Autoimmune Disorders Flashcards

(109 cards)

1
Q

Diseases in which immune responses target self-antigens, leading to organ and tissue damage.

A

Autoimmune diseases

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2
Q

The two primary immune mechanisms involved in autoimmune diseases.

A

T cell-mediated immune responses and autoantibodies

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3
Q

The immune system’s ability to avoid attacking its own tissues.

A

Self-tolerance

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4
Q

The location where central tolerance occurs.

A

Primary lymphoid organs (thymus for T cells and bone marrow for B cells)

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5
Q

The elimination of self-reactive T cells during development.

A

Negative selection

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6
Q

The modification of self-reactive B cell receptors to prevent autoimmunity.

A

Receptor editing

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7
Q

A state of unresponsiveness in weakly self-reactive B or T cells.

A

Anergy

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8
Q

The immune cells that suppress immune responses to prevent autoimmunity.

A

Regulatory T cells

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9
Q

The three key mechanisms involved in peripheral tolerance.

A

Anergy, inhibition by regulatory T cells, and apoptosis

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10
Q

The main factors that contribute to autoimmune diseases.

A

Genetics, environmental factors, and immune regulation defects

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11
Q

Certain genes increase susceptibility to this group of diseases.

A

Autoimmune diseases

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12
Q

Infections, toxins, and dietary components that can trigger autoimmunity.

A

Environmental factors

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13
Q

The process in which the immune system mistakenly attacks the body’s own tissues.

A

Immune regulation defects

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14
Q

The theory that suggests autoimmunity arises due to mutations creating altered lymphocyte clones.

A

Forbidden Clone Theory

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15
Q

The theory that states self-antigens become foreign due to physical, chemical, or biological changes.

A

Altered Antigen Theory

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16
Q

The theory in which trauma or infection exposes hidden antigens, triggering an immune response.

A

Sequestered Antigen Theory

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17
Q

The theory that suggests autoimmunity results from defects or deficiencies in immune regulation.

A

Immunologic Deficiency Theory

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18
Q

The theory that explains how foreign antigens share epitopes with self-antigens, leading to immune cross-reactivity.

A

Cross-Reactive Antigen Theory

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19
Q

A chronic autoimmune disease of unknown origin that primarily manifests as vasculitis and can affect various organs and tissues.

A

Systemic Lupus Erythematosus (SLE)

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20
Q

The human leukocyte antigen (HLA) markers associated with SLE.

A

HLA-A1, HLA-B8, and HLA-DR3

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21
Q

The hallmark autoantibodies of SLE.

A

Anti-nuclear antibodies (ANA)

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22
Q

The demographic group primarily affected by SLE.

A

Women, with a strong hereditary tendency

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23
Q

A red rash across the nose and upper cheeks commonly seen in SLE.

A

Butterfly rash

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24
Q

The most common cause of morbidity and mortality in SLE.

A

Nephritis (diffuse proliferative glomerulonephritis)

