Immunopathology Flashcards
1
Q
List types of PRRs
A
1) Toll-Like
2) NOD-Like
3) C-type Lectin
2
Q
Activation of NK cells
A
1) MHC I
2) NKG2D Ligands
3
Q
Inhibition of NK cells
A
Presentation of MHC I
4
Q
Describe Type I Hypersensitivity
A
1) IgE Mediated
2) Sensitization and Second exposure
5
Q
Function of Leukotrienes and Prostaglandins
A
1) Leukocyte Infiltration
2) Epithelial Damage
3) Bronchospasm
6
Q
Histamine Function
A
Vasodilation
Vascular Leakage
smooth Muscle Spasm
7
Q
What causes late phase reaction in Type I hypersensitivity
A
Cytokines –> leukocyte recruitment
8
Q
What cells are recruited during Type I hypersensitivity
A
Eosinophils
Basophils
Neutrophils
9
Q
Examples of type I hypersensitivity
A
1) Asthma
2) Seasonal Allergies
3) Food allergies
4) Severe Allergic rxns
5) eosinophilic esophagitis
6) skin prick test
10
Q
Characteristics of Asthma
A
Bronchoconstriction
Bowel peristalsis
Vasodilation Permeability
11
Q
List some Severe Allergic Reactions
A
1) Urticaria
2) Angioedema
3) Anaphylaxis
12
Q
Describe Eosinophilic Esophagitis
A
Recurrent Dysphagia Weight Loss (due to painful to swallow)
13
Q
What causes Eosinophilic Esophagitis
A
Food Driven antigen
14
Q
Morphology of Type II Hypersensitivity
A
Smooth Linear
15
Q
Mechanism of Type II Hypersensitivity
A
1) Opsonization and Phagocytosis
2) Complement and Fc Receptor-mediated Inflammation
3) Antibody-Mediated Cellular Dysfunction
16
Q
Type II Hypersensitivity: Opsonization/Phagocytosis Examples
A
1) Autoimmune Hemolytic Anemia
- RBC membrane Proteins (Rh; I antigen)
2) Autoimmune thrombocytopenia Purpura
- GpIIb:IIIa
17
Q
Type II Hypersensitivity: Complement and Fc receptor-mediated inflammation Mechanism
A
1) Basement membrane proteins
2) Streptococcal Proteins that Cross-react w/ Myocardium
18
Q
Describe a Lentivirus
A
Goes under extensive latency
19
Q
Type II Hypersensitivity: Complement and Fc receptor-mediated inflammation examples
A
1) Vasculitis
2) Goodpasture
3) Acute Rheumatic Fever
20
Q
What is the cause of Vasculitis
A
Neutrophil Degranulation
21
Q
What type of antibodies are present in vasculitis
A
ANA
22
Q
MOA of Good pastures syndrome
A
1) Type II Hypersensitivity
2) Basement Membrane
23
Q
What are some common clinical manifestations in Goodpasture’s syndrome
A
1) Nephritis
2) Lung Hemorrhage
24
Q
MOA of Acute Rheumatic Fever?
A
Molecular Mimicry
25
Clinical Manifestations of Acute Rheumatic Fever
Arthritis
26
Type II Hypersensitivity: Anti-Mediated Cellular Dysfunction mechanisms
1) Disrupted Endocrine Signaling
| 2) Disrupted Neural Signaling
27
Type II Hypersensitivity: Anti-Mediated Cellular Dysfunction Examples
1) Disrupted Endocrine Signaling
- Graves Disease
- Insulin-Resistant Diabetes
2) Disrupted Neural Signal
- Myasthenia Gravis
28
What is the MOA of Graves Disease
Ab to TSH receptor
29
MOA of Type II Diabetes
Ab to insulin
30
MOA of Myasthenia Gravis
Ab inhibits ACh receptors
31
MOA of Type III Hypersensitivity
Ag-Ab Complexes
32
Morphology of Type III Hypersensitivity
Granular
33
Examples of Type III Hypersensitivity
1) Serum Sickness
2) Arthus Reaction - Local Rxn
3) Lupus Erythematosus
34
MOA of Type IV hypersensitivity
Cell Mediated (T Cell-mediated)
35
Morphology of Type IV Hypersenitivity
Immune Granulomas
36
Examples of Type IV Hypersensitivity
1) Type I Diabetes
2) RA
3) MS
4) IBD
5) Psoriasis
6) Contact Sensitivity
37
Inhibitory receptors and their ligands
1) CD80/86 and CTLA4
| 2) PDL-1/2 and PD-1
38
Which inhibitory receptors/ligands do APCs have?
1) CD80/86
| 2) PDL-1/2
39
Ankylosing SPondylitis is associated with which type of gene?
