heme

A

Systemic Lupus Erythematosus

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45
Q

Arthritis in Systemic Lupus Erythematosus involves these types of joints

A

Small joints (hands, fingers)

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46
Q

Autoimmune destruction of salivary and lacrimal glands

A

Sjogren Syndrome

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47
Q

Sjogren Syndrome typically involves this age and gender

A

50-60 year old woman

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48
Q

Is Sjogren Syndrome primary or secondary?

A

May be either

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49
Q

Sjogren Syndrome may be secondary, most commonly to this

A

Rheumatoid arthritis

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50
Q

Sjogren Syndrome may result in this symptom complex involving glandular enlargement and inflammation, usually with decreased secretions

A

Mikulicz Syndrome

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51
Q

Mikulicz Syndrome is a symptom complex involving glandular enlargement and inflammation, usually with decreased secretions, and results from this condition

A

Sjogren Syndrome

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52
Q

Sjogren Syndrome involves autoimmune destruction of these

A

Salivary and lacrimal glands

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53
Q

Clinical symptom of Sjogren Syndrome involving dry, irritated eyes, and photobia

A

Xerophthalmia

54
Q

Clinical symptom of Sjogren Syndrome involving dry, cracked lips and decreased taste, dysphagia to solids

A

Xerostomia

55
Q

Xerophthalmia and Xerostomia are characteristic of this condition

A

Sjogren Syndrome

56
Q

In Sjogren Syndrome, these types of cells infiltrate glands
Mostly periductal

A

CD4+ T cells and B cells

57
Q

Lymphoepithelial lesions are present in this condition

A

Sjogren Syndrome

58
Q

Keratitis, Ulceration and infection, Oral ulcers, caries and candidiasis, and MALT lymphoma are complications of this condition

A

Sjogren Syndrome

59
Q

Progressive interstitial fibrosis of skin and other organs
Combination of vascular damage and tissue fibrosis
Always involves vasculature and skin
Variable involvement of other organs

A

Systemic Sclerosis

60
Q

Other name for Systemic sclerosis

A

Scleroderma

61
Q

Systemic Sclerosis is a combination of these two things

A

Vascular damage and tissue fibrosis
(always involves vasculature and skin; variable involvement of other organs)

62
Q

In Systemic Sclerosis, these produce pro-fibrotic cytokines (TGF-beta, PDGF, IL-13)

A

CD4+ T cells

63
Q

In Systemic Sclerosis, CD4+ T cells produce these 3 pro-fibrotic cytokines
Activate fibroblasts and myofibroblasts to produce collagen

A

TGF-beta, PDGF, IL-13

64
Q

Type of autoantibodies associated with limited scleroderma

A

Anti-centromere ANA

65
Q

Type of autoantibodies associated with diffuse scleroderma

A

Anti-RNA pol III

66
Q

Type of autoantibodies associated with both limited and diffuse scleroderma

A

Anti-Scl-70 (topoisomerase I)

67
Q

Anti-centromere ANA autoantibodies are associated with this type of scleroderma

A

Limited

68
Q

Anti-RNA pol III autoantibodies are associated with this type of scleroderma

A

Diffuse

69
Q

Anti-Scl-70 (topoisomerase I) autoantibodies are associated with this type of scleroderma

A

Both limited and diffuse

70
Q

Dystrophic calcium deposition is characteristic of this condition

A

Systemic Sclerosis

71
Q

Symptom of Systemic Sclerosis involving tapered fingers, joint contractures, ischemic digits, nail dystrophy

A

Sclerodactyly

72
Q

Sclerodactyly is characteristic of this condition

A

Systemic Sclerosis

73
Q

Facial telangiectasias and tight perioral skin with furrowed lips are characteristic of this condition

A

Systemic Sclerosis

74
Q

Arteriolar intimal thickening and narrowing is characteristic of this condition

A

Systemic Sclerosis

75
Q

Raynaud’s phenomenon and telangiectasias are characteristic of this condition

A

Systemic Sclerosis

76
Q

Vascular narrowing and interstitial fibrosis that may decrease renal function and accelerate hypertension is characteristic of this condition

A

Systemic Sclerosis

77
Q

Mural fibrosis in the GI tract (in esophagus leads to poor peristalsis and in intestine causes poor absorption and dysmotility) is characteristic of this condition

A

Systemic Sclerosis

78
Q

Vascular ectasias is characteristic of this condition

A

Systemic Sclerosis
Leads to chronic blood loss and iron deficiency

79
Q

This is the most common cause of death in untreated Systemic Sclerosis

A

Renal hypertension

80
Q

Tight, thickened skin and atrophic adnexa are characteristic of this condition

A

Systemic Sclerosis

81
Q

Type of Systemic Sclerosis that mainly involves skin, especially distal fingers and face
Raynauds as a long preclude to skin findings

A

Limited scleroderma

82
Q

Type of Systemic Sclerosis that involves late systemic findings

A

Limited scleroderma

83
Q

Type of Systemic Sclerosis that is also known as “CREST syndrome”
(Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyly, Telangiectasias)

A

Limited scleroderma

84
Q

Type of Systemic Sclerosis that involves a short prelude of Raynauds

A

Diffuse scleroderma

85
Q

Type of Systemic Sclerosis that involves earlier and more progressive organ involvement

A

Diffuse scleroderma

86
Q

Type of Systemic Sclerosis with rapid development and more frequent fatality (most often due to renal or lung disease)

A

Diffuse scleroderma

87
Q

Anti-centromere autoantibodies are most common in this type of Systemic Sclerosis