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25
The autoantibodies that target antigens in the nuclei of mammalian cells and are not specific to SLE.
Antinuclear antibodies (ANA)
26
The autoantibody most specific to SLE and correlates with disease activity.
Anti-double-stranded DNA (Anti-dsDNA)
27
The autoantibody associated with drug-induced SLE.
Anti-histone
28
The autoantibody that targets the DNA-histone complex (nucleosomes) and is associated with both SLE and drug-induced SLE.
Anti-deoxyribonucleoprotein (Anti-DNP)
29
The autoantibody diagnostic of SLE that targets uridine-rich RNA.
Anti-Smith (Anti-Sm)
30
Common laboratory tests used in the diagnosis of SLE.
CBC Urinalysis ESR C3 measurement
31
What is the most widely used method for detecting antinuclear antibodies (ANA) due to its high sensitivity?
Indirect immunofluorescence assay (IFA)
32
What is the standard substrate used in fluorescent ANA testing?
HEp-2 cells
33
What fluorescent ANA pattern shows uniform staining of the entire nucleus?
Homogeneous (diffuse) pattern
34
What autoantibodies are associated with the homogeneous ANA pattern?
Anti-double-stranded DNA (Anti-dsDNA) Anti-histone Anti-deoxyribonucleoprotein (Anti-DNP)
35
What fluorescent ANA pattern shows greater staining intensity around the outer circle of the nucleus and is highly specific for SLE?
Peripheral (rim/outline) pattern
36
What autoantibody is associated with the peripheral ANA pattern?
Anti-double-stranded DNA (Anti-dsDNA)
37
What fluorescent ANA pattern consists of discrete fluorescent specks throughout the nuclei, without staining the nucleolus or chromatin region?
Speckled pattern
38
What autoantibodies are associated with the speckled ANA pattern?
Extractable nuclear antigens (ENA)
39
What fluorescent ANA pattern shows prominent staining of the nucleoli within the nucleus?
Nucleolar pattern
40
What autoantibodies are associated with the nucleolar ANA pattern?
Anti-ribonucleoprotein (Anti-RNP) Anti-RNA
41
What fluorescent ANA pattern shows discrete speckles in the nuclei during interphase and chromatin of dividing cells?
Centromere pattern
42
What autoantibody is associated with the centromere ANA pattern?
Anti-centromere
43
What are two immunoassay techniques used for detecting antinuclear antibodies (ANA)?
Enzyme immunoassay (EIA) Chemiluminescent immunoassay (ChLIA)
44
What is the microsphere-based method used for ANA detection?
Microsphere multiplex immunoassay (MIA)
45
What fluorescence-based test uses Crithidia species for ANA detection?
Crithidia luciliae Immunofluorescence
46
What outdated method was historically used for ANA detection?
Ouchterlony double diffusion
47
What is a chronic inflammatory disease that primarily affects joints and periarticular tissues but can also involve the heart, blood vessels, and lungs?
Rheumatoid arthritis (RA)
48
What happens to the articular cartilage in rheumatoid arthritis?
It becomes replaced by fibroid granulation tissue.
49
What are the key autoantibodies associated with rheumatoid arthritis?
Rheumatoid factor (RF) Anti-cyclic citrullinated peptide (Anti-CCP)
50
An IgM autoantibody that reacts with the Fc portion of IgG.
Rheumatoid factor (RF)
51
What tests are used to detect rheumatoid factor (RF)?
Manual agglutination using charcoal or latex particles Enzyme-linked immunosorbent assay (ELISA) Chemiluminescent immunoassay (ChLIA) Nephelometry
52
An atypical amino acid formed from arginine by peptidyl arginine deiminase.
Citrulline
53
Where is citrulline found?
Granulocytes Monocytes Macrophages
54
What laboratory test is used to detect anti-cyclic citrullinated peptide (Anti-CCP) antibodies?
Enzyme-linked immunosorbent assay (ELISA)
55
What diseases are associated with anti-neutrophil cytoplasmic antibodies (ANCA)?
Wegener's granulomatosis Microscopic polyangiitis (MPA) Churg-Strauss Syndrome
56
What method is used to screen for anti-neutrophil cytoplasmic antibodies (ANCA)?
Indirect immunofluorescence (IIF) using ethanol-fixed leukocytes
57
What are the two fluorescence patterns observed in ANCA testing?
Cytoplasmic (c-ANCA) Perinuclear (p-ANCA)
58
What ANCA pattern is characterized by diffuse, granular staining in the neutrophil cytoplasm?
Cytoplasmic (c-ANCA)
59
What autoantibody causes the cytoplasmic ANCA (c-ANCA) pattern?
Proteinase 3 (PR3) ANCA
60
What disease is associated with cytoplasmic ANCA (c-ANCA)?
Granulomatosis with polyangiitis (GPA/Wegener’s granulomatosis)
61
What ANCA pattern shows fluorescence surrounding the lobes of the neutrophil nucleus?
Perinuclear (p-ANCA)
62