HLA- B27
40
Clinical Manifestations of Ankylosing Spondylitis
1) Neck and Lower Back Pain
| 2) Bamboo Spine
41
Pathogenesis of Ankylosing Spondylitis
Vertebral Disc Degeneration
42
Etiology of Rheumatoid Arthritis
1) Abnormal B and T Cells
2) Type IV Hypersensitivity
3) Absence of Central/Peripheral Tolerance
43
Genetic association of RA
PTPN22 Polymorphism
44
Clinical Manifestation of RA
1) Swollen Joints
2) Stiffness
3) Redness
45
What cell dysfunction is present in chron's disease
Paneth Cells
46
What receptor is dysfunction in Chrons
NOD-2
47
Pathogenesis of Chron's Disaese
Microbial Overgrowth
48
Clinical Presentations of Rheumatic Fever
1) Pharyngitis
2) PGST Complication
3) Heart Valve Destruction
49
MOA of Oral Lichen planus
Epitope Spreading
50
Etiology of Oral Lichen Planus
Epithelial Damage due to T Cell response
51
Pathogenesis of Oral Lichen Planus
Basement membrane destruction --> DAMPs
52
Exposure to DAMPs leads to a ___ B cell response
Secondary
53
Clinical Manifestaitons of Oral Lichen Palnus
1) Keratosis
| 2) Secondary Pemphigoid --> Blistering
54
List ANA + Disorders
1) Lupus
2) Systemic Sclerosis
3) Sjogren Syndrome
55
What are ANA disorders
Abs against nuclear content w/in nucleus of cells
56
How would you diagnose:
1) Lupus
2) Systemic Sclerosis
3) Sjogren Syndrome
1) Lupus: Anti DS DNA and Anti Smith
2) Systemic Sclerosis: Anti DNA Topoisomerase (ScI-70)
3) Sjogren Syndrome: Anti Ro/SS-A and Anti La/SS-B
57
What is the ANA pattern in Lupus?
Homogenous
58
What is the ANA pattern in Systemic Sclerosis
Nucleolar
59
What is the ANA pattern in Sjogren
Speckled
60
Young female pt presents with Malar Rash, Renal and/or Heart
Systemic Lupus Erythematosus
61
What is Diffuse Lupus?
Lupus Nephritis
62
Pathogenesis of Diffuse Lupus
1) Increased Cellularity
| 2) Type II HS: Immune Deposits
63
Clinical Manifestations of Diffuse Lupus
1) Proteinuria
| 2) Hematuria
64
Cardiovascular Complications in Systemic Lupus
Libman-Sacks Endocarditis
65
Pathogenesis of Libman-Sacks Endocarditis
Warty valve deposits of fibrin - LSE
66
Morphology of Libman-Sacks Endocarditis
L-E Cells: Neutrophil or Macrophage engulfed damage lymphocytes
67
When you suspect amyloid and see a bubble gum pink appearance what would you want to do next?
Congo Red Stain
68
Lab Findings for Discoid Lupus
1) + ANA
| 2) - DS DNA
69
Clinical Manifestations of Discoid Lupus
Disseminated Skin Lesion: Looks similar to burns and contracted appearance
70
Common Causes for Graft V. Disease
1) bone Marrow
2) Thymus
3) Liver Transplant
*** Anything with increased immune cells
71
Manifestation of Graft v. Host Disease.
1) Skin: Rash --> Desquamation
2) Liver - Jaundice --> Cholestasis
3) Intestines - Bloody Diarrhea --> Strictures
72
Lab finding for Drug-induced Lupus
+ Anti-Histone Ab
73
Clinical Manifestations of Drug induced lupus
1) Cutaneous Lesions
| 2) Arthralgias and Fever
74
Clinical Manifestations of Sjogren Syndrome
1) Dry, Irritated Eyes
- Sand in eyes
2) Dry Mouth (xerostomia) and Difficulty Swallowing
3) Root Caries
4) Smooth Tongue w. Candida Yeast
75
Complications of Sjogren Syndrome
1) Pulmonary Fibrosis
| 2) Lymphoma
76
Etiology Sjogren Syndrome
Lacrimal and Salivary Gland Tissue Destruction
77
Pathogenesis of Sjogren Syndrome
B and T- Cell mediated inflammatory rxn
| - Fibrotic Destruction Follows
78
Systemic Sclerosis Morphology
1) Loss of Subcutaneous Tissue
- Replaced by collagen and fibrosis
2) Vascular Hyalinization
79
Clinical Features of Systemic Sclerosis
1) Sclerodactyly
2) Raynaud Phenomenon
- Episodic vessel obstruction in hands
- Leads to Necrosis
3) Gastrointestinal Tract
- GERD
- Esophageal Ulceration
4) Renal Vascular Disease
5) Pulmonary
- Pulmonary HTN
- Pulmonary Fibrosis
80
CREST Syndrome ANA Pattern
Centromere
81
What is CREST Syndrome
limited sclerosis w/ less visceral involvement (Better prognosis)
82
Clinical Manifestations of CREST
1) Calcinosis
2) Raynaud's Phenomenon
3) Esophageal Dysfunction
4) Sclerodactyly
5) Telangiectasias - Vascular Malformations
- Blotchy Skin Appearance
83
IgG4-Releated Disease Etiology
Autoinflammatory Disease
