Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology 2 > Autoimmune > Flashcards

Autoimmune Flashcards

1
Q

Principal mechanism of central tolerance

A

antigen-induced deletion of self-reactive lymphocytes in central lymphoic organs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

___ is often the first manifestation of SS

A

Puffy Fingers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

___ of the muscularis may develop in gut in SS

A

Progressive atrophy and collagenous fibrous replacement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

2 ANA’s virtually unique to SS

A

Anti-Scl 70 (topo1) and Anticentromere Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

4 environmental factors implicated in SLE

A

UV, Cigarrettes, Sex hormones, Drugs (procainamide, hydralazine)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Ab’s for dsDNA and Smith antigen

A

Specific for SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Ab’s in Limited vs Diffuse SS

A

Limited: Anti-centromere; Diffuse: Anti-Scl-70

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Ab’s to __ and __ are specific for SLE

A

dsDNA and Smith antigen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

About half of SS deaths are attributable to

A

Renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Acute cellular rejection is typically accompanied by clinical signs of

A

renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Acute cellular transplant rejection primarily involves

A

Tubules and Interstitium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Acute GVHD causes epithelium necrosis in 3 principal organs

A

Liver, Skin, Gut

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Acute humoral rejection mainly effects

A

Graft vasculature

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Acute humoral transpant rejection is dx by

A

Staining for C4d

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Advanced Sclerosing Lupus Nephritis (VI) is characterized by

A

Complete sclerosis of >90% of glomeruli –> Corresponds to end stage renal disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Almost all SS patients exhibit

A

Raynaud

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

ALPS is characterized by

A

Lymphadenopathy and multiple auto-antibodies, including anti-DNA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Anti-CD20 Ab

A

Rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Anti-centromere Ab’s are MOST associated with

A

CREST syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Anti-histones indicates

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Anti-Jo 1 (histidyl tRNA ligase) Ab’s are associated with

A

Inflammatory myopathies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Anti-RNP U1 Ab’s are associated with

A

Mixed CT Disease, SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Anti-RNP U1 indicates

A

SLE, mixed CT disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Anti-Scl 70 (topo1) and Anticentromere Ab

A

2 ANA’s virtually unique to SS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Anti-Scl 70 Ab in SS indictaes

A

more aggressive disease with pulmonary fibrosis and peripheral vascular changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Anti-Scl-70 Ab’s are associated with

A

Systemic Sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Anti-Scl-70 indicates

A

Systemic Sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Anti-SS-A and -B are associated with

A

Sjogren Syndrome, SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Anti-SS-A, -B indicates

A

Sjogren, SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Anticentromere Ab in SS indicates

A

relatively benign course

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Atherosclerosis in SLE

A

accelerated –> coronary artery disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Auto-Ab’s against phospholipids lead to

A

increased thrombosis –> recurrent spont abortions and thrombotic episodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Autoamputaton

A

SS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Autoimmune disorder characterized by excessive fibrosis in multiple tissues, obliterative vascular disease, and multiple auto-Ab’s

A

Systemic Sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

B cells can become anergic if they encounter antigen in absence of

A

specific helpter T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

B cells in Sjogren

A

Hyperactive, evidenced by ANA’s and RF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Blood vessels in SLE

A

Acute necrotizing vasculitis –> Fibrinoid deposits withing vessel walls containing Ab, DNA, complement, and fibrinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Cardiac involvement in SLE

A

primarily endocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Chronic GVHD develop skin lesions resembling

A

SS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Chronic rejection is dominated by

A

vascular changes (ateriosclerosis), interstitial fibrosis, loss of renal parenchyma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Chronic rejection is probably mediated by

A

T cell reaction and secretion of cytokines that induce proliferation of vascular SM cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

CNS disease in SLE is often ascribed to

A

Vascular lesions causing ischemia or multifocal cerebral microinfarcts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Concentric sclerosing intimal thickening of interlobar arteris

A

Onion skinning in SS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

CREST presentation is seen in

A

Limited Scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

CREST Syndrome

A

Scleroderma: Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangectasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Cyclosporine and FK506 suppress

A

T cell mediated immunity by inhibiting IL-2 transcription

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Diffuse Lupus Nephritis (IV) is distinguished from Focal Lupus Nephritis by

A

involvement of half or more glomeruli

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Distinctive feature of SS

A

Striking cutaneous involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Does having a C1q, C2, or C4 deficiency predispose to Lupus?

