Chapter 17 Flashcards
(109 cards)
1
Q
Type II autoimmunity is mediated by?
A
Antibodies
2
Q
The antibodies in type II autoimmunity are specific for?
A
Cell surface & ECM
3
Q
Type III autoimmunities are mediated by?
A
Antibodies
4
Q
Type III autoimmunities are caused by?
A
Immune complexes deposited in tissues
5
Q
Type IV autoimmunity is mediated by?
A
T cells
6
Q
Are there immune diseases mediated by IgE?
A
No
7
Q
5 ways in which we tolerate our own self?
A
- Negative selection in BM and thymus
- Expression of tissue specific proteins in thymus
- No lymphocyte access to some tissues
- Suppression of autoimmune responses by Treg cells
- Induction of anergy in auto reactive B and T cells
8
Q
In this disease, a patient makes autoantibodies for their own erythrocytes
A
Autoimmune hemolytic anemia
9
Q
What happens to the RBC’s in someone with autoimmune hemolytic anemia?
A
They are covered in antibodies or complement and are phagocytosed (in spleen)
10
Q
Result of autoimmune hemolytic anemia?
A
Anemia (duh!!)
11
Q
Test for autoimmune hemolytic anemia?
A
Direct Coomb’s test
12
Q
What would you see in a direct coombs test that was positive for autoimmune hemolytic anemia?
A
Hemagglutination
13
Q
IgG inhibition of an enzyme responsible for cleaving vWF?
A
Autoimmune (Idiopathic) Thrombocytopenic Purpura
14
Q
Clinical presentation of ITP?
A
Bleeding. vWF is not properly cleaved. It is too big so it clots up small vessels. You use up all your platelets and clotting factors in these small clots so you bleed
15
Q
Organs effected in ITP?
A
liver, kidneys, blood vessels, brain
16
Q
Symptoms of ITP?
A
- Thrombocytopenia
- Purpura (bruising)
- Neurological symptoms
- Microangioplastic hemolytic anemia
17
Q
Why do you get anemia in ITP?
A
RBCs are damaged as they are pushed through clots
18
Q
What is microangioplastic hemolytic anemia?
A
Hemolytic anemia (low RBCs due to RBC destruction) microangioplastic (because it is due to clotting in small blood vessels)
19
Q
In ITP, patient would present with?
A
Purpura (bruises), thrombocytopenia (low platelets), anemia (low RBCs), bleeding (due to using up platelets and clotting factors in small blood vessels)
20
Q
Treatment of ITP?
A
Plasmapheresis with plasma from healthy donors
21
Q
Pathogenesis of Goodpasture’s.. What do you make antibodies against?
A
Type IV collagen
22
Q
Where is type IV collagen found?
A
Lines basement membranes throughout the body
23
Q
Clinical presentation of Goodpasture’s?
A
Hemoptysis and hematuria (from Immunopath). When you think Goodpasture, think bloody lungs and bloody kidneys
24
Q
Miller’s notes say that “kidney damage” results from Goodpasture’s. What does this imply?
A
Glomerulonephritis
25
Treatment of Goodpasture?
Plasma exchange and anti-inflammatory drugs
26
Goodpasture is autoimmunity type II, III, or IV?
II
27
Inflammatory destruction of vascular endothelial cells of arterioles and smooth muscle cells; replacement with collagen and other fibrous tissue
Systemic sclerosis (scleroderma)
28
Main symptom?
Hardening/thickening of skin
29
Is rheumatoid factor required for Scleroderma?
No
30
What test do you use to diagnose scleroderma?
1. Anti-Scl70 (anti-DNA topoisomerase)
2. Anti-nuclear
3. Anti-centromere
31
Treatment for Scleroderma?
No cure, no standard treatment. Can only give drugs that increase blood flow to periphery
32
Scleroderma is what type of autoimmune disease?
II
33
This autoimmune disease is caused by antibodies produced from Streptococcus pyogenes doing molecular mimicry and attacking heart cells
Acute rheumatic fever
34
Result of acute rheumatic fever?
Heart valve scarring, myocarditis
35
Acute rheumatic fever is what type of autoimmune disease?
Type II
36
Acute rheumatic fever occurs after?
Strep throat!
