Disorders of the immune system Flashcards
1
Q
Immunity is from a latin term that means what?
A
Exemption from duty.
2
Q
What are the 2 types of immune responses?
A
- Natural immunity (non-specific). 2. Acquired immunity (Specific).
3
Q
What are the 3 proteins that work under the natural immunity response?
A
- Complement. 2. Properdin. 3. Lysozyme.
4
Q
What is the general concept of acquired immunity?
A
Self vs. non-self.
5
Q
What is the 1st, 2nd, and 3rd lines of defense in natural protection?
A
- Mechanical/ chemical barrier. 2. inflammatory/ Phagocytosis. 3. Specific immune responses.
6
Q
What are the different kinds of T-Lymphocytes?
A
T-helper cells (CD4+), T-suppressor or cytotoxic cells (CD8+), and Natural killer cells.
7
Q
B-lymphocytes will become what in tissues?
A
Plasma cells.
8
Q
Where will T and B lymphocytes and Natural killer cells come from?
A
Bone marrow.
9
Q
Basophils become what in tissues?
A
Mast cells.
10
Q
Monocytes become what in tissues?
A
Macrophages.
11
Q
All antibodies are composed of what?
A
Light and heavy chains.
12
Q
Which chains of antibodies are changeable and which chain is the same?
A
Light- same. Heavy are specific for each Ig.
13
Q
What is another name for light and heavy chains?
A
Light-Fc. Heavy-Fab.
14
Q
What is the largest Ig?
A
IgM.
15
Q
What is the function of the IgM?
A
To neutralize microorganisms.
16
Q
The IgM has how many complement binding sites?
A
five.
17
Q
What is the first Ig to appear?
A
IgM.
18
Q
IgM is the natural antibody against what?
A
ABO blood group ag.
19
Q
Which Ig has the smallest molecular weight?
A
IgG.
20
Q
When will IgG be produced?
A
Small amounts produced on initial immunization and boosted on re-exposure.
21
Q
IgG’s act as an opsonin which is what?
A
bacteria coating and phagocytosis.
22
Q
IgA will be mainly found where?
A
In mucosal secretions; milk, tears, respiratory, GI tract.
23
Q
What is the function of IgA?
A
Protective.
24
Q
What Ig is secreted by sensitized plasma cells in tissues?
A
IgE.
25
IgE are attached locally to what?
Mast cells.
26
What is the function of IgE's?
mediates allergic reactions in tissues, and to protect against parasites.
27
Where are IgD's found at?
Cell membrane-bound found exclusively on B cells.
28
What is the function of the IgD?
participates in antigenic activation of B cells
29
Which of the Ig's will not be released in the serum?
IgD.
30
What is the Major Histocompatiblity complex essential for?
presentation of antigens to T cells.
31
The major histocompatibility complex is AKA?
human leukocyte antigen (HLA).
32
What is an immunologic finger print unique to me?
my major histocopmatibility complex.
33
What are the 2 groups of the major histocompatibilty complex?
Type I and Type II.
34
The type I major histocompatibility complex has receptors for what?
CD8+ or the T suppressor or cytotoxic cells.
35
The type II major histocompatibility complex reacts with what?
CD4+ or the T-helper cells.
36
What are the antigen-presenting cells of the major histocompatibility complex?
Macrophages, and B cells if antigen is internalized.
37
What is the function of the Major histocompatibility complex (MHC)?
To present antigens to T cells.
38
T cells can only react to membrane-bound antigens and this means what?
Without Antigen presenting cells T cell will have no reaction to antigens.
39
MHC type I is a receptor for the CD8+ cells and are found where?
On all nucleated cells of the body.
40
MCH type I links what to what?
CD8+ or the T suppressor or cytotoxic cells to antigen presenting cells (APC).
41
MCH type II react with the CD4+ or T helper cells and this will lead to what?
antigen presenting cells to Helper T lymphocytes. This serves as a presentation of exogenous antigens that are first internalized and processed.
