Diseases of the Immune System Flashcards
(204 cards)
1
Q
protection from infectious pathogens
A
immunity
2
Q
Body’s first line of defense
A
innate immunity
3
Q
mechanisms that are ready to react to infections even before they occur, and that have evolved to specifically recognize and combat microbes
A
innate immunity
4
Q
mechanisms that are stimulated by (“adapt to”) microbes and are capable of recognizing microbial and nonmicrobial substances
A
adaptive immunity
5
Q
what constitutes innate immunity?
A
- recognition of microbes and damaged cells
- activation of various mechanisms
- elimination of the unwanted substances
6
Q
components of innate immunity
A
epithelia monocytes and neutrophils dendritic cells natural killer cells mast cells innate lymphoid cells soluble proteins
7
Q
acts as mechanical barriers to the entry of microbes from the outside environment
A
EPITHELIA of the skin, gi, and respiratory tracts
8
Q
are phagocytes in the blood that can be rapidly recruited in sites of infection
A
Monocytes and neutrophils
9
Q
antigen presenting function; sense microbes
and cell damage and stimulate the secretion of cytokines
A
Dendritic cells
10
Q
provide early protection against many viruses and intracellular bacteria
A
NK cells
11
Q
produce mediators of inflammation
A
Mast cells
12
Q
protects against extracellular microbes and their toxins
A
Humoral immunity
13
Q
mediated by B lymphocytes
A
Humoral immunity
14
Q
secreted products of humoral immunity
A
antibodies/ immunoglobulins
15
Q
for defense against intracellular microbes
A
Cell-mediated (or cellular) immunity
16
Q
mediated by T lymphocytes
A
Cell-mediated (or cellular) immunity
17
Q
constitute 60-70% of lymphocytes in the blood
A
T lymphocytes
18
Q
types of T lymphocytes
A
cytotoxic, helper and regulatory T cells
19
Q
where do T lymphocytes develop
A
thymus
20
Q
T lymphocytes are mainly seen where
A
in paracortex of lymph nodes, periarteriolar lymphoid sheaths of spleen
21
Q
recognize a cell-bound antigen by means of an antigen-specific T-cell receptor (TCR)
A
T lymphocytes
22
Q
co-receptors in T-cell
activation
A
CD4 and CD8
23
Q
recognize antigens displayed by MHC molecules on the surfaces of antigen- presenting cells
A
αβ TCR
24
Q
CD4 binds to
A
Class II Membrane Histocompatibility Complex
25
CD8 binds to
Class I MHC
26
constitute 10-20% of circulating lymphocytes
B lymphocytes
27
B lymphocytes are found
in peripheral lymphoid tissues (follicles of the cortex of lymph nodes and spleen, and mucosa-associated lymphoid tissues)
28
recognize antigen via the B-cell antigen receptor complex (Membrane-bound IgM and IgD)
B lymphocytes
29
develop into plasma cells that secrete antibodies
B lymphocytes
30
present antigens to T cells when recruited in T-cell zones of lymphoid organs
Dendritic cells (Interdigitating dendritic cells)
31
example of dendritic cells
Langerhans cells
32
present in the germinal centers of lymphoid follicles in the spleen and lymph nodes
- present antigens to B cells
Follicular dendritic cells
33
APC that are capable of phagocytosis
Macrophages
34
key effector cells in certain forms of cell- mediated immunity
Macrophages
35
participate in the effector phase of humoral immunity
Macrophages
36
destroy abnormal cells without prior exposure
Natural Killer Cells
37
aka large granular lymphocytes
Natural Killer Cells
38
enumerate cells of the immune system
```
T lymphocytes
B lymphocytes
Dendritic cells
macrophage
NK cells
```
39
primary, or central lymphoid organs (thymus & bone marrow)
Generative Lymphoid organs
40
where T and B lymphocytes develop or mature
Generative Lymphoid organs-
41
secondary lymphoid organs where adaptive immune response are initiated
Peripheral lymphoid organs
42
- concentrate antigens, APCs, and lymphocytes