37
This autoimmune disease is mediated by IgG specific antibodies for two proteins (desmoglein 1 and 3); results in loss of cohesion of keratinocytes in epidermis
Pemphigus vulgaris
38
Symptom of pemphigus vulgaris?
Painful, chronic blistering of skin
39
Which IgG (1,2,3,4) is thought to be the pathogenic one in the case of pemphigus vulgaris?
IgG4
40
How do you diagnose pemphigus vulgaris?
Punch biopsy of a lesion (skin blister) and immunofluourescent staining
41
How do you treat pemphigus vulgaris?
Corticosteroids and other anti-inflammatory drugs, rituximab
42
Hashimoto's thyroiditis, Grave's disease, Subacute thyroiditis, Idiopathic hypothyroidism are all diseases of what endocrine gland?
Thyroid gland
43
Type I diabetes and type II diabetes are autoimmune diseases of which endocrine gland?
Islets of Langerhans (pancreas)
44
Addison's disease is a disease of which endocrine gland?
Adrenal gland
45
This disease is caused by antibodies specific for self thyroid stimulating hormone (TSH) receptors. The antibody acts as an agonist and stimulates the receptor as if it were binding to its actual ligand
Grave's disease
46
Graves disease- what type?
Type II autoimmue disease
47
Symptoms of graves disease?
Bulging eyes, heat/cold intolerance, irritability, weight loss
48
Treatment of Grave's disease?
Plasmapheresis
49
Antibodies in this disease are specific for Acetylcholine receptors
Myasthenia gravis
50
Clinical presentation with myasthenia gravis?
Muscle weakness
51
Treatment of myasthenia gravis?
Anti-inflammatory drugs and pyridostigimine
52
How does pyridostigimine work?
Inhibits cholinesterase (which degrades acetylcholine) so acetylcholine "lives longer" and has more of a chance to bind to its receptor
53
This disease is mediated by antibodies that bind normal flora (Streptococcus viridans), but are now binding to damaged heart valves
Subacute bacterial endocarditis
54
Which antibody mediates subacute bacterial endocarditis?
IgG
55
T/F This disease usually only occurs after someone has already had damage to their heart valves?
True, typically patient has previously had rheumatic fever or a congenital heart condition
56
How is inflammation mediated in subacute bacterial endocarditis?
Inflammation is mediated by phagocytes that recognize opsonized bacteria; and anaphylatoxins that are produced as a result of the complement cascade
57
Subacute bacterial endocarditis is which type of autoimmune disease?
Type III (immune complex mediated)
58
This disease is characterized by the production of cryoglobulins
Mixed essential cryoglobulinemia
59
What are cryoglobulins?
Immunoglobulins that become insoluble at reduced temperatures
60
What are cryoglobulins called when only the light chain is present?
Bence-Jones proteins
61
Characteristic trait of people with mixed essential cryoglobulinemia?
They usually have a B cell proliferative disorder
62
Along with B cell proliferative disorder, what other infection is common in conjunction with Mixed essential cryoglobulinemia?
Hepatitis C
63
What do cryoglobulins do?
Act like rheumatoid factor and bind to Fc regions of Ig's causing immune complex disorder
64
Symptoms of mixed essential cryoglobulinemia?
Meltzer's triad: purpura, arthalgia, myalgia
65
Mixed essential cryoglobulinemia is what type of autoimmune disease?
Type III (immune complex)
66
The prototypical systemic autoimmune disease. Patient makes autoantibodies for DNA, histones, ribosomes, etc...
Systemic Lupus Erythmatosis
67
How is inflammation initially initiated in SLE?
Auto-antibodies bind to cell surface components which initiates inflammation leading to tissue destruction
68
The damaged cells release macromolecules; immune complexes form and are deposited in blood vessels, kidneys, and joints. What does this lead to?
Further inflammation
69
Treatment of Lupus?
Anti-inflammatory drugs
70
Lupus is what type of autoimmune disease?
Type III (immune complex mediated)
71
This autoimmune disease results in destruction of insulin-producing cells in the pancreas
Insulin dependent diabetes mellitus (type I diabetes)
72
What cells are the effectors of type I diabetes?
CTL's; that makes type I diabetes a type IV autoimmune disease
73
What produces insulin?