42
MHC is AKA?
HLA or human leukocyte antigen.
43
What chromosome are the HLA subtypes found on?
6 regions A,B, and DR.
44
HLA A3 is assoicated with what?
Hemochoromatosis, myathenia gravis, Multiple sclerosis.
45
HLA B27 is associated with what?
Psoriasis, Ankylosing spondylitis, Inflammatory bowel disease, Reiter's syndrome.
46
HLA B8 is associated with what?
Grave's disease, celiac sprue.
47
HLA DR2 is associated with what?
Multiple sclerosis, hay fever, SLE, Goodpasture.
48
HLA DR3 is associated with what?
Diabetes Mellitus type I.
49
HLA DR4 is accoicated with what?
Rheumatoid arthritis, DM type I.
50
HLA DR5 is associated with what?
Pernicious anemia, hasimoto's throiditis.
51
HLA DR7 is assoicated with what?
Steroid-responive nephrotic syndrome.
52
Pernicious anemia is caused by what?
A B12 deficinecy.
53
What is an epitope?
An antigenic site.
54
How many epitopes can be on an antigen?
Many.
55
Antibodies to soluable antigens form what?
Soluable complexes that may be found in circulation.
56
What do antibodies that are bound to fixed antigens on cells do?
Coat the cell surface.
57
What happens when antigen-antibody complexes bind?
This activates the complement system.
58
The complement system does what?
Lysis of cells, agglutination or recruitment of inflammatory cells.
59
What is immune hemolysis?
The immune system breaking down RBC.
60
Immune hemolysis is what type of reaction?
A hypersensitive one.
61
How many types of immune hemolysis are there?
types I-IV.
62
Which types of immune hemolysis are caused by antibodies?
I,II, III.
63
Type I hypersensitive reaction is due to what?
Anaphylactic.
64
Type II hypersensitive reaction is due to what?
Cytotoxic antibody mediated.
65
Type III hypersensitive reaction is due to what?
Immune complex mediated.
66
Type IV hypersensitive reaction is due to what?
Cell mediated, delayed type.
67
Type I hypersensitivity is mediated by what?
IgE, mast or basophils.
68
Type I hypersensitivity takes how long to respond?
4-6 hours.
69
Is hay fever always seasonal?
No.
70
What are the treatments for Type I hypersensitivity?
anti-histamines, Vitamin C, Desensitization.
71
Asthma is what type of hypersensitivity?
Type I.
72
Type I hypersensitivty associated with asthma affects what?
Bronchi.
73
Will asthma always be a hypersensitivty disease?
No there are many types.
74
Hypersensitive asthma is due to an inhaled antigen mediated by what?
SRS-As a slow release.
75
What supplement can relax the airways?
Magnesium.
76
What is a side effect of magnesium?
It relaxes the smooth muscle and can lead to a warm feeling.
77
Atopic dermatitis is a type I hypersensivity and it is typically a _____ disease.
Childhood.
78
Atopic dermatitis is aka?
Eczema.
79
what % of children get atopic dermatitis?
10%.
80
OF the 10% of children that get atopic dermatitis how many of them have family history of atopic dermatitis?
50%.
81
How will allergen that causes atopic dermatitis enter the body?
inhaled, ingested in food, or skin contact.
82
Atopic deramtitis is sometimes treated with corticosteroids and this can cause what?
Asthma/hay fever later in life.
83
What is a severe sytemic response to an allergen called?
Anaphylactic shock.
84
Anaphylactic shock is what type of hypersensitve reaction?
Type I.
85
What happens with anaphylactic shock?
A massive release of histamine/other vasoactive substances.
86
What are the signs of anaphylactic shock?
chocking, wheezing, shortness of breath.
87
What is the treatment of anaphylactic shock?
epinephrine STAT, high flow O2 non-rebreather.
88
What type of hypersensitivity is associated with autoimmune diseases?
Type II.
89
What causes Type II hypersensitivity?
