UNIT 3: Autoimmune Diseases Flashcards Preview

Immunology and Serology [LEC] > UNIT 3: Autoimmune Diseases > Flashcards

Flashcards in UNIT 3: Autoimmune Diseases Deck (147)
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1
Q

Class I associated

A

Ankylosing spondylitis
Myasthenia gravis

2
Q

Class II associated [7]

A

Hashimoto’s disease
Graves’ disease
Type 1 diabetes
Addison’s disease
Rheumatoid arthritis
Sjögren’s syndrome
Multiple sclerosis

3
Q

B27

A

Ankylosing spondylitis

4
Q

B8

A

Myasthenia gravis

5
Q

DR2

A

Multiple sclerosis

6
Q

DR3

A

Graves’ disease
Addison’s disease
Sjögren’s syndrome

7
Q

DR4

A

Rheumatoid arthritis

8
Q

DR5

A

Hashimoto’s disease

9
Q

DQ2+DQ8

A

Type 1 diabetes

10
Q

DQ6

A

Type 1 diabetes

11
Q

DQ8

A

Type 1 diabetes

12
Q

RA, several others

A

PTPN22

13
Q

Abnormal tyrosine phosphatase regulation of T cell selection and activation

A

PTPN22

14
Q

Crohn disease

A

NOD2

15
Q

Defective resistance or abnormal responses to intestinal microbes

A

NOD2

16
Q

IBD, inflammatory bowel disease

psoriasis, PS

AS

A

IL23R

17
Q

Component of IL-23 receptor; role in generation and maintenance of Th17 cells

A

IL23R

18
Q

T1D, RA

A

CTLA4

19
Q

Inhibitory receptor of T cells, effector of regulatory T cells

A

CTLA4

20
Q

MS, type 1 diabetes, others

A

CD25 (IL-2Rα)

21
Q

Abnormalities in effector and/or regulatory T cells?

A

CD25 (IL-2Rα)

22
Q

SLE

A

C2, C4 (Complement proteins)

FCGRIIB (FCγRIIb)

23
Q

Defects in clearance of immune complexes or in B cell tolerance?

A

C2, C4 (Complement proteins)

24
Q

Defective feedback inhibition of B cells

A

FCGRIIB (FCγRIIb)

25
Q

Autoimmune polyendocrine syndrome (APS-1)

A

AIRE

26
Q

Reduced expression of peripheral tissue antigens in the thymus, leading to defective elimination of self- reactive T cells

A

AIRE

27
Q

Autosomal dominant immune dysregulation syndrome

A

CTLA4

28
Q

Impaired regulatory T cell function leading to loss of B and T cell

A

CTLA4

29
Q

Immune dysregulation, X-linked Polyendocrinopathy and enteropathy (IPEX)

A

FOXP3

30
Q

Deficiency of regulatory T cells

A

FOXP3

31
Q

Autoimmune lymphoproliferative syndrome (ALPS)

A

FAS

32
Q

Defective apoptosis of self-reactive T and B cells in the periphery

A

FAS

33
Q

IL23R

A

Ankylosing spondylitis

34
Q

PTPN22

A

RA

35
Q

CTLA4

A

RA
T1D

36
Q

CD25 (IL-2Rα)

A

T1D
MS

37
Q

C2, C4

A

SLE

38
Q

FCGRIIB (FCγRIIb)

A

SLE

39
Q

Presence of [?] autoantibodies and/or autoreactive lymphocytes in vivo

A

high titer

40
Q

Autoantibody binding and/or T‐cell reactivity to [?] in vitro

A

autoantigen

41
Q

[?] with autoreactive serum and/or autoreactive lymphocytes

A

Transfer of disease

42
Q

Immunopathology consistent with

A

autoimmune‐mediated processes

43
Q

Beneficial effect of

A

immunosuppressive interventions

44
Q

Exclusion of [?] of disease

A

other possible causes

45
Q

association

A

MHC

46
Q

[?] mirroring the human disease

A

Animal model

47
Q

Similarities of Organ-Specific and Organ-Nonspecific Disorders

Circulating autoantibodies react with [?]

Increased [?] in serum often found.

Antibodies may appear in each of the main [?] classes.

Disease process not always [?]; exacerbations and remissions occur.

