UNIT 3: Autoimmune Diseases Flashcards
(147 cards)
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
Autoimmune polyendocrine syndrome (APS-1)
AIRE
26
Reduced expression of peripheral tissue antigens in the thymus, leading to defective elimination of self- reactive T cells
AIRE
27
Autosomal dominant immune dysregulation syndrome
CTLA4
28
Impaired regulatory T cell function leading to loss of B and T cell
CTLA4
29
Immune dysregulation, X-linked Polyendocrinopathy and enteropathy (IPEX)
FOXP3
30
Deficiency of regulatory T cells
FOXP3
31
Autoimmune lymphoproliferative syndrome (ALPS)
FAS
32
Defective apoptosis of self-reactive T and B cells in the periphery
FAS
33
IL23R
Ankylosing spondylitis
34
PTPN22
RA
35
CTLA4
RA
T1D
36
CD25 (IL-2Rα)
T1D
MS
37
C2, C4
SLE
38
FCGRIIB (FCγRIIb)
SLE
39
Presence of [?] autoantibodies and/or autoreactive lymphocytes in vivo
high titer
40
Autoantibody binding and/or T‐cell reactivity to [?] in vitro
autoantigen
41
[?] with autoreactive serum and/or autoreactive lymphocytes
Transfer of disease
42
Immunopathology consistent with
autoimmune‐mediated processes
43
Beneficial effect of
immunosuppressive interventions
44
Exclusion of [?] of disease
other possible causes
45
association
MHC
46
[?] mirroring the human disease
Animal model
47
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
normal body constituents
immunoglobulin concentration
immunoglobulin
progressive
Autoantibody tests
48
Antibodies and lesions are organ-specific
Organ-specific
49
Antibodies and lesions nonspecific
Non-organ specific
50
Clinical and serologic overlap
Organ-specific
51
Overlap of SLE, RA, and other connective tissue disorders
Non-organ specific
52
Antigens only available to lymphoid system in low concentrations
Organ-specific
53
Antigens in high concentrations
Non-organ specific
54
Antigens evoke organ-specific antibodies in normal animals with complete Freund’s adjuvant
Organ-specific
55
No antibodies produced in animals with comparable stimulation
Non-organ specific
56
Familial tendency to develop organ
Specific autoimmunity
Organ-specific
57
Familial tendency to develop connective tissue disease.
'
Non-organ specific
58
Questionable abnormalities in immunoglobulin synthesis in relatives
Non-organ specific
59
Lymphoid invasion, parenchymal destruction by questionable cell mediated hypersensitivity or antibodies
Organ-specific
60
Lesions caused by deposition of antigen antibody (immune) complexes
Non-organ specific
61
Tendency to develop cancer in the organ
Organ-specific
62
Tendency to develop lymphoreticular
neoplasia
Non-organ specific
63
joints
SLE
RA
64
lungs
SLE
Granulomatosis with polyangiitis
65
Multiple cells and organs
SLE
66
skin
kidneys
brain
heart
SLE
67
bone; other tissues in some cases
RA
68
Upper respiratory system
blood vessels
Granulomatosis with polyangiitis
69
• Antibodies to double-stranded DNA
and other nuclear components, such as Sm (ANAs)
SLE
RA
Granulomatosis with polyangiitis
70
• Phospholipid antibodies
SLE
71
• Antibody to RBCs
SLE
72
• Antibody to platelets
SLE
73
• Antibody to lymphocytes
SLE
74
• Antibody to ribosomal components
SLE
75
• Antibody to endothelium
SLE
76
• Rheumatoid factor
SLE
RA
Granulomatosis with polyangiitis
77
• Anti-CCP (cyclic citrullinated proteins)
RA
78
• Antineutrophil cytoplasmic antibodies (ANCA); c-ANCA pattern
Granulomatosis with polyangiitis
79
Liver
Autoimmune hepatitis (AIH)
80
Small intestine and other
organs
Celiac disease
81
Kidneys
Goodpasture’s syndrome
Poststreptococcal glomerulonephritis
82
lungs
Goodpasture’s syndrome
83
Thyroid gland
Graves disease
Hashimoto’s thyroiditis
84
Myelin sheath
of nerves
Multiple sclerosis
85
Nerve-muscle synapses
Myasthenia gravis
86
Stomach
Pernicious anemia
87
Intrahepatic bile ducts
Primary biliary cirrhosis
88
Heart
Rheumatic fever
89
Connective tissue
Scleroderma
90
Eyes, mouth
Sjogren syndrome
91
Pancreas
Type 1 diabetes mellitus
92
Antibody to adrenal cells
Addison’s disease
93
AIH-1—smooth muscle antibodies
Autoimmune hepatitis
94
