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Tim's Cards > Immunology III > Flashcards

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

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2
Q

What is an ANA?

A

Anti-nuclear Antibody

*diagnosis of autoimmune disorders
but “not diagnostic of any”
**higher titer, worse the disease

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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

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4
Q

What is Progressive System Sclerosis?

A

Scleroderma

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5
Q

What are the most common organs affected by Progressive System Sclerosis (Scleroderma)?

A

GI tract, heart, kidneys, muscles, lungs

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6
Q

T/F

Scleroderma limits itself to certain organs.

A

False.

Many organs involved

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7
Q

What autoimmune disorder begins with Raynaud phenomenon (distal ischemia)?

A

Scleroderma

*this can lead to auto-amputation

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8
Q

What often causes sudden death in Scleroderma?

A

Ventricular arrhythmias

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9
Q

What is the milder form of Scleroderma called?

A

CREST syndrome

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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)
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11
Q

What are 2 Autoimmune diseases of the muscle?

note: they are chronic inflammatory myopathies

A

Polymyositis

Dermatomyositis

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12
Q

What virus has been implicated in Polymyositis/Dermatomyosits?

A

Picornaviruses

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13
Q

What are the most specific antibodies associated with Polymyositis/Dermatomyositis?

A

anti-Jo-1

*an MSA (myositis specific antibody)

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14
Q

What is Heliotropic Rash specific to?

note: this is humorally-mediated

A

Dermatomyositis eyelids

*no angiopathy in Polymyositis

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15
Q

What are Anti-centromeric antibodies associated with?

A

Both Scleroderma and CREST

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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)

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17
Q

What cancers are most associated with Polymyositis and Dermatomyositis in men and women?

A

Men: Lung, colon, stomach

Women: Breast, ovaries, uterus

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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)

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19
Q

What does SLE stand for?

A

Systemic Lups Erythematosus

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20
Q

SLE affects all organs, but preferentially targets…

A

Kidneys
Serousal membranes
Joints
Skin

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21
Q

What are two risk factors involved in SLE?

A

Female sex hormones

Familial correlation

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22
Q

What is the staining pattern of a homogenous, non-specific ANA?

A

Diffuse

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23
Q

What is the staining pattern of Anti-double stranded DNA seen in SLE?

A

Rim

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24
Q

What is the staining pattern of the antibody to extractable nuclear antigens seen in Mixed Connective Tissue Disorder?

A

Speckled

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25
Q

What is the staining pattern seen in PSS (Progressive Systemic Sclerosis)?

A

Nucleolar

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26
Q

What staining pattern is seen for the antibody in CREST syndrome?

A

Centromeric

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27
Q

*Which of the following types of Lupus Glomerulonephritis is mildest with an excellent prognosis?

A

Mesangial Lupus

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28
Q

How does SLE manifest on the skin?

A

Butterfly on face

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29
Q

How does SLE affect serous membranes?

A

1/3 have pleuritis with pleural effusion

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30
Q

What causes changes in the brain in Lupus?

A

Interstitial fibrosis

Vasculitis leads to hemmorrhage and infarction

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31
Q

What type of carditis is involved in Lupus?

A

Pancarditis.

pericarditis - most common
endocarditis
myocarditis

(nonbacterial vegetations (Libman-Sacks Endo) in latter two)

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32
Q

What autoantibodies are prevalent in Drug-Induced Lupus?

A

Anti-Histone Antibodies

DS-DNA and SM antigen UNusual

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33
Q

What autoimmune Syndrome is characterized by dry eyes and dry mouth (keratoconjunctivitis and xerostomia), often secondary to SLE and RA?

A

Sjogren Syndrome

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34
Q

What condition is marked by the lack of all immunoglubulin in the blood?

A

Agammaglobulinemia of Bruton

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35
Q

What type of disorder is Agammaglobulinemia of Bruton?

A

Congenital X-linked

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36
Q

What types of infections are handled well in pts with Agammaglobulinemia of Bruton?

A

Most viral/fungal

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37
Q

What gene and enzyme is broken in congenital x-linked Agammaglobulinemia of Bruton?

A

Xq21
B-cell Tyrosine Kinase
(ATK)

*critical for the maturation of B-cells

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38
Q

What types of infections are common in Agammaglobulinemia of Bruton?

What is the treatment for the disease?

A

Bacterial

Replacement of all immunoglobulins

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39
Q

When is Agammaglobulinemia of Bruton first observed?

A

5-8 months, when maternal antibodies wear out, usually with Pyogenic Infections

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40
Q

What causes RA-like conditions in x-linked agammaglobulinemia of Bruton pts?

A

Mycoplasma

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41
Q

What is another name for Swiss-type Agammaglobulinemia?

What does it affect?

A

Severe Combined Immunodeficiency

T and B cells

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42
Q

What are the two types of Severe Combined Immunodeficiency?

Treatment?

A

X-linked (more common)
Autosomal recessive

Bone marrow transplantation

(this is boy in the bubble)

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43
Q

What is embryologically defective in DiGeorge Syndrome?

A

3rd and 4th pharyngeal pouches

*Thymus and Parathyroids
hypocalcemia and tetany prevalent b/c no parathyroids

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44
Q

What cell is completely lacking in DiGeorge syndrome?

A

T-cells

no thymus

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45
Q

What are the internal and external manifestations of DiGeorge’s?

A

Internal: interrupted aortic arch, microencephaly

External: Low set ears, cleft lips

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46
Q

T/F

DiGeorge’s pts have a good response to Fungal and Viral infections.

A

False

complete lack of T-cells

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47
Q

What are the 4 main receptors on the HIV virus?

A 
gp 120     (lollipop)
gp 41     (lollipop stick)

p17 (outer)
p24 (inner)

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48
Q

What is the most common cause of meningitis in AIDS patients?

A

Cryptococcus

a fungus

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49
Q

What cells are affected in HIV?

these cells carry HIV

A

CD4 T-helper cells

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50
Q

What type of virus is HIV?

A

RNA retrovirus

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51
Q

What is the most common cause of death in AIDS patients?

A

Pneumocystis

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52
Q

What increases the transmission of HIV?

A

co-existing STI’s
male to female (not female to male)
anal sex

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53
Q

T/F

Breast milk cannot transmit HIV.

A

False

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54
Q

What are the 3 neoplasms of HIV?

A

Kaposi sarcoma
B-cell non-Hodgkin lymphoma (Primary lymphoma of brain)
Invasive caner of uterine cervix

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55
Q

What is the presumptive and definitive test for HIV?

A

ELISA - presumptive

Western Blot - definitive

56
Q

What antibody is bound in Enzyme-Linked Immunosorbent Assay?

A

IgG

57
Q

What test for HIV involves Acrylamide gel electrophoresis and measures HIV antibodies binding to separated viral proteins?

A

Western Blot

58
Q

What CD-4 level do opportunistic infections usually begin to occur?

A

Below 200

59
Q

What are some of the viruses that act synergistically with HIV?
How is this manifested?

A

HHV-8 (Kaposi’s Sarcoma)

HPV (Cervical cancer)

60
Q

Microglial nodules with multinucleated giant cells in HIV are indicative of what?

A

AIDS Encephalopathy

61
Q

What are some of the Parasites of concern with AIDS pts?

A

Pneumocystis carinii (protozoan causes pneumonia)

62
Q

What are the most common viral infections of the brain in patients with HIV?

A

Herpesvirus

Cytomegalovirus

63
Q

Name 4 parasites commonly found in AIDS patients.

A

Toxoplasmosis (brain)
Pneumocystis carinii (lungs)
Cryptosporidium (GI)
Isospora (GI)

64
Q

Name 4 Fungi commonly affecting AIDS patients.

A

Cryptococcus
Histoplasma
Aspergillus
Candida

65
Q

What makes up the Acute phase of AIDS?

What timeframe?

A

Initial spike of virus leveling at about 9 weeks

66
Q

How long is the typical chronic phase of clinical latency?

A

9 weeks to 9 or 10 years

67
Q

At what T-cell level does a patient go from HIV to AIDS?

What does the CDC call this?

A

200 CD4 T-cells/mm3

CDC group 4

68
Q

Aseptic meningitis occurs on what percentage of cases?

When does this occur?

A

10%

within 1-2 weeks of seroconversion (contracting virus)

69
Q

When do the lymph nodes become infected with the HIV virus?

When do they involute?

A

Initial seeding comes from acute spike

(HIV replication continues in nodes throughout chronic phase)

Involution in Crisis phase

70
Q

What invasive fibrillar condition causes atrophy through pressure to adjacent tissue?