Cytotoxic antibody that reacts to antigen in cells or tissue components.
90
With type II hypersensitivity the antigen can be where?
Extrinsic or intrinsic.
91
Type II hypersensitivity is mediated by what Ig's?
IgG or IgM.
92
Type II hypersensitivity activates the complement system which will do what?
Lysis of cell.
93
Give an example of a type II hypersensitive reaction?
Blood transfusion mismatch.
94
What type of hypersensitive reaction is hemolytic anemia?
Type II.
95
What is hemolytic anemia?
A systemic autoimmune disorder can be caused by foreign chemicals.
96
Goodpasture's syndrome involves what pathologies?
renal and pulmonary.
97
What causes goodpastures syndrome?
autoimmune to component of Type IV collagen.
98
What type of hypersensitivity is goodpasture's syndrome?
Type II.
99
What are the signs and symptoms of goodpastures syndrome?
Hemoptysis (coughing of blood), and hematuria.
100
What will cause Grave's disease?
Autoantibody against the thyroid receptor for thyroid-stimulating hormone.
101
How will the autoantibody for the thyroid receptor for thyroid-stimulating hormone be affected with Graves disease?
It will be stimulated to make more T3 and T4.
102
Graves disease will cause exopthalmus which is what?
Protrusion of the eyeball.
103
What is myasthenia gravis?
Episodic muscle weakness and easy fatigability.
104
What causes myasthenia gravis?
Autoantibody and cell-mediated destruction of acetylcholine receptors.
105
What type of hypersensitivity is myasthenia gravis?
Type II.
106
What will worsen and lessen symptoms of myasthenia gravis?
Worsen-muscle activity. Lessen-rest.
107
How is myasthenia gravis tested?
By antibody testing to aceylcholine receptors.
108
Initially and eventually what % of ocular muscles are affectd by myasthenia gravis?
initially-40%. Eventually-85%.
109
Type III hypersensitive reactions cause what?
Acute inflammation in response to circulating antigen-antibody immune complexes deposited in vessels or tissues.
110
The antigen-antibody complex that are circulating around that casue Type III hypersensitive reactions activate what?
complement system, or they bind to and activate certain immune cells. This results in release of inflammatory mediators.
111
When will type III hypersensitive reactions become systemic?
When antigen and antibody are more balanced instead of when there is much more antigen than antibody. When we get a balanced amount of antigen and antibodies there will be lots of complexes formed which tend to be deposited in various tissues and this leads to a systemic reaction.
112
Poststerptococcal glomerulonephritis typically follows what?
Upper respiratory tract infection by a nephritogenic streptococcal strain.
113
Poststerptococcal glomerulonephritis is when the antigen from the upper respiratory tract infection gets onto the glomerulonephritis during filtration of plasma and what happens?
The Complement system is evoked, and this leads to a type III hypersensitive reaction.
114
What Type III hypersensitive reaction was discovered by injecting horse serum into rabbits repeatedly?
Arthus reaction.
115
What happens with arthus reaction?
The antigen/antibody complexes collect in the vascular walls, serosa, and glomeruli.
116
What did the CDC have to say about arthus reaction?
Rarely reported after vaccination, but can happen after tetanus or diphtheria vaccinations.
117
What is the arthus phenomenon?
Polyarteritis Nodosa (Many arteries Knotty).
118
What disease can cause arthus phenomenon?
Unknown antigen, but seen with chronic hepatitis B virus.
119
What is SLE?
Systemic Lupus erythematosus.
120
What is SLE glomberulonephritis?
A deposition of complement-activating immune complexes.
121
Type IV hypersensitive reactions are reactions of what?
T cell mediated. Not antibodies
122
How will Type IV hypersensitive reactions in general happen?
T cells get sensitized and after second contact they are activated and damage tissue by direct toxic effects or through release of cytokines.
123
What will T cell cytokines do?
activate eosinphils, monocytes, macrophages, neutrophils, or Killer cells.
124
Caseating granuloma is seen in what?
TB.
125
How will Type I DM be triggered?
Autoimmune response.
126
What type of reaction is Type I DM?
Type IV hypersensitive.
127
What is an autograph, isograft, homograft, and xenograft?
auto-self to self. Iso-identical twin or clone. Homo-within species. Xeno-outside of species.
128
What is a hyperacute reaction to a transplant?
reaction during operation; have antibodies to donors antigen, like a reaction to wrong blood type.
129
What is the time frame for an acute reaction to a transplant?
first few weeks.
130
How long will chronic transplant rejections last and they can casue what?
months to years, obliteration of arterial lumen.
131
What types of transplants are used clinically?
kindey, skin, liver, heart, lung, pancreas, bone marrow.
132
Graft-versus-host reaction is mediated by what?
Transplanted T lymphocytes.
133
Graft-versus-host reaction is most often a complication of what?
Bone marrow transplantation.
134
What are the organs most commonly effected by graft-versus-host reactions?
skin, intestines, liver.
135
What is the most common minor blood group antigen?
Rh.
136
What is the major blood group antigens?
A and B.
137
Blood type A can give blood to who?
A and AB.
138
Blood type A can get blood from who?
A,O.
139
Blood type B can give blood to who?
B, AB.
140
Blood type B can get blood from who?
B, O.
141
Blood type AB can give blood to who?
Type AB.
142
Blood type AB can get blood from who?
Universal (so A,B,AB, O).
143
Bood type O can give blood to who?
Universal (so A,B,AB, O).
144
Bood type O can get blood from who?
O.
145
Rh factor is important in what?
Mother being - and baby being +, but only after mother is sensitized so can affect second birth.
146
The Rh factor causing problems with pregnancy is prevented by what?
Rhogam.
147
Fetal hydrops is caused by Rh factor incompatibility and leads to what?
Pleural effusion, ascites, scalp edema.
148
In general what is an autoimmune disease?
Abnormal response to self-antigen.
149
What are 3 things that make autoimmune diseases more common?
1. Familial predisposition. 2. Certain HLA-haplotypes. 3. Sex differences more common in females than males.
150
Name 5 systemic autoimmune diseases?
sle, rheumatic fever, rheumatoid arthritis, systemic sclerosis, polyarteritis nodosa.
151
Name 5 autoimmune diseases that are limited to a single organ?
multiple sclerosis, Hashimotos thyroiditis, autoimmune hemolytic anemia, pemphigus vulgaris, myasthenia gravis.
152
Then next many question will give the autoantibody and you must give the associated disorder.
ok
153
antinuclear antibodies (ANA)?
SLE.
154
Anti-dsDNA, Antio-smith?
Specific for SLE.
155
Antihistone?
Drg-induced lupus.
156
Anti-IgG?
Rhematoid arthritis.
157
anticentromere?
scleroderma.
158
Anti-Scl-70?
scleroderma (diffuse).
159
Antimitochondrial?
primary biliary cirrhosi.
160
antigliadin?
Ciliac disease.
161
anti-basement membrane?
goodpasture.
162
anti-epithelial cell?
pemphigus vulgaris.
163
antimicrosomal, antithyroglobulin?
hashimotos.
164
anti-jo-1?
polymyositis dermatomyositis.
165
anti-ss-A?
Sjogren syndrome.
166
Anti-ss-B?
Sjogren syndrome.
167
Anti-U1 RNP?
Mixed Connnective tissue.
168
Anti-smooth muscle?
Autoimmune hepatitis.
169
Anti-glutamate decarboxylase?
Type I DM.
170
C-ANAC?
Wegner's granulomatosis.
171
P-anca?
Other vasculitides.
172
Virtually all Systemic Lupus Erythematosus (SLE) will have what?
ANA or the antinuclear anitbodies.
173
75% of SLE patients will have what involvment?
KD.
174
What is the major sign of SLE?
Butterly rash.
175
What type of people most often get SLE?
Females in late 30-40s
176
What are the treatments of SLE?
Vacation, Yoga (breathing helps shift from acid to alkalosis), drinking backing soda, more vegatables, reduce stress.
177
The first episode of acute rhematic fever occurs most often?
between 5 and 15 years old.
178
Acute rhematic fever is uncommon when?
Before 3 and after 21.
179
What is the precursor of acute rhematic fever?
GAS (group A strptococcal). An infection.
180
What infection will share an epitope with GAS?
GAS M.
181
What is most often affected by rhematic fever?
Joints, heart, skin, CNS.
182
joints are ______ \_\_\_\_\_\_\_\_ with rheumatoid arthritis.
symmetrically inflamed.
183
Symmetrically inflamed joints with rheumatoid arthritis leads to what?
progressive destruction of aticular structures.
184
What are 3 ways to treat rheumatoid arthritis?
drugs, physical measures, and surgery.
185
What type of drug is used for rheumatoid arthritis?
DMAR disase-modifying antirheumatic drugs.
186
What part of the population is affected with rheumatoid arthritis?
1% of population with 2-3 times more women than men. Can happen at any age, but most often between 35-50.
187
Antibody known as anti-IgG is aka?
Rheumatoid factor.
188
What type of cells make rheumatoid factor?
plasma cells.
189
Can rheumatoid arthritis occur without anti-IgG aka rheumatoid factor?
Yes.
190
What are the symptoms of Rheumatoid arthritis?
Early morning stiffness, joint pain swelling and stiffness.
191
Rheumatoid artritis will have symetric pain and the stiffness lasts how long in the mornings?
more than 60 min.
192
How can we treat rheumatid arthritis?
No red meat in diet, Rust Ox/poison ivy.
193
How will fingers drift with rheumatoid arthritis?
Ulnar drift.
194
What are the 2 types of immuneodeficiency diseases?
1. Primary (congenital=present at birth). 2. Secondary (aquired).
195
What is the secondary or aquired immunodeficiency?
AIDS aquired immunodeficiency syndrome.
196
What will cause severe combined immunodeficiency, and what type of immunodeficiency is it?
It is primary and caused by a defect of lymphoid stem cells pre-B, Pre-T cells.
197
What is the most common type of primary immunodeficiency diseases?
Isolated deficiency of IgA.
198
What % of people have Isolated deficiency of IgA?
1 out of every 700 people
199
What is isolated deficiency of IgA like in the population?
Often asymptomatic.
200
What type of immunodeficiency disease is DiGeorge's syndrome and what causes it?
It is a primary one caused by T-cell deficiency related to aplasia of thymus associated with aplasia of parathyroid glands.
201
What type of virus is HIV aka Human immunodeficiency virus?
RNA retrovirus.
202
AIDS infects what?
T helper Cells (CD4+).
203
Where is AIDS stored at?
Macrophages and related phagocytic cells.
204
What are the clinical presentations of HIV/AIDS?
acute illness, asymptomatic infection, persistent generalized lymphadenopathy.
205
What are 5 pahtologic findings in AIDS?
1. Lymphadenopathy. 2. Kaposis sarcoma. 3. Nephrophaty. 4. Weakness. 5. oral thresh.
206
What type of infections happen with AIDS?
Opportunistic.
207
What 3 places are prone to opportunisitic infections?
1. Lungs. 2. GI tract. 3. CNS.
208
What are the 3 tumors that develop with AIDS?
1. Lymphoma of lymph nodes or GI tract. 2. Lymphoma of the CNS. 3. Kaposis sarcoma.
209
What type of cell is deficient with immune deficiencies?
Phagocytic.
210
What immune deficiency will cause a decreased production of B cells?
Bruton's agammaglobulinemia.
211
How is Bruton's agammaglobulinemia caused and who can get it?
Associated with recurrent Bacterial infections after 6 months of age when maternal IgG levels drop off. It is a X-linked recessive gene that occurs in boys.
212
What immune deficiency will cause a decreased production of T cells?
Thymic aplasia (DiGeorge syndrome).
213
T cell deficiency will present as what?
Tetany
214
What is tetany?
a state marked by severe, intermittent tonic contractions and muscular pain, due to hypocalcemia.
215
What immune deficency will cause a decreased production of both T and B cells?
SCID (severe combined immunodeficiency).
216
What immune deficency will cause a decreased T cell activation?
IL2 receptor deficency.
217
What will cause a IL2 receptor deficency?
Infections due to decreased Th1 response.
218
What immune deficiency will cause a decreased activation of B cells?
Hyper IgM syndrome, and Wiskott-aldrich syndrome.
219
What causes Hyper IgM syndrome?
Defect in CD40 ligand on CD4 + T helper cell.
220
What causes Wiskott-aldrich syndrome?
X-linked defect in the ability to mount an IgM response.
221
What is the triad of symptoms for Wiskott-aldrich syndrome?
WIPE. Wiskott. Infections. Purpura. Eczema.
222
What immune deficiency will cause a decreased activation of Macrophages?
Job's syndrome.
223
Job's syndrome presents with what?
FATED. Facies, Abscesses, Teeth, increased IgE, Dermatologic problems.
224
What is the leukocyte adhesion deficiency syndrome?
An immune deficiency leading to phagocytic cell deficiency because of a LFA-1 defect.
225
How will leukocyte adhesion deficiency syndrome present?
With recurrent bacterial infections, absent pus formation, and delayed separation of umbilicus.
226
What is Chediak-higashi disease?
An immune deficiency leading to phagocytic cell deficiency because of autosomal recessive defect in microtubular function and lysosomal emptying of phagocytic cells.
227
How will Chediak-Higashi's disease present?
with recurrent pyogenic infections by staphylococci and strptococci, partial albinism, and peripheral neuropathy.
228
What is Chronic granulomatous disease?
An immune deficiency leading to phagocytic cell deficiency because of lack of NADPH oxidase activity or similar enzymes.
229
How will chronic granulomatous disease present?
Marked opportunisitic infections with bacteria, especially S. aureus, E. coli, and Aspergillus.
230
What does idiopathic mean?
Of unknown cause.
231
What is an idiopathic dysfunction of T cells?
Chronic mucocutaneous candidiasis.
232
Chronic mucocutaneous candidiasis is a T cell dysfunction against what?
Candida albicans.
233
How will Chronic mucocutaneous candidiasis present?
With skin and mucous membrane candida infections.
234
There are 3 B cell idiopathic dysfunctions is due to a deficiency in a specific class of immunoglobins?
Selective immunoglobulin deficiency.
235
How will selective immunoglobulin deficiency present?
Siuns and lung infections, Milk allergies and diarrhea are common.
236
What B cell idiopathic dysfunction is due to a defect in DNA repair enzymes?
Ataxia-Telangiectasia.
237
Ataxia-Telangiectasia is associated with what deficiency?
IgA.
238
How will ataxia-Telengiectasia present?
with cerebellar problems and spider angiomas.
239
What B cell idiopathic dysfunction has normal numbers of circulating B cells?
Common variable immunodeficiency.
240
When can common variable immunodeficiency be acquired?
20s-30s.
241
What is an Amyloid?
A space occupying lesion that is an inert protein.
242
What are amyloid fibers like?
7.5 nm thick when seen by elctron microscopy.
243
How will amyloids stain and be seen?
Stain congo red and appears apple green under polarized light.
244
What type of sheets will Amyloids have by radiographic cyrstallography?
Beta-pleated.
245
What is the usual cause of systemic amyloidosis?
Deposition of AA or AL amyloid in various organs, like; liver, kidney, adrenals, spleen, or heart.
246
Give an example of localized organ-specific amyloid deposits?
Brain-alzheimers.