Peripheral lymphoid organs
43
Peripheral lymphoid organs
lymph node
spleen
Cutaneous and mucosal lymphoid systems
44
concentrate antigens from lymph
lymph node
45
trap blood-borne antigens
spleen
46
(GI and respiratory tracts, pharyngeal tonsils, Peyer’s patches)
Cutaneous and mucosal lymphoid systems
47
excessive and harmful reaction to antigens
hypersensitivity
48
Elicited by exogenous environmental antigens or endogenous self antigens
hypersensitivity
49
Imbalance between the effector mechanisms and the control mechanisms
hypersensitivy
50
Associated with the inheritance of particular susceptibility genes
hypersensitivity
51
Mechanisms of tissue injury are the same as the effector mechanisms of defense
hypersensitivity
52
types of hypersensitivity diseases
- Immediate Hypersensitivity (Type I Hypersensitivity)
- Antibody-mediated Disorders (Type II Hypersensitivity)
- Immune Complex–mediated Disorders (Type III Hypersensitivity)
- Cell-mediated Immune Disorders (Type IV Hypersensitivity)
53
TH2 cells, igE antibodies, mast cells, other leukocytes
type 1
54
secreted antibodies igM and igG injure cells by promoting phagocytosis and injure tissue by inducing inflammation
type 2
55
igM and igG will form a antigen-antibody complex that will be deposited in the tissues and induce inflammation affecting many organs in the body
type 3
56
sensitized t lymphocytes ( th1, th17, cytotoxic t cells) cause of tissue injury
type 4
57
allergy
type 1
58
Rapid immunologic reaction occurring in a previously
| sensitized individual
type 1
59
Triggered by the binding of an antigen to IgE antibody on the surface of mast cells
type 1
60
Systemic disorder (anaphylactic shock) or local reaction (localized cutaneous rash or blisters, hives, skin allergies, nasal and conjunctival discharge in allergic rhinitis and conjunctivitis, bronchial asthma, hay fever, allergic gastroenteritis in food allergies
type 1
61
characterized by vasodilation, vascular leakage, and smooth muscle spasm or glandular secretions
Immediate reaction
62
characterized by infiltration of tissues with eosinophils, neutrophils, basophils, monocytes, and CD4+ T cells, as well as tissue destruction
Late-phase reaction
63
Most immediate hypersensitivity disorders are caused by
excessive TH2 responses which stimulate IgE production and promote inflammation
64
first to be released mediator of hypersensitivity
Preformed Mediators
65
Preformed Mediators
Vasoactive amines (Histamine)
Enzymes
Proteoglycans
66
cause intense sm contraction, increase vascular permeability and increase mucus secretions
histamine
67
cause tissue damage leading to degeneration of kinins and activated components of complement
enzymes
68
heparin; package and store amines in the granules
proteoglycans
69
Lipid Mediators
Leukotriene B4, C4, D4
Prostaglandin D2
Platelet-activating factor
70
Cytokines
TNF, IL-1, chemokines, IL-4
71
most potent, vasoactive and spasmogenic agent known. they are more active than histamine in increasing vascular permeability and cause bronchial smooth muscle contraction
leukotriene c4 and d4
72
highly chemotactic for neutophils, eosinophils and monocytes
leukotriene b4
73
most abundant mediators produced in mast cells by the cyclooxigenase pathway and causes intense bronchus spasm and increase mucus secretion
prostaglandin d2
74
produced by some mast cells population. causes platelet aggregation, release of histamine, bronchus spasm, increase vascular permeability and vasodilation
platelet-aggregating factor
75
promotes leukocyte recruitment
chemokines
76
amplify th2 response
IL4
77
characterized by fall in blood pressure (shock) caused by vascular dilation; airway obstruction due to laryngeal edema
anaphylaxis
78
characterized by airway obstruction caused by bronchial smooth muscle hyperactivity; inflammation and tissue injury caused by late phase reaction