A

Limited scleroderma

88
Q

Overlap syndrome with features of SLE, Scleroderma, Polymyositis
May evolve into single defined syndrome
Clinical – usually a milder course than others
(Constitutional syndrome, Raynauds, Arthralgias, Myalgias)

A

Mixed connective tissue disease

89
Q

Speckled ANA and anti-U1 RNP are found in this condition

A

Mixed connective tissue disease

90
Q

Deposition of fibrillar proteins with Beta-pleated sheet structure

A

Amyloidosis

91
Q

In amyloidosis, there is a deposition of fibrillar proteins with this structure

A

Beta-pleated sheet

92
Q

This stains with Congo red stain –> Apple green birefriingence

A

Amyloid

93
Q

In amyloidosis, this stain is used

A

Congo Red stain

94
Q

Type of Amyloidosis that is the most common form in the US

A

AL: Light chain amyloidosis

95
Q

Type of Amyloidosis that is seen in plasma cell neoplasms, results in systemic amyloidosis

A

AL: Light chain amyloidosis

96
Q

Type of Amyloidosis that is most common in the developing world

A

AA: amyloid associated protein (AAP)

97
Q

Type of Amyloidosis that involves acute phase protein (seen in chronic inflammatory condition), results in systemic amyloidosis

A

AA: amyloid associated protein (AAP)

98
Q

Carrier molecule for thyroid hormone and retinol

A

Transthyretin

99
Q

Problems with this protein is seen in Senile cardiac amyloidosis

A

Transthyretin

100
Q

Problems with this protein is seen in Familial amyloid polyneuropathy

A

Transthyretin

101
Q

Problems with this protein are seen in Alzheimer’s plaques and cerebral amyloid angiopathy

A

Amyloid precursor protein

102
Q

Type of Amyloidosis due to misfolded Ig light chain deposition

A

AL amyloidosis

103
Q

AL amyloidosis is caused by this

A

Misfolded Ig light chain deposition

104
Q

Type of amyloidosis involving neoplastic plasma cells (–> monoclonal immunoglobulin)

A

AL amyloidosis

105
Q

In AL amyloidosis, light chains (usually lambda) deposit as these

A

Beta-pleated sheets

106
Q

In AL amyloidosis, these deposit as beta-pleated sheets

A

Light chains (usually lambda)

107
Q

Type of amyloidosis due to misfolded AAP

A

AA amyloidosis

108
Q

AA amyloidosis is due to this

A

Misfolded AAP (amyloid precursor protein)

109
Q

Type of amyloidosis involving a chronic inflammatory state

A

AA amyloidosis

110
Q

APP amyloidosis is due to this

A

Misfolded APP

111
Q

In APP amyloidosis, there are deposits in these 2 places

A

Blood vessels and Neuritic Plaques

112
Q

Type of amyloidosis involving elderly state or increased production

A

APP amyloidosis

113
Q

Macroglossia is characteristic of this condition

A

Amyloidosis

114
Q

Impaired glomerular function, mild hepatomegaly, interstitial deposition in heart (decreased myocardial compliance) and tongue (macroglossia), CNS plaques and amyloid angiopathy, and PNS vascular and neural deposition (compression and ischemic neuropathy) are clinical symptoms of this condition

A

Amyloidosis

115
Q

Typical patient with amyloidosis

A

70 year old man with multiple myeloma

116
Q

Centromere pattern in indirect immunofluorescence is specific for this

A

Limited cutaneous systemic sclerosis

117
Q

Anti-Smith autoantibodies are seen in this condition

A

Systemic lupus erythematosus

118
Q

Anti-dsDNA autoantibodies are seen in this condition

A

Systemic lupus erythematosus

119
Q

Anti-SS-A, anti-SS-B and anti-RF autoantibodies are seen in this condition

A

Systemic lupus erythematosus

120
Q

Autoantibodies seen in Systemic lupus erythematosus

A

Anti-Smith
Anti-dsDNA
Anti-cardiolipin
Anti-SS-A (Ro), SS-B (La), RF

121
Q

Autoimmune disease mediated by immune complexes and direct cell destruction

A

Systemic lupus erythematosus

122
Q

Are acute phase reactants seen in Systemic lupus erythematosus?

A

Yes

123
Q

CD4+ T cells produce pro-fibrotic cytokines (TGF-beta, PDGF, IL13) in this condition
Activated fibroblasts and myofibroblasts to produce collagen

A

Systemic sclerosis

124
Q

Raynaud’s phenomenon is seen in these 2 autoimmune conditions

A

Systemic sclerosis
Mixed connective tissue disease

125
Q

Systemic sclerosis effects on renal vasculature may do this

A

Accelerate hypertension

126
Q

Drawn, tight facial mask appearance is characteristic of this autoimmune disorder

A

Systemic sclerosis

127
Q

Most common cause of death in treated systemic sclerosis

A

Pulmonary disease
(Dyspnea)

128
Q

Dysphagia, gastroesophageal reflux, malabsorption, abdominal pain, renal hypertension, and dyspnea are seen in this autoimmune disorder

A

Systemic sclerosis

129
Q

What does CREST stand for when used to describe limited scleroderma?

A

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasias

130
Q

Most systemic sclerosis patients are positive for this laboratory test

A

ANA +

131
Q

2 autoantibodies seen in mixed connective tissue disease

A

Speckled ANA
Anti-U1 RNP

132
Q

Type of amyloidosis characterized by a chronic inflammatory state

A

AA amyloidosis

MF2 Final study (28 decks)

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