What causes perinuclear ANCA (p-ANCA)?
Antibodies against positively charged antigens
63
What diseases are associated with perinuclear ANCA (p-ANCA)?
Microscopic polyangiitis (MPA) EGPA/Churg-Strauss Syndrome
64
What type of autoimmune hemolytic anemia is IgG-mediated and may be idiopathic or secondary?
Warm autoimmune hemolytic anemia (Warm AIHA)
65
What type of autoimmune hemolytic anemia is IgM-mediated and associated with primary atypical pneumonia?
Cold autoimmune hemolytic anemia (Cold AIHA)
66
What percentage of warm autoimmune hemolytic anemia cases are idiopathic?
1/2 of the cases
67
What condition is cold autoimmune hemolytic anemia linked to?
Cold agglutinin disease
68
What autoimmune hemolytic anemia is mediated by cold-reacting IgG?
Paroxysmal Cold Hemoglobinuria (PCH)
69
What antigen is targeted in Paroxysmal Cold Hemoglobinuria (PCH)?
P antigen
70
What is the temperature-dependent behavior of the biphasic antibody in PCH?
Sensitizes red blood cells at 4°C Causes hemolysis at 37°C
71
What laboratory test is used to demonstrate Paroxysmal Cold Hemoglobinuria (PCH)?
Donath-Landsteiner test
72
What autoimmune disease causes thyroid destruction and hypothyroidism?
Hashimoto’s Thyroiditis
73
What histological changes occur in Hashimoto’s Thyroiditis?
Lymphocytic and plasma cell infiltration Germinal centers replace thyroid tissue
74
What autoantibodies are present in Hashimoto’s Thyroiditis?
Anti-thyroglobulin (Tg) Anti-thyroid peroxidase (TPO)
75
How does Hashimoto’s differ from Graves’ Disease?
Hashimoto’s: Hypothyroidism Graves’: Hyperthyroidism
76
What autoimmune disease causes hyperthyroidism?
Graves' Disease
77
What autoantibody overstimulates the thyroid?
TSH receptor antibodies (TRAbs)
78
What are the treatments for Graves’ Disease?
Surgery Radioactive iodine
79
What is another name for Type I Diabetes Mellitus?
Insulin-dependent diabetes mellitus (IDDM) Juvenile-onset diabetes mellitus
80
What causes Type I Diabetes Mellitus?
Autoimmune destruction of pancreatic beta cells.
81
What autoantibodies are linked to Type I Diabetes Mellitus?
Anti-ICA512 Anti-IAA Anti-GAD65 Anti-IA-2β
82
When does hyperglycemia occur in T1DM?
After most beta cells are destroyed.
83
What protein triggers celiac disease?
Gluten (in wheat, barley, rye)
84
Why is gliadin significant in celiac disease?
It becomes more immunogenic after modification by tissue transglutaminase.
85
What genetic markers are linked to celiac disease?
HLA-DQ2 HLA-DQ8
86
What does celiac disease damage?
Intestinal mucosa
87
What antibodies are found in celiac disease?
Anti-deamidated gliadin peptides Anti-tissue transglutaminase Anti-endomysium
88
What was Autoimmune Hepatitis (AIH) formerly called?
Chronic active hepatitis
89
What HLA markers are associated with AIH?
HLA-DRB1 HLA-DQB1
90
What autoantibodies are present in AIH?
Anti-smooth muscle (SMA) Anti-liver kidney microsomal (anti-LKM-1) Anti-liver cytosol type 1 (anti-LC-1) Anti-mitochondrial (AMA)
91
What is the most common autoimmune liver disease?
Primary Biliary Cirrhosis (PBC)
92
What is the pathology of PBC?
Progressive destruction of intrahepatic bile ducts
93
What HLA markers are linked to PBC?
HLA-DRB1 HLA-DQA1 HLA-DPB1 HLA-DQB1
94
What autoantibody is characteristic of PBC?
Anti-mitochondrial antibody (AMA)
95
What neuromuscular disorder causes muscle weakness and fatigue?
Myasthenia Gravis
96
What autoantibody is found in Myasthenia Gravis?
Anti-acetylcholine receptor (Anti-AChR)
97
Where are AChRs located?
Skeletal muscles
98
What is the function of AChRs?
Bind acetylcholine to trigger muscle contractions
99
Why does Myasthenia Gravis cause muscle weakness?
Block or destroy acetylcholine receptors
100
What percentage of Myasthenia Gravis patients have Anti-AChR?
90%
101
What autoimmune disease affects the brain and spinal cord white matter?
Multiple Sclerosis
102
Why is Multiple Sclerosis considered an autoimmune disease?
T-cell attack CNS
103
What immunoglobulin is abnormally produced?
IgG
104
What test detects oligoclonal bands?
CSF electrophoresis
105
How do oligoclonal bands appear in normal vs. abnormal conditions?
Normal: Absent in CSF & plasma Abnormal: Present in CSF, absent in plasma
106
What autoantibody is present in Goodpasture’s Syndrome?
Anti-GBM
107
Why does Goodpasture’s Syndrome cause kidney & lung damage?
Anti-GBM attacks basement membranes
108
What are the renal & pulmonary symptoms?
Renal: Hematuria, proteinuria, low creatinine clearance, uremia Pulmonary: Cough, SOB, hemoptysis
109
What HLA marker is linked to Goodpasture’s Syndrome?
HLA-DRB1-15