[?] of diagnostic value

A

normal body constituents

immunoglobulin concentration

immunoglobulin

progressive

Autoantibody tests

48
Q

Antibodies and lesions are organ-specific

A

Organ-specific

49
Q

Antibodies and lesions nonspecific

A

Non-organ specific

50
Q

Clinical and serologic overlap

A

Organ-specific

51
Q

Overlap of SLE, RA, and other connective tissue disorders

A

Non-organ specific

52
Q

Antigens only available to lymphoid system in low concentrations

A

Organ-specific

53
Q

Antigens in high concentrations

A

Non-organ specific

54
Q

Antigens evoke organ-specific antibodies in normal animals with complete Freund’s adjuvant

A

Organ-specific

55
Q

No antibodies produced in animals with comparable stimulation

A

Non-organ specific

56
Q

Familial tendency to develop organ
Specific autoimmunity

A

Organ-specific

57
Q

Familial tendency to develop connective tissue disease.

A

Non-organ specific

58
Q

Questionable abnormalities in immunoglobulin synthesis in relatives

A

Non-organ specific

59
Q

Lymphoid invasion, parenchymal destruction by questionable cell mediated hypersensitivity or antibodies

A

Organ-specific

60
Q

Lesions caused by deposition of antigen antibody (immune) complexes

A

Non-organ specific

61
Q

Tendency to develop cancer in the organ

A

Organ-specific

62
Q

Tendency to develop lymphoreticular
neoplasia

A

Non-organ specific

63
Q

joints

A

SLE
RA

64
Q

lungs

A

SLE
Granulomatosis with polyangiitis

65
Q

Multiple cells and organs

A

SLE

66
Q

skin
kidneys
brain
heart

A

SLE

67
Q

bone; other tissues in some cases

A

RA

68
Q

Upper respiratory system
blood vessels

A

Granulomatosis with polyangiitis

69
Q

• Antibodies to double-stranded DNA
and other nuclear components, such as Sm (ANAs)

A

SLE
RA
Granulomatosis with polyangiitis

70
Q

• Phospholipid antibodies

A

SLE

71
Q

• Antibody to RBCs

A

SLE

72
Q

• Antibody to platelets

A

SLE

73
Q

• Antibody to lymphocytes

A

SLE

74
Q

• Antibody to ribosomal components

A

SLE

75
Q

• Antibody to endothelium

A

SLE

76
Q

• Rheumatoid factor

A

SLE
RA
Granulomatosis with polyangiitis

77
Q

• Anti-CCP (cyclic citrullinated proteins)

A

RA

78
Q

• Antineutrophil cytoplasmic antibodies (ANCA); c-ANCA pattern

A

Granulomatosis with polyangiitis

79
Q

Liver

A

Autoimmune hepatitis (AIH)

80
Q

Small intestine and other
organs

A

Celiac disease

81
Q

Kidneys

A

Goodpasture’s syndrome
Poststreptococcal glomerulonephritis

82
Q

lungs

A

Goodpasture’s syndrome

83
Q

Thyroid gland

A

Graves disease
Hashimoto’s thyroiditis

84
Q

Myelin sheath
of nerves

A

Multiple sclerosis

85
Q

Nerve-muscle synapses

A

Myasthenia gravis

86
Q

Stomach

A

Pernicious anemia

87
Q

Intrahepatic bile ducts

A

Primary biliary cirrhosis

88
Q

Heart

A

Rheumatic fever

89
Q

Connective tissue

A

Scleroderma

90
Q

Eyes, mouth

A

Sjogren syndrome

91
Q

Pancreas

A

Type 1 diabetes mellitus

92
Q

Antibody to adrenal cells

A

Addison’s disease

93
Q

AIH-1—smooth muscle antibodies

A

Autoimmune hepatitis

94
Q

AIH-2—anti-liver kidney microsomal antibody

A

Autoimmune hepatitis

95
Q

anti-liver cytosol type 1 antibody (anti-LC-1)

A

Autoimmune hepatitis

96
Q

Antitransglutaminase (tTG)

A

Celiac disease

97
Q

Antibodies to deamidated gliadin peptides (DGPs)

A

Celiac disease

98
Q

Endomysial antibodies

A

Celiac disease

99
Q

Antibody to an antigen in the renal and
pulmonary basement membranes

A

Goodpasture’s syndrome

100
Q

Thyroid-stimulating hormone receptor antibodies (TRAbs)

A

Graves disease

101
Q

Antithyroglobulin

A

Graves disease
Hashimoto’s thyroiditis

102
Q

Antithyroid peroxidase (TPO)

A

Graves disease
Hashimoto’s thyroiditis

103
Q

Antibodies to myelin basic protein

A

Multiple sclerosis

104
Q

Antibodies to acetylcholine receptors (AChR)