A

Yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Does having low leves of IFN-alpha predispose to getting lupus?

A

No, high levels do

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Dominant T cells in Scleroderma

A

Th2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Dysfunction of LESphincter and Barret’s Disease

A

SS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Esophageal involvement in SS leads to

A

Aspiration pneumonia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Extraglandular manifestations of Sjogren

A

Synovitis, Pulmonary Fibrosis, Peripheral Neuropathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Fingers take on a tapered, clawlike appearance with limited motion

A

Late Scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Genetic deficiencies in classical complement pathway are seen in __ patients with SLE

A

10%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

GI tract is affected in what percent of SS patients

A

90%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Having lymphocytes with __ predisposes to getting lupus

A

TLR7, 9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Histology of *cellular acute transplant rejection is marked by

A

Interstitial mononuclear cell infiltrate with edema and parenchymal injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Histology of *humoral acute transplant rejection

A

Vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Histology of Hyperacute rejection

A

Acute arteritis, vessel thrombosis, and ischemic necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Histopathological findings in skin of early SS

A

Edema and perivascular infiltrates containing CD4 T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Histopathological findings in skin of SLE

A

(1) Liquefactive degeneration of basal layer of epidermis; (2) Edema at dermoepidermal jxn; (3) Mononuclear inflitrates around blood vessels and skin appendages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

HLA types predisposing to Lupus

A

HLA-DR2, HLA-DR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

How do Ab’s mediate graft rejection

A

Bind to graft endothelium and cause vascular injury thru complement activation and recruitment of leukocytes

66
Q

Hyperacute rejection is due to

A

Pre-formed anti-donor Ab’s

67
Q

IgG4-RD is characterized by

A

Tendency to form tumor-like lesions in severl organs

68
Q

immune complex deposits are thought to cause tissue damage by

A

activating classical complement pathway

69
Q

Immune complex is what type of hypersensitivity

A

Type 3

70
Q

Immune responses in acute rejection

A

both cellular and humoral

71
Q

In early SS, affeccted skin is somewhat

A

edematous and doughy in consistency

72
Q

In hyperacute rejection, Ab’s bind to graft endothelium and cause

A

Thrombosis, Ischemia, Rapid Graft failiure

73
Q

In Scleroderma, activated T cells and mac’s produce

A

cytokines that activated fibroblasts and stimulate collagen production

74
Q

In Scleroderma, cytokines that activate fibroblasts include

A

TGF-Beta, IL-13, PDGF, others

75
Q

In Scleroderma, TH2 cells and their cytokines induce

A

Alternative macrophage activation and collagen deposition

76
Q

In Sjogren, there is a 40-fold increased risk for developing

A

non-Hodgkin b cell lymphoma

77
Q

In SS, capillaries and small arteries may show

A

thickening of Basal Lamina, endothelial damage, and partial occlusion

78
Q

In SS, in the absence of __ involvement, lifespan may be normal

A

Renal

79
Q

In SS, repeated bouts of endothelial damage followed by platelet agg lead to

A

Endothelial proliferation and Intimal Fibrosis –> Narrow small vessels –> Ischemia injury

80
Q

In SS, there is marked increase in __ in the dermis

A

compact collagen

81
Q

Increasing number of patients with SLE show this cardiac pathology:

A

Coronary Artery Disease

82
Q

INF-alpha in SLE

A

patients produce abnormally large amounts

83
Q

Joint involvement in SLE

A

Swelling and nonspecific mononuclear cell infiltration of synovial membranes

84
Q

Kidney transplant biopsy can also reveal

A

infections such as polyomavirus

85
Q

LE/Hematoxylin Bodies

A

Nuclei of damaged cells react with ANAs, lose their chromatin pattern, and become homogenous