bronchial asthma
79
characterized by increase mucus secretion; inflammation of upper airways, sinuses
allergic rhinitis, sinusitis (hay fever)
80
characterized by increased peristalsis due to contraction of intestinal muscles
food allergies
81
type II hypersensitivity
Antibody-Mediated (Type II) Hypersensitivity
Opsonization and Phagocytosis Inflammation
Cellular Dysfunction
82
antigen combines with antibody in the circulation
type 3
83
complexes may be formed at sites where antigen has been “planted” previously
type 3
84
caused by inflammation resulting from cytokines produced by CD4+ T cells and cell killing by CD8+ T cells
type 4
85
Affect the humoral and/or cellular arms of adaptive immunity
| or the defense mechanisms of innate immunity
Primary immunodeficiency disorders
86
Most are detected in infancy between 6 months and 2 years of life
Primary immunodeficiency disorders
87
May arise as complications of cancers, infections, malnutrition, or side-effects of immunosuppression, irradiation, or chemotherapy for cancer and other diseases
Secondary immunodeficiency states
88
AIDS
Secondary immunodeficiency states
89
caused by Human immunodeficiency virus (HIV)
AIDS
90
non transforming human retro virus, lenti virus family
HIV
91
types of HIV
HIV-1 and HIV-2
92
most common type of HIV associated with AIDS in US, EUROPE, CENTRAL AFRICA
HIV 1
93
causes AIDS in WEST AFRICA and INDIA
HIV 2
94
two major targets of AIDS
Immune system and the CNS
95
hallmark of AIDS
Profound immune deficiency, primarily affecting cell- mediated immunity
96
structures of HIV
virus core
p17
virion envelope
viral glycoproteins gp120 and gp141
97
the most readily detected viral antigen and is the target for the antibodies that are used for the diagnosis of HIV infection
major capsid protein p24 ( virus core)
98
3 viral enzymes
protease, reverse transcriptase, and integrase
99
what are found in the virus core
major capsid protein p24
2 copies of genomic RNA
nucleocapsid protein p7/p9
3 viral enzymes
100
matrix protein that surrounds viral core
p17
101
critical for HIV infection of cells.
Viral glycoproteins gp120 and gp41
102
epidemiology of AIDS
- Homosexual or bisexual men
- Intravenous drug abusers
- Hemophiliacs
- Recipients of blood and blood components
- Heterosexual contacts of members of other high-risk groups
- HIV infection of the newborn
103
constitute the largest group of people affected with aids
Homosexual or bisexual men
104
routes of transmission of aids
Sexual transmission
Parenteral transmission
Mother-to-infant transmission
105
Enhanced by coexisting sexually transmitted diseases
Sexual transmission
106
2 ways,of sexual transmission
1. Direct inoculation into the blood vessels breached by
trauma
2. Infection of dendritic cells or CD4+ cells within the mucosa
107
intravenous drug abusers, hemophiliacs, recipients of blood transfusion
Parenteral transmission
108
most common cause of pediatric aids
Mother-to-infant transmission
109
in utero ( transplacental spread), during delivery ( infected birth canal), after birth ( ingestion of breast milk)
Mother-to-infant transmission
110
most common mode in US
transmission during birth ( intrapartum), immediate period thereafter ( peripartum)
111
co receptors of hiv
CCR5 and CXCR4
112
CD4 binding
gp120
113
penetration
gp41
114
Defective macrophage and dendritic cell
| functions
Loss of CD4+ T cells
115
Destruction of architecture of lymphoid tissues
Loss of CD4+ T cells
116
self-limited acute illness with nonspecific symptoms, including sore throat, myalgias, fever, weight loss, and fatigue, resembling a flulike syndrome
Acute retroviral syndrome
117
few or no clinical manifestations of the HIV infection are present.
Chronic infection: Phase of clinical latency
118
Patients are either asymptomatic or develop minor opportunistic infections, such as oral candidiasis (thrush), vaginal candidiasis, and perhaps mycobacterial tuberculosis
Chronic infection: Phase of clinical latency
119
characterized by a breakdown of host defense, and severe, life-threatening clinical disease
AIDS
120
serious opportunistic infections, secondary neoplasms, or clinical neurologic disease emerge
AIDS
121
clinical features or common manifestations of AIDS
```
Fever
Weight loss
Diarrhea
Generalized lymphadenopathy
Multiple opportunistic infections
Neurologic disease
Secondary neoplasms
```
122
account for the majority of deaths for untreated patients with AIDS
Multiple opportunistic infections
123
treatment for aids
HAART Highly Active Antiretoviral therapy
124
lack of responsiveness to an individual’s own antigens
Self-tolerance
125
Unresponsiveness to an antigen induced by exposure of lymphocytes to that antigen
Immunologic Tolerance
126
Immature self-reactive T and B lymphocyte clones that recognize self antigens during their maturation in the central lymphoid organs are killed or rendered harmless
Central Tolerance
127
Negative selection or deletion
T cells
128
Receptor editing
B cells
129
Anergy
Suppression by regulatory T cells
Deletion by apoptosis
Peripheral Tolerance
130
when lymphocytes that recognizes self antigens may be rendered functionally unresponsive
anergy
131
prevent immune reaction against self antigens
Suppression by regulatory T cells
132
t cells that recognizes self antigens may receive signals that promote their death by apoptosis
Deletion by apoptosis
133
general principles
Defective tolerance or regulation
Abnormal display of self antigens
Inflammation or an initial innate immune response
134
General Features of Autoimmune Diseases
Chronic, with relapses and remissions, and the damage is often progressive
Clinical and pathologic manifestations are determined by the nature of the underlying immune response
135
an immune response against one self antigen causes tissue damage releasing other antigens resulting in the activation of lymphocytes by newly encountered epitope
epitope spreading
136
Injury is caused by deposition of immune complexes and binding of antibodies to various cells and tissues
Systemic Lupus Erythematosus (SLE)
137
Systemic Lupus Erythematosus (SLE) hypersensitivity reaction
type 3
138
characterized by vast array of autoantibodies ( ANAs Anti Nuclear Antibodies)
Systemic Lupus Erythematosus (SLE)
139
Injury to the skin, joints, kidney, and serosal
| membranes is prominent
Systemic Lupus Erythematosus (SLE)
140
Predominantly affects women of childbearing age
Systemic Lupus Erythematosus (SLE)
141
may be acute or insidious in its onset typically chronic remitting and relapsing and often febrile illness
Systemic Lupus Erythematosus (SLE)
142
SLE manifestations
Discoid rash
Oral Ulcers
Photosensitivity
Arthritis
Malar rash
Iimmunologic disorders
Neurologic disorder (seizure or psychosis)
Renal Disorder (Nephritic manefestations)
ANA
Serocitis (Pleuritis, pericarditis)
Hematologic disorders ( anemia, leukopenia, thrombocytopenia)
143
Hallmark of SLE
production of ANA
144
positive in 95-100% of cases but are also positive for a lot of autoimmune diseases
generic ANA
145
more specific antibodies for SLE
DS DNA
| SMITH antibodies
146
factors that contribute in the development of SLE
Genetic Factors
Immunologic Factors
Environmental Factors- ultraviolet (UV) light, gender bias, drugs
147
fundamental defect of SLE
failure of the mechanisms that maintain self-tolerance
148
SLE is not only a hypersensitivity disease, but also an autoimmune disease
true
149
organ mainly affected by SLE
kidney
150
least common lupus nephritis
class 1
151
most common lupus nephritis
class 4
152
classes of nephritis
```
class 1 Minimal mesangial LN
class 2 Mesangial proliferative LN
class 3 Focal LN
class 4 Diffused LN
class 5 Membranous LN
class 6 Advance Sclerosing LN
```
153
end stage renal disease
class 6
154
most patients of SLE are diagnosed with
class 4
155
other organ affected by SLE
skin
156
SLE Clinical Features
Young woman with butterfly rash over the face
Fever, photosensitivity
Characterized by flare-ups and remissions for years or decades
157
treatment for SLE
Steroids, immunosuppressive drugs
158
cause of death in SLE
Renal failure and infections
159
Chronic disease characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) resulting from immunologically mediated destruction of the lacrimal and salivary glands
Sjögren Syndrome
160
dry mouth
xerostomia
161
dry eyes
keratoconjunctivitis sicca
162
ANAs are detected in 50% to 80% of patients
Sjögren Syndrome
163
more specific antibodies that can be detected in 90% of patients
SS-A (Ro) and SS-B (La)
164
diagnosis of Sjögren Syndrome
Biopsy of the lip (to examine minor salivary glands)
165
diagnosis of Sjögren Syndrome on microscopic examination
intense lymphocytic and plasma cell infiltration with ductal epithelial hyperplasia in the salivary gland
166
Chronic inflammation thought to be the result of autoimmunity
Systemic Sclerosis (Scleroderma)
167
Widespread damage to small blood vessels
Systemic Sclerosis (Scleroderma)
168
Progressive interstitial and perivascular fibrosis
| in the skin and multiple organs
Systemic Sclerosis (Scleroderma)
169
Characterized by excessive fibrosis throughout the body
Systemic Sclerosis (Scleroderma)
170
most commonly affected organ in Systemic Sclerosis (Scleroderma)
skin
171
frequently involved organs in Systemic Sclerosis (Scleroderma)
```
GIT
Kidney
Heart
Muscles
Lungs
```
172
cause of death in Systemic Sclerosis (Scleroderma)
renal failure
cardiac failure
pulmonary insufficiency
intestinal malabsorption
173
types of scleroderma
Diffuse scleroderma
| Limited scleroderma
174
widespread skin involvement at onset, with rapid progression and early visceral involvement
Diffuse scleroderma
175
skin involvement is often confined to fingers, forearms, and face. limited in the skin
Limited scleroderma
176
CREST syndrome
Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia
177
more common in female with 3:1 ratio. and on 50-60yo
scleroderma
178
manifestations of scleroderma
```
Raynaud phenomenon
Dysphagia
Respiratory difficulties
Malignant hypertension
Renal failure
```
179
episodic vasoconstriction of the arteries and arterioles of the extermities
raynau phenomenon
180
attributed to esophageal fibrosis and its resultant hypomotility abdominal pain, intestinal obstruction or malabsorption syndrome with weightloss and anemia reflect involvement of small intestine
dysphagia
181
pulmonary fibrosis, and may result in right sided cardiac dysfunction
myocardial fibrosis may cause arrhythmias or cardiac failure
Respiratory difficulties
182
majority of patients of scleroderma would have
diffused sclerotic atrophy of the skin which usually begins in the fingers and the distal regions of the upper extremities and extends proximally to involve the upper arms, shoulder, neck and face
183
histologic manifestations of scleroderma
edema and peri vascular infiltrates together with swelling and degeneration of collagen fibers
184
progressive atrophy and collagenous fibrous replacement of the muscularis
Alimentary tract
185
affected in 90% of patients with scleroderma
Alimentary tract
186
inflammation of the synovium, with hypertrophy and hyperplasia of the synovial soft tissues; fibrosis later ensues
Musculoskeletal system
187
prominent vascular lesions
kidneys
188
pulmonary hypertension and interstitial
| fibrosis
lungs
189
pericarditis with effusion, myocardial fibrosis, and thickening of intramyocardial arterioles
heart
190
extracellular deposits of fibrillar proteins are responsible for tissue damage and functional compromise
Amyloidosis
191
abnormal folding of proteins which becomes insoluble aggregates
Amyloidosis
192
a pathologic proteinaceous substance, deposited in the extracellular space in various tissues and organs of the body
Amyloidosis
193
appears as an amorphous, eosinophilic, hyaline, extracellular substance that, with progressive accumulation, encroaches on and produces pressure atrophy of adjacent cells
Amyloidosis
194
made up largely of continuous, nonbranching fibrils
amyloid
195
stain for amyloids which under ordinary light impart a pink or red color to the tissue deposits
congo red stain
196
more striking and specific stain of amyloids when observed by polarizing microscopy
green bifringens
197
most common forms of amyloid
AL (amyloid light chain)
AA (amyloid-associated)
β amyloid (Aβ)
198
derived from Ig light chains produced in
| plasma cells
AL (amyloid light chain)
199
seen in multiple myeloma
AL (amyloid light chain)
200
non-Ig protein synthesized by the liver
| - associated with chronic inflammation, and is often called secondary amyloidosis
AA (amyloid-associated)
201
core of cerebral plaques found in Alzheimer disease
β amyloid (Aβ)
202
Other biochemical distinct proteins found in amyloid deposits:
Transthyretin/TTR)
| β2-microglobulin
203
Senile systemic amyloidosis
Transthyretin/TTR
204
Hemodialysis-Associated Amyloidosis
β2-microglobulin