A

Myasthenia gravis

105
Q

Anti-muscle-specific kinase (MuSK)

A

Myasthenia gravis

106
Q

Antibody to the lipoprotein LRP4

A

Myasthenia gravis

107
Q

Parietal cell antibody, intrinsic factor
antibody

A

Pernicious anemia

108
Q

Streptococcal antibodies that cross-react
with kidney tissue

A

Poststreptococcal glomerulonephritis

109
Q

Antimitochondrial antibodies (AMA)

A

Primary biliary cirrhosis

110
Q

Streptococcal antibodies that cross-react
with cardiac tissue

A

Rheumatic fever

111
Q

Antinuclear antibodies: anti-Scl-70

A

Scleroderma

112
Q

anticentromere antibody

A

Scleroderma

113
Q

rheumatoid factor

A

Sjogren

114
Q

antisalivary duct antibodies

A

Sjogren

115
Q

antilacrimal gland antibodies

A

Sjogren

116
Q

Anti-insulin

A

Type 1 diabetes mellitus

117
Q

IA-2 and anti–IA-2Ba

A

Type 1 diabetes mellitus

118
Q

Antibody to glutamic acid phosphatase (GAD)

A

Type 1 diabetes mellitus

119
Q

HLA-A1, B8, and DR3

A

SLE

120
Q

may cause DNA to form thymine dimers

A

UV light

121
Q

described in anti–TNF-treated rheumatic patients

A

lupus-like autoantibodies

122
Q

Antibody-forming B lymphocytes are stimulated in a relatively nonspecific manner

A

polyclonal B cell activation

123
Q

specific for SLE and are observed at a frequency of 75% to 90% in SLE patients with active disease

A

Anti-ds DNA Auto Ab

124
Q

containing complement-activating IgG3

have a particular tropism for basement membranes

readily deposited in the kidney glomeruli

A

DNA-anti-DNA immune complexes

125
Q

can also cause cellular destruction by other mechanisms

A

Autoantibodies to nuclear and nonnuclear antigens

126
Q

can cause hemolytic anemia

A

Antibodies to red blood cells (RBCs

127
Q

and antibodies
to platelets can cause thrombocytopenia by

A

antibody-mediated cytotoxic (type II) hypersensitivity

128
Q

can cause inflammation of the blood vessels, and
vascular damage in lupus, responsible for the vasculitis and neuropsychiatric symptoms are seen in some SLE patients

A

Antibodies to endothelial cells

129
Q

associated with increased miscarriage, stillbirth, and preterm delivery in pregnant women with lupus

A

Phospholipid antibodies

130
Q

Neonatal lupus, which occurs in up to 8% of babies born to pregnant women with
SLE, is associated with antibodies to the nuclear antigens,

A

SS-A/Ro and SS-B/La

131
Q

diverse; nonspecific symptoms

A

SLE

132
Q

may appear on any area of the body exposed to UV light

A

erythematous rash

133
Q

butterfly rash (“malar rash”) across the nose and cheeks

A

Wolf-like

134
Q

increased in those who have antibodies to nuclear RNP in their serum

A

Raynaud’s phenomenon

135
Q

polyarthralgia or arthritis

A

Joint involvement

136
Q

Nephritis

A

Renal involvement

137
Q

the most dangerous type of lesion characterized by cell proliferation

A

diffuse
proliferative glomerulonephritis

138
Q

pericarditis, tachycardia, ventricular enlargement; pleuritis

A

cardiac involvement

139
Q

seizures, mild cognitive dysfunction, psychoses, or depression

A

neuropsychiatric manifestations

140
Q

anemia, leukopenia,
thrombocytopenia, or lymphopenia

A

Hematologic abnormalities

141
Q

About 20% of episodes of fever are caused by

A

Infections

142
Q

leading cause of death in hospitalized patients

A

Infections

143
Q

can interfere with host defense against opportunistic infections

A

Immunosuppression

144
Q

✓ Complete blood count (CBC) ✓ Platelet count
✓ Urinalysis ✓ Erythrocyte sedimentation rate (ESR) determination ✓ CRP levels

A

SLE

145
Q

quantification of complement proteins
and the detection of specific autoantibodies

A

SLE

146
Q

is the most commonly measured complement protein

A

C3

147
Q

may be low during disease flares as a result of
complement consumption by immune complexes. Thus, complement levels can be helpful not only in the initial diagnosis but also for monitoring patients over time

A

Serum complement levels