86
Q

Limited Scleroderma is often confined to

A

Fingers and Face

87
Q

Liver in SLE

A

Nonspecific inflammation of portal tracts

88
Q

Lung involvement in SS

A

Pulmonary htn and/or interstitial fibrosis

89
Q

Lung involvement in SS leads to

A

Interstitial fibrosis

90
Q

Lungs in SLE

A

Interstitial fibrosis, Pleural inflammation

91
Q

Lupus anticoagulants

A

Anti-phospholipid antibodies –> interfere with clotting tests

92
Q

Major culprits contributing to direct recognition

A

DC’s in the graft (many MHC’s expressed)

93
Q

Major target of Systemic Sclerosis

A

Skin

94
Q

Membranous Lupus Nephritis is characterized by

A

widespread thickening of capillary wall due to deposition of subepithelial immune complexes

95
Q

Mixed CT Disorder have high titers of

A

Anti-RNP U1

96
Q

Mixed CT Disorder respond very well to

A

Corticosteroids

97
Q

Most common associated disorders with Sjogren

A

RA first, SLE and others second

98
Q

Most common causes of death from SLE are:

A

Renal Failure, Intercurrent Infections, Cardiovascular Disease

99
Q

Most common glandular targets in Sjogrens

A

Lacrimal and Salivary glands

100
Q

Most frequent pathologic CNS lesion in SLE

A

Small vessel angiopathy w/ noninflammatory intimal proliferation

101
Q

Most important consequence of direct recognition

A

Activation of host CD8 T cells that recognize class I MHC (HLA-A and -B) in the graft

102
Q

Most organ damage in SLE is caused by

A

Immune Complex Deposition

103
Q

Most widely used method for detecting ANAs

A

Indirect immunofluorescence assay

104
Q

Musculoskeletal changes in SS

A

Early: Synovial hyperplasia and inflammtion; Late: Fibrosis

105
Q

Mutation in FAS gene

A

Autoimmune Lymphoproliferative Syndrome (ALPS)

106
Q

Mutation in what causes ALPS

A

FAS

107
Q

Mutation in what causes IPEX

A

Foxp3

108
Q

Mutations in FOXP3 cause

A

IPEX –> Treg deficiency

109
Q

Non-specific auto-Ab’s for SLE

A

Anti-phospholipid, Anti-RNP U1, Anti-SSA A and B

110
Q

Onion-Skin Lesions

A

Spleen of SLE –> Thickening and Perivascular fibrosis of Central penicillilary arteries

111
Q

Organs affected in IgG4-RD

A

Almost every one

112
Q

Pathogenesis of all forms of glomerulonephritis in SLE involves

A

deposition of anti-DNA immune complexes

113
Q

Patients present with chronic rejection with progressive rise in

A

Serum Creatinine levels

114
Q

Peak age incidence of SS

A

50-60

115
Q

Percent of Sjogren patients that acquire extraglandular disease

A

25%

116
Q

Percent of Sjogren patients with another accompanying autoimmune disorder

A

60%

117
Q

Polyarteritis Nodosa belongs to a group of diseases characterized by

A

necrotizing inflammation of walls of blood vessels

118
Q

Predominant cell type in acute transpant rejection

A

Depends on patient

119
Q

Predominant cell type in IgG4-RD

A

T cells

120
Q

Presentation of Sjogren

A

90% women between 35-45

121
Q

Presenting manifestation of Systemic Sclerosis

A

Cutaneous involvement

122
Q

Progressive rise of serum creatinine levels indicates

A

Chronic transplant rejection

123
Q

Puffy fingers are often the first manifestation of

A

Systemic Sclerosis

124
Q

Renal abnormalities are present in what percent of SS patients

A

two thirds

125
Q

Renal lesions in Sjogren

A

Mild interstitial nephritis

126
Q

Renal manifestation of SS

A

Thickening of vessel walls and interlobular arteries

127
Q

Scleroderma is characterized by

A

(1) Excessive fibrosis in multiple tissues; (2) Obliterative vascular disease; (3) Multiple Auto-Ab’s