AIH-2—anti-liver kidney microsomal antibody
Autoimmune hepatitis
95
anti-liver cytosol type 1 antibody (anti-LC-1)
Autoimmune hepatitis
96
Antitransglutaminase (tTG)
Celiac disease
97
Antibodies to deamidated gliadin peptides (DGPs)
Celiac disease
98
Endomysial antibodies
Celiac disease
99
Antibody to an antigen in the renal and
pulmonary basement membranes
Goodpasture’s syndrome
100
Thyroid-stimulating hormone receptor antibodies (TRAbs)
Graves disease
101
Antithyroglobulin
Graves disease
Hashimoto’s thyroiditis
102
Antithyroid peroxidase (TPO)
Graves disease
Hashimoto’s thyroiditis
103
Antibodies to myelin basic protein
Multiple sclerosis
104
Antibodies to acetylcholine receptors (AChR)
Myasthenia gravis
105
Anti-muscle-specific kinase (MuSK)
Myasthenia gravis
106
Antibody to the lipoprotein LRP4
Myasthenia gravis
107
Parietal cell antibody, intrinsic factor
antibody
Pernicious anemia
108
Streptococcal antibodies that cross-react
with kidney tissue
Poststreptococcal glomerulonephritis
109
Antimitochondrial antibodies (AMA)
Primary biliary cirrhosis
110
Streptococcal antibodies that cross-react
with cardiac tissue
Rheumatic fever
111
Antinuclear antibodies: anti-Scl-70
Scleroderma
112
anticentromere antibody
Scleroderma
113
rheumatoid factor
Sjogren
114
antisalivary duct antibodies
Sjogren
115
antilacrimal gland antibodies
Sjogren
116
Anti-insulin
Type 1 diabetes mellitus
117
IA-2 and anti–IA-2Ba
Type 1 diabetes mellitus
118
Antibody to glutamic acid phosphatase (GAD)
Type 1 diabetes mellitus
119
HLA-A1, B8, and DR3
SLE
120
may cause DNA to form thymine dimers
UV light
121
described in anti–TNF-treated rheumatic patients
lupus-like autoantibodies
122
Antibody-forming B lymphocytes are stimulated in a relatively nonspecific manner
polyclonal B cell activation
123
specific for SLE and are observed at a frequency of 75% to 90% in SLE patients with active disease
Anti-ds DNA Auto Ab
124
containing complement-activating IgG3
have a particular tropism for basement membranes
readily deposited in the kidney glomeruli
DNA-anti-DNA immune complexes
125
can also cause cellular destruction by other mechanisms
Autoantibodies to nuclear and nonnuclear antigens
126
can cause hemolytic anemia
Antibodies to red blood cells (RBCs
127
and antibodies
to platelets can cause thrombocytopenia by
antibody-mediated cytotoxic (type II) hypersensitivity
128
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
Antibodies to endothelial cells
129
associated with increased miscarriage, stillbirth, and preterm delivery in pregnant women with lupus
Phospholipid antibodies
130
Neonatal lupus, which occurs in up to 8% of babies born to pregnant women with
SLE, is associated with antibodies to the nuclear antigens,
SS-A/Ro and SS-B/La
131
diverse; nonspecific symptoms
SLE
132
may appear on any area of the body exposed to UV light
erythematous rash
133
butterfly rash (“malar rash”) across the nose and cheeks
Wolf-like
134
increased in those who have antibodies to nuclear RNP in their serum
Raynaud’s phenomenon
135
polyarthralgia or arthritis
Joint involvement
136
Nephritis
Renal involvement
137
the most dangerous type of lesion characterized by cell proliferation
diffuse
proliferative glomerulonephritis
138
pericarditis, tachycardia, ventricular enlargement; pleuritis
cardiac involvement
139
seizures, mild cognitive dysfunction, psychoses, or depression
neuropsychiatric manifestations
140
anemia, leukopenia,
thrombocytopenia, or lymphopenia
Hematologic abnormalities
141
About 20% of episodes of fever are caused by
Infections
142
leading cause of death in hospitalized patients
Infections
143
can interfere with host defense against opportunistic infections
Immunosuppression
144
✓ Complete blood count (CBC) ✓ Platelet count
✓ Urinalysis ✓ Erythrocyte sedimentation rate (ESR) determination ✓ CRP levels
SLE
145
quantification of complement proteins
and the detection of specific autoantibodies
SLE
146
is the most commonly measured complement protein
C3
147
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
Serum complement levels