84
Pathogenesis of IgG4 Related Disease
IgG4 - producing plasma cells --> Fibrosis
85
Clinical Manifestations of IgG4 Related Disease
Autoimmune Pancreatitis
Fibrous Bile Duct: Sclerosing Cholangitis
Submandibular Fibrosis w/ IgG4 abs
86
Types of Allorecognition
1) Direct
| 2) Indirect
87
Types of Direct Allorecognition
1) Acute
| 2) Chronic
88
Types of Indirect Allorecognition
1) Hyperacute Rejection
| 2) Acute/Chronic Antibody-Mediated Rejection
89
Describe Acute Cellular Rejection
W/in days months and years of transplant
90
Clinical Manifestation of Chronic Cellular Manifestation
Arteriosclerosis
91
Morphology of Chronic Cellular Rejection
Fibrosis/Sclerosis
92
Pathogenesis of acute/chronic antibody mediated rejection
Inflammation and Complement
93
What allows you to detect an acute/chronic antibody-mediated rejection
C4d
94
List Treatments for Rejections
1) Corticosteroids
2) Tacrolimus
3) Rituximab (anti CD20 recombinant ab)
95
____ inhibits T cell proliferation and is important for Cell Mediated Rejection
Tacrolimus
96
____ inhibits reactive B cells
Rituximab
97
Manifestations of Graft v. Host disease
1) Skin: Rash --> Desquamation
2) Liver: Jaundice --> Cholestasis
3) Intestine: Bloody Diarrhea --> Strictures
98
Clinical Manifestations of Chediak-Higshi Syndrome
1) Grey Hair
2) Albinism
3) Easy Bleeding
99
Morphology of Chediak-Higshi Syndrome
Giant Granules in Leukocytes
100
Complication of Chediak-Higshi Syndrome
1) Increased bacterial infecion risk w/ catalase - bacteria
- Streptococcus
- Enterococcus
- Staphylococcus
101
MAC def leads to
Sus. Neisseria Infections
| -Meningitis
102
Pathogenesis and Inheritance of Chediak-Higshi
1) AR
| 2) Failed Phagolysosome
103
Inheritance of Hereditary Angioedema
1) AD
104
PAthogenesis of Hereditary Angioedema
Def. C1-INH
105
Clinical manifestations of Hereditary Angioedema
1) Kinin-Mediated Edema at multiple sites
| 2) Corkscrew pattern of bowel on CT
106
Types of Leukocyte Adhesion Deficiency
1) LAD Type I: Lack of selectin (no Fucosyl Transferase)
| 2) LAD Type 2: Defective B2 integrin
107
Severe Combined Immunodeficiency Inheritance
X Linked or Autosomal Recessive
108
Population for Severe Combined Immunodeficiency X Linked
Males
109
Pathogenesis of Severe Combined Immunodeficiency : X Linked
Mutated IL receptors
| - Reduced T Cell # --> no b cell response
110
Autosomal Recessive SCIDS Pathogenesis
1) Adenosine Deaminase Deficiency
- Toxic Purine Accumulation
- Blocks t Cell formation
- Suppresses Humoral Response
111
Gene associated with X-Linked Agammaglobulinemia
BTK (Burotn Tyrosine Kinase) on X Chromosome
112
1) Pathogenesis of X-Linked Agammaglobulinemia
| 2) Complications
1) B cells can't mature ( no light chain rearrangement)
| 2) Increased infections w/ encapsulated bacteria, viruses and protozoa
113
Gene associated with DiGeorge
22q11 Deletion
114
Clinical Manifestations of DiGeorge Syndrome
1) Underdeveloped Thymus
2) Hypoparathyroidism --> Hypocalcemia --> Tetany
3) Heart and Great Vessel Abnormalities
115
Hyper-IgM Syndrome Pathogensis
CD40/CD40L Mutation: Can't class switch
116
Complications of Hyper IgM
Encapsulated Bacterial Infections: Lack of opsonizing antibodies
117
Isolated IgA syndrome conplication
1) Less defense against inhaled and ingested pathogens
118
Clinical Manifestations of Isolated IgA deficiency
1) Sinus and Respiratory Infections
2) Urinary bladder infections
3) Gastrointestinal infectionss
4) Autoimmune disease and allergies
119
When someone has isolated IgA def. and gets a blood transfusion ... what occurs
Anaphylactic Rxn
120
Gene association with Wiskott Aldrich Syndrome
WASP
121
Clinical Manifestations of Wiskott Aldrich
1) thrombocytopenia
2) eczema
3) recurrent infections
122
Pathogenesis of Wiskott-Aldrich
1) T cell loss
| 2) Hypogammaglobulinemia
123
Ataxia-telangiectasia is a def, of what?
1) IgG
| 2) IgA
124
Consequences of Ataxia-telangiectasia
1) Respiratory infections
2) autoimmune disease
3) cancer
125
Inheritance of Ataxis Telangiectasia
AR
126
Gene mutation associated with Ataxia Telangiectasia
ATM
| - Defective DNA repair