128
Q

Self-reactive B cells that are not deleted undergo

A

Receptor editing

129
Q

Serosal manifestations of SLE

A

Inflammatory changes: Serous effusions, fibrinous exudates

130
Q

Sjogren is autoimmune mediated by

A

CD4 T cells

131
Q

SLE complement deficiencies may result in

A

defective clearance of immune complexes and apoptotic cells; failure of B cell tolerance

132
Q

SLE patients have higher frequency of auto-reactive __ cells

A

B cells

133
Q

SLE principally affects the

A

skin, kidneys, serosal membranes, joints, and heart

134
Q

Specificity and Sensitivity of IFA for SLE

A

High Sensitivity, Limited Specificity

135
Q

SS is most associated with Ab’s against

A

DNA Topo 1 (Anti-Scl 70)

136
Q

Systemic changes in SLE consist essentially of

A

acute vasculitis of small vessels, foci of mononuclear infiltrations, and fibrinoid deposits

137
Q

T cells destroy graft parenchyma (and vessels) by cytotoxicyt and inflammatory reactions

A

Acute Cellular Rejection

138
Q

T/F: Anti-RNP antigens SS-A and SS-B are dx for Sjogren

A

False, also present in SLE

139
Q

T/F: People deficient in C1q are protected from SLE

A

FALSE

140
Q

T/F: Sjogren has RF even in absence of RA

A

TRUE

141
Q

The best-defined populations of Tregs express

A

CD25, one of the chains of the receptor for IL-2

142
Q

Timeline of acute rejection of transplant

A

Days to months

143
Q

Transplant patients can also have

A

infection

144
Q

Tregs require __ for their generation and survival

A

IL-2

145
Q

Vascular lesions in chronic rejection typically result in

A

compromised vascular perfusion –> Renal ischemia

146
Q

Virtually are arteries in hyperacute rejection exhibit

A

characteristic acute fibrinoid necrosis of walls, with narrowing or complete occlusion of lumens

147
Q

What causes ischemia injury in SS

A

Endotheial proliferation and Intimal Fibrosis –> Narrow small vessels –> Ischemic Injury

148
Q

What causes wire loops

A

Circumferential thickening of capillary wall by extensive, subendothelial immune complexes

149
Q

What clinical manifestation is present in 100% of patients with SLE

A

Hematologic

150
Q

What has been implicated in neuropsychiatric complications of SLE

A

Auto-Ab’s against CNS receptors for various NT’s

151
Q

What indicates B cell activation in SS

A

Hypergammaglobulinemia, ANAs

152
Q

What indicates more aggressive SS

A

Anti-Scl 70

153
Q

What indicates more benign SS

A

Anti-centromere Ab

154
Q

What infiltrates affected glands in Sjogrens

A

Lymphocytes (CD4) and Plasma Cells

155
Q

What is keratoconjunctivitis

A

Drying of corneal epithelium, inflammation, erosion, ucleration

156
Q

What is used to detect antibody-mediated rejection of kidney allografts

A

Local deposition of complement breakdown products

157
Q

Where is atrophy and fibrous replacement of GI most severe in SS

A

Esophagus

158
Q

Which parts of glomerulus show proliferation in lupus nephritis

A

endothelium and mesangium

159
Q

Wire loops are due to

A

subendothelial immune complex deposits

160
Q

With advanced lung involvement in SS, what may develop

A

Secondary pulmonary htn –> Right sided heart failure

161
Q

With progress of SS, edematous phase is replaced by

A

progressive fibrosis of dermis

162
Q

Xerostomia

A

Inflammatory fissuring and ulceration of oral mucosa

Pathology 2 (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions