Immunology III Flashcards
1
Q
What is an Autoimmune disorder of Connective Tissue
caused by Activated Fibroblasts?
A
Scleroderma
*Fibroblasts > Collagen can’t stop won’t stop
2
Q
What is an ANA?
A
Anti-nuclear Antibody
*diagnosis of autoimmune disorders
but “not diagnostic of any”
**higher titer, worse the disease
3
Q
What 3 antibodies are specific to Scleroderma?
Name 1 factor commonly present in Scleroderma that is also seen in another disease.
A
Nucleolar
Anti-DNA topoisomerase Scl-70 (nonhistone, 70% of patients)
Anticentromere
Rheumatoid factor
4
Q
What is Progressive System Sclerosis?
A
Scleroderma
5
Q
What are the most common organs affected by Progressive System Sclerosis (Scleroderma)?
A
GI tract, heart, kidneys, muscles, lungs
6
Q
T/F
Scleroderma limits itself to certain organs.
A
False.
Many organs involved
7
Q
What autoimmune disorder begins with Raynaud phenomenon (distal ischemia)?
A
Scleroderma
*this can lead to auto-amputation
8
Q
What often causes sudden death in Scleroderma?
A
Ventricular arrhythmias
9
Q
What is the milder form of Scleroderma called?
A
CREST syndrome
10
Q
What are the 5 features of CREST?
A
C Calcinosis R Raynaud Phenomenon E Esophageal dysfunction S Sclerodactyly T Telangiectasia (capillary dilation/purple clusters)
11
Q
What are 2 Autoimmune diseases of the muscle?
note: they are chronic inflammatory myopathies
A
Polymyositis
Dermatomyositis
12
Q
What virus has been implicated in Polymyositis/Dermatomyosits?
A
Picornaviruses
13
Q
What are the most specific antibodies associated with Polymyositis/Dermatomyositis?
A
anti-Jo-1
*an MSA (myositis specific antibody)
14
Q
What is Heliotropic Rash specific to?
note: this is humorally-mediated
A
Dermatomyositis eyelids
*no angiopathy in Polymyositis
15
Q
What are Anti-centromeric antibodies associated with?
A
Both Scleroderma and CREST
16
Q
Why are Polymyositis/Dermatomyositis considered to by paraneoplastic syndromes?
A
Esp. in men over 50, 3/4 with Polymyositis/Dermatomyositis already have cancer (or will within a year)
17
Q
What cancers are most associated with Polymyositis and Dermatomyositis in men and women?
A
Men: Lung, colon, stomach
Women: Breast, ovaries, uterus
18
Q
What 2 Autoantibodies are diagnostically definitive for Lupus?
A
Anti double stranded DNA (native DNA)
Anti Smith
*Rim and Speckled (diffuse also seen)
19
Q
What does SLE stand for?
A
Systemic Lups Erythematosus
20
Q
SLE affects all organs, but preferentially targets…
A
Kidneys
Serousal membranes
Joints
Skin
21
Q
What are two risk factors involved in SLE?
A
Female sex hormones
Familial correlation
22
Q
What is the staining pattern of a homogenous, non-specific ANA?
A
Diffuse
23
Q
What is the staining pattern of Anti-double stranded DNA seen in SLE?
A
Rim
24
Q
What is the staining pattern of the antibody to extractable nuclear antigens seen in Mixed Connective Tissue Disorder?
A
Speckled
25
What is the staining pattern seen in PSS (Progressive Systemic Sclerosis)?
Nucleolar
26
What staining pattern is seen for the antibody in CREST syndrome?
Centromeric
27
*Which of the following types of Lupus Glomerulonephritis is mildest with an excellent prognosis?
Mesangial Lupus
28
How does SLE manifest on the skin?
Butterfly on face
29
How does SLE affect serous membranes?
1/3 have pleuritis with pleural effusion
30
What causes changes in the brain in Lupus?
Interstitial fibrosis
Vasculitis leads to hemmorrhage and infarction
31
What type of carditis is involved in Lupus?
Pancarditis.
pericarditis - most common
endocarditis
myocarditis
(nonbacterial vegetations (Libman-Sacks Endo) in latter two)
32
What autoantibodies are prevalent in Drug-Induced Lupus?
Anti-Histone Antibodies
| DS-DNA and SM antigen UNusual
33
What autoimmune Syndrome is characterized by dry eyes and dry mouth (keratoconjunctivitis and xerostomia), often secondary to SLE and RA?
Sjogren Syndrome
34
What condition is marked by the lack of all immunoglubulin in the blood?
Agammaglobulinemia of Bruton
35
What type of disorder is Agammaglobulinemia of Bruton?
Congenital X-linked
36
What types of infections are handled well in pts with Agammaglobulinemia of Bruton?
Most viral/fungal
37
What gene and enzyme is broken in congenital x-linked Agammaglobulinemia of Bruton?
Xq21
B-cell Tyrosine Kinase
(ATK)
*critical for the maturation of B-cells
38
What types of infections are common in Agammaglobulinemia of Bruton?
What is the treatment for the disease?
Bacterial
Replacement of all immunoglobulins
39
When is Agammaglobulinemia of Bruton first observed?
5-8 months, when maternal antibodies wear out, usually with Pyogenic Infections
40
What causes RA-like conditions in x-linked agammaglobulinemia of Bruton pts?
Mycoplasma
41
What is another name for Swiss-type Agammaglobulinemia?
What does it affect?
Severe Combined Immunodeficiency
T and B cells
42
What are the two types of Severe Combined Immunodeficiency?
Treatment?
X-linked (more common)
Autosomal recessive
Bone marrow transplantation
(this is boy in the bubble)
43
What is embryologically defective in DiGeorge Syndrome?
3rd and 4th pharyngeal pouches
| *Thymus and Parathyroids
hypocalcemia and tetany prevalent b/c no parathyroids
44
What cell is completely lacking in DiGeorge syndrome?
T-cells
| no thymus
45
What are the internal and external manifestations of DiGeorge's?
Internal: interrupted aortic arch, microencephaly
External: Low set ears, cleft lips
46
T/F
| DiGeorge's pts have a good response to Fungal and Viral infections.
False
complete lack of T-cells
47
What are the 4 main receptors on the HIV virus?
```
gp 120 (lollipop)
gp 41 (lollipop stick)
```
p17 (outer)
p24 (inner)
48
What is the most common cause of meningitis in AIDS patients?
Cryptococcus
| a fungus
49
What cells are affected in HIV?
| these cells carry HIV
CD4 T-helper cells
50
What type of virus is HIV?
RNA retrovirus
51
What is the most common cause of death in AIDS patients?
Pneumocystis
52
What increases the transmission of HIV?
co-existing STI's
male to female (not female to male)
anal sex
53
T/F
| Breast milk cannot transmit HIV.
False
54
What are the 3 neoplasms of HIV?
Kaposi sarcoma
B-cell non-Hodgkin lymphoma (Primary lymphoma of brain)
Invasive caner of uterine cervix
55
What is the presumptive and definitive test for HIV?
ELISA - presumptive
| Western Blot - definitive
56
What antibody is bound in Enzyme-Linked Immunosorbent Assay?
IgG
57
What test for HIV involves Acrylamide gel electrophoresis and measures HIV antibodies binding to separated viral proteins?
Western Blot
58
What CD-4 level do opportunistic infections usually begin to occur?
Below 200
59
What are some of the viruses that act synergistically with HIV?
How is this manifested?
HHV-8 (Kaposi's Sarcoma)
| HPV (Cervical cancer)
60
Microglial nodules with multinucleated giant cells in HIV are indicative of what?
AIDS Encephalopathy
61
What are some of the Parasites of concern with AIDS pts?
Pneumocystis carinii (protozoan causes pneumonia)
62
What are the most common viral infections of the brain in patients with HIV?
Herpesvirus
| Cytomegalovirus
63
Name 4 parasites commonly found in AIDS patients.
Toxoplasmosis (brain)
Pneumocystis carinii (lungs)
Cryptosporidium (GI)
Isospora (GI)
64
Name 4 Fungi commonly affecting AIDS patients.
Cryptococcus
Histoplasma
Aspergillus
Candida
65
What makes up the Acute phase of AIDS?
| What timeframe?
Initial spike of virus leveling at about 9 weeks
66
How long is the typical chronic phase of clinical latency?
9 weeks to 9 or 10 years
67
At what T-cell level does a patient go from HIV to AIDS?
| What does the CDC call this?
200 CD4 T-cells/mm3
| CDC group 4
68
Aseptic meningitis occurs on what percentage of cases?
When does this occur?
10%
within 1-2 weeks of seroconversion (contracting virus)
69
When do the lymph nodes become infected with the HIV virus?
When do they involute?
Initial seeding comes from acute spike
(HIV replication continues in nodes throughout chronic phase)
Involution in Crisis phase
70
What invasive fibrillar condition causes atrophy through pressure to adjacent tissue?
Amyloidosis
71
What is the makeup of Amyloid tissue?
95% Fibrillar
5% Glycoproteins
arranged into beta-pleated sheets
72
What is the cause of Amyloidosis?
Immune system pathology/abnormality -or-
| Abnormal response to Chronic Infection
73
What are the 2 types of Amyloid and their derivations?
AL Amyloid: antibody light chains from neoplastic B-cells
(cancers like lymphoma/multiple myeloma)
AA Amyloid: Liver
74
AA Amyloid (from the liver) is produced in response to what 4 things?
Infections - TB
Inflammations - Alzheimers, RA, Inflammatory Bowel
Neoplasms - Renal cell carcinomas
I.V. drug use
75
T/F
| Amyloid is a type of cancer.
False
This tissue doesn't invade so much as compress.
76
Amyloid biopsies are analyzed using what stain?
What does this look like under light microscopy?
Polarized light?
Congo Red
Red
Apple-green birefringence
77
Name 3 Granulomatous causes of fever.
```
Sarcoidosis
Crohn's Disease
Giant Cell (temporal) Arteritis
```
78
```
What class of drugs is associated with Neuroleptic Malignant Syndrome?
examples?
```
Anti-psychotics and neuroleptics
Thorazine, Haloperidol, Metoclopramide
79
If a FUO (fever of unknown origin) is present, what organ systems are most likely?
Respiratory
Urinary
Skin
Soft Tissue
(these make up 80% of infections)
80
Any substance causing a fever is called?
a Pyrogen
81
In what 2 ways do bacteria cause fever?
Directly - bacterial pyrogens
| Indirectly - Cytokines
82
What are the dominant cytokines that cause fever?
IL-1, 2, and 6
| TNF
83
What mediates Cytokine action in the Hypothalamus?
| the hypothalamus synthesizes this in response to cytokines
Prostaglandins (PGE-2)
84
What directly affects the hypothalamic thermoregulatory center?
PGE-2
| prostaglandin
85
How does aspirin block fever?
Inhibits IL-1 from making PGE-2 in hypothalamus
86
What type of bacteria release endotoxins?
Gram negative
87
What substance in gram negative bacteria make up endotoxin?
LPS - lipopolysaccharide
88
What does the complex of LPS/LPS binding protein in serum bind to?
Toll-like receptors
*on WBC's/tissues. This releases inflammatory mediators
89
What do Toll-Like receptors do?
Release Inflammatory Cytokines
90
What is another name for fever?
Pyrexia
91
Neoplasms in what areas most often cause fever?
Blood, Lymph, Kidney, Liver, Brain
92
Systemic Inflammatory diseases are either _____ or ______.
Autoimmune
| Granulomatous
93
T/F
| Sickle cell, seizure, dehydration, and gout can all cause fever.
True
94
Name on enterotoxin that cause fever.
St aureus
95
What 3 cytokines stimulate the production of PGE-2?
IL-1, IL-2, and TNF
96
What causes Septic shock?
LPS from Gram Negative bacteria
Gram Positive Fungi
*over 100k deaths/yr in ICU's
97
What are the most common Gram negative bacteria causing Septic shock?
(probably not on test)
```
E.coli
Proteus
Serratia
Pseudomonas
K. pneumoniae
Bacteroides
```
98
T/F
| Abnormal hypothalamic function is a common cause of fever.
False
very rare
99
What is an autosomal dominant skeletal muscle condition involving hypothalamic dopamine inhibition that causes increased temperature in response to Halothane or Succinylcholine?
This presents with muscle breakdown (rhabdomyoloysis) and rigidity.
Malignant Hyperthermia
100
Band neutrophils at 12-15k WBC/mm3 suggest what?
Leukocytosis/Acute inflammation
101
An amorphous eosinophilic hyaline-appearing extracellular substance:
Amyloidosis
102
What type of deposit is typical in Alzheimer's Disease?
Amyloid
103
What is the most common organ affected by amyloidosis (causes most deaths)?
What is the general features of gross examination on all organs?
Kidneys (all nephrons affected)
Waxy, pale
104
What is often the 1st symptom in Amyloidosis?
Proteinuria
Nephrotic Syndrome 70% pts
105
Why don't AIDS pts die of bacterial infections?
B-cell humoral immunity remains intact.
106
What are the two HIV types?
| What is most common in the US?
HIV-1 and HIV-2
HIV-1 in US
HIV-2 in Africa
107
Pneumocystis in AIDS patients is caused by what?
a fungus
108
What blood product used by hemophiliacs transmitted HIV?
Factor 8
109
What is the risk of accidental seroconversion with HIV?
| Hep B?
0.3%
| 30%
110
HIV preferentially infects T-helper lymphocytes, but also infects what other cells?
```
Monocytes (macrophage)
Dendritic cells (fixed macrophage)
Microglial cells (brain macrophage)
```
111
What is a common fatal tumor in AIDS patients?
B-cell Non-Hodgkin's Lymphoma
| from brain
112
Kaposi Sarcoma is caused by an ______ that is activated in endothelial cells in the small bv's.
Oncogene
| HIV and EBV may also act as oncogenes
113
A disease affecting the lymph nodes is...
Lymphadenopathy
114
Why does the last stages of AIDS almost never contain granulomas?
No immune cells
115
What is the only organ that shows HIV-specific changes?
Brain
| AIDS Encephalopathy - microglial response
116
What presents as dementia in AIDS patients?
HIV Subacute Encephalitis
117
What is the disease of the spinal cord found in 20-30% of AIDS pts resembling B12 deficiency?
Vacuolar Myelopathy
118
What are the 2 neurologic manifestations of AIDS in children?
Microencephaly
| Spasticity
119
DiGeorge Syndrome, aka...
Thymic Hypoplasia
120
What is the chromosome and gene affected in DiGeorge?
22
TBX1
note: this is 90% cases, other 10% autosomal dominant
121
What is a treatment for DiGeorge's?
Thymic or Marrow transplant
122
What causes Chronic Mucocutaneous Candidiasis?
Congenital lack of T-cell function
123
T/F
| Only males have Agammaglobulinemia of Bruton
True
*Remember, this is a B-cell disorder
124
What is the gene and chromosome defect in Agammaglobulinemia of Bruton?
Xq21 (Tyrosine Kinase ATK)
| X chromo
125
What is the most common primary immunodeficiency?
Selective IgA
126
What disease results in hypogammaglobulinemia from failure of B-cells to mature (by defect or improper T-cell signaling)?
Common Variable Immunodeficiency (CVID)
127
What is the classic triad of Wiskott-Aldrich Syndrome?
Thrombocytopenia
Infections
Eczema
128
Describe platelets in Wiskott-Aldrich Syndrome
Small in number
| Small in morphology
129
What antibody is absent or low in WAS?
| What cures WAS in 90% cases?
IgM
| Marrow transplant
130
What is the Boy in the Bubble disease?
What enzyme is lacking?
Treatment?
Severe Combined Immunodeficiency
ADA - adenosine deaminase
Bone marrow transplantation
131
What does a titer measure?
The dilution at which detectable
132
What is the second most common autoimmune disorder that destroys the lacrimal and salivary glands?
Sjogren Syndrome
133
What antibodies are present in Sjogren's?
SS-A
SS-B
also Rheumatoid factor
134
If diagnosed with Sjogren's Syndrome, the risk of _____ increases 40x.
Lymphoma
135
What combines some features from SLE, Scleroderma, and RA?
Mixed Connective Tissue Disease
| MCTD
136
What ANA's are present in Mixed Connective Tissue Disease?
UI-RPN
| Uridine-rich ribonucleoprotein