Beta cells in islets of Langerhans in the pancreas
74
Treatment for insulin dependent diabetes?
Insulin injections (either from pig or human recombinant insulin)
75
Inflammatory disease of the joints mediated by auto-reactive T cells
Rheumatoid arthritis
76
Describe rheumatoid factor
The production of IgM, IgG, and IgA specific for the Fc region of antibodies
77
Is rheumatoid factor required for rheumatoid arthritis?
No
78
Treatment of rheumatoid arthritis?
Infliximab (anti-TNF Ab)
| Rituximab (uses anti-CD20 ADCC to deplete B cells)
79
Rheumatoid arthritis is what type of autoimmune disease?
Type IV
80
This disease is a T cell mediated auto-reactivity against the exocrine glands that produce tears and saliva
Sjogren's syndrome
81
Symptoms of Sjogrens?
Dry eyes and mouth
82
Is rheumatoid factor required for Sjogrens?
No
83
How do you diagnose Sjogrens syndrome?
Schirmer test to measure tear output; also ANA and rheumatoid factor test (often occurs secondary to rheumatic fever and SLE)
84
Treatment of Sjogrens?
No cure; use artificial tears, wear goggles
85
Sjogrens syndrome is what type of autoimmune disease?
Type IV
86
This autoimmune disease is mediated by Th1 CD4 T cells that are directed towards the myelin sheath of nerve cells
Multiple sclerosis
87
Causes demyelination of white matter in CNS?
MS
88
Symptoms of MS?
motor weakness, impaired vision, lack of coordination, spasticity
89
Is MS early onset, sudden onset, progressive?
Progressive, and the progression is highly variable
90
Treatment of MS?
Immunosuppressive drugs and IFN-beta1
91
This malady occurs in 85% of MS patients
Oligoclonal bands of IgG
92
Do HLA allotypes play a role in autoimmune diseases?
You betcha, for some diseases, if you have a specific haplotype you have an increased risk of getting the disease
93
Position 57 of the DQ-beta chain affects susceptibility to?
Type 1 diabetes
94
Name 3 immunologically privileged sites?
Eyes, testes, placenta/fetus
95
Molecular mimicry- Group A Strep could lead to?
Acute rheumatic fever
96
Chlamydia trachomatis infection can lead to what autoimmune disease?
Reiter's syndrome (arthritis)
97
Shigella flexneri, Salmonella typhimurium, Salmonella enteritidis, Yersinia enterocolitica, Campylobacter jejuni can lead to?
Reactive arthritis
98
Borrelia burgdorferi can lead to?
Chronic arthritis in Lyme disease
99
Coxsackie A virus, Coxsackie B virus, echoviruses, rubella can lead to?
Type 1 diabetes
100
In molecular mimicry, is it the same or a different MHC molecule that presents the self antigen?
The same MHC molecule presents both the infectious antigen and the similar self antigen
101
What 3 diseases from this lecture are diseases of molecular mimicry?
1. Acute rheumatic fever
2. Guillain Barre Syndrome
3. Wegener's Granulomatosis
102
This is a type II autoimmune disease mediated by IgG specific for gangliosides. It results in demyelination of nerve cells
Guillain Barre syndrome
103
Guillain Barre is a disease of molecular mimicry. What infection leads to this disease?
Campylobacter jejuni
104
Symptoms of Guillain Barre?
Symmetrical weakness of lower limbs ascending to upper limbs and face; difficulty swallowing and breathing; drooling; partial paralysis often occurs
105
Treatment for Guillain Barre?
plasma exchange; immunosuppressive drug treatment
106
A disease of molecular mimicry mediated by anti-neutrophil cytoplasmic antibodies (ANCA); it is IgG mediated; most common determinant is proteinase-3
Wegener's Granulomatosis
107
Pathology of Wegener's?
ANCA's bind to neutrophils activating them; the neutrophils up regulate adhesion molecules allowing them to bind to vascular endothelial cells; then the neutrophils degranulate causing damage to the vasculature (vasculitis)
108
Symptoms of Wegener's?
1. Rhinitis & conjunctivitis
2. Lung infiltrates
3. Rapidly progressive glomerulonephritis
4. Granulomas usually found in all affected tissues
109
Treatment of Wegener's?
Plasma exchange; anti-inflammatory