A

Amyloidosis

71
Q

What is the makeup of Amyloid tissue?

A

95% Fibrillar
5% Glycoproteins
arranged into beta-pleated sheets

72
Q

What is the cause of Amyloidosis?

A

Immune system pathology/abnormality -or-

Abnormal response to Chronic Infection

73
Q

What are the 2 types of Amyloid and their derivations?

A

AL Amyloid: antibody light chains from neoplastic B-cells
(cancers like lymphoma/multiple myeloma)

AA Amyloid: Liver

74
Q

AA Amyloid (from the liver) is produced in response to what 4 things?

A

Infections - TB
Inflammations - Alzheimers, RA, Inflammatory Bowel
Neoplasms - Renal cell carcinomas
I.V. drug use

75
Q

T/F

Amyloid is a type of cancer.

A

False

This tissue doesn’t invade so much as compress.

76
Q

Amyloid biopsies are analyzed using what stain?
What does this look like under light microscopy?
Polarized light?

A

Congo Red
Red
Apple-green birefringence

77
Q

Name 3 Granulomatous causes of fever.

A 
Sarcoidosis
Crohn's Disease
Giant Cell (temporal) Arteritis
78
Q 
What class of drugs is associated with Neuroleptic Malignant Syndrome?
examples?
A

Anti-psychotics and neuroleptics

Thorazine, Haloperidol, Metoclopramide

79
Q

If a FUO (fever of unknown origin) is present, what organ systems are most likely?

A

Respiratory
Urinary
Skin
Soft Tissue

(these make up 80% of infections)

80
Q

Any substance causing a fever is called?

A

a Pyrogen

81
Q

In what 2 ways do bacteria cause fever?

A

Directly - bacterial pyrogens

Indirectly - Cytokines

82
Q

What are the dominant cytokines that cause fever?

A

IL-1, 2, and 6

TNF

83
Q

What mediates Cytokine action in the Hypothalamus?

the hypothalamus synthesizes this in response to cytokines

A

Prostaglandins (PGE-2)

84
Q

What directly affects the hypothalamic thermoregulatory center?

A

PGE-2

prostaglandin

85
Q

How does aspirin block fever?

A

Inhibits IL-1 from making PGE-2 in hypothalamus

86
Q

What type of bacteria release endotoxins?

A

Gram negative

87
Q

What substance in gram negative bacteria make up endotoxin?

A

LPS - lipopolysaccharide

88
Q

What does the complex of LPS/LPS binding protein in serum bind to?

A

Toll-like receptors

*on WBC’s/tissues. This releases inflammatory mediators

89
Q

What do Toll-Like receptors do?

A

Release Inflammatory Cytokines

90
Q

What is another name for fever?

A

Pyrexia

91
Q

Neoplasms in what areas most often cause fever?

A

Blood, Lymph, Kidney, Liver, Brain

92
Q

Systemic Inflammatory diseases are either _____ or ______.

A

Autoimmune

Granulomatous

93
Q

T/F

Sickle cell, seizure, dehydration, and gout can all cause fever.

A

True

94
Q

Name on enterotoxin that cause fever.

A

St aureus

95
Q

What 3 cytokines stimulate the production of PGE-2?

A

IL-1, IL-2, and TNF

96
Q

What causes Septic shock?

A

LPS from Gram Negative bacteria
Gram Positive Fungi

*over 100k deaths/yr in ICU’s

97
Q

What are the most common Gram negative bacteria causing Septic shock?

(probably not on test)

A 
E.coli
Proteus
Serratia
Pseudomonas
K. pneumoniae
Bacteroides
98
Q

T/F

Abnormal hypothalamic function is a common cause of fever.

A

False

very rare

99
Q

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.

A

Malignant Hyperthermia

100
Q

Band neutrophils at 12-15k WBC/mm3 suggest what?

A

Leukocytosis/Acute inflammation

101
Q

An amorphous eosinophilic hyaline-appearing extracellular substance:

A

Amyloidosis

102
Q

What type of deposit is typical in Alzheimer’s Disease?

A

Amyloid

103
Q

What is the most common organ affected by amyloidosis (causes most deaths)?
What is the general features of gross examination on all organs?

A

Kidneys (all nephrons affected)

Waxy, pale

104
Q

What is often the 1st symptom in Amyloidosis?

A

Proteinuria

Nephrotic Syndrome 70% pts

105
Q

Why don’t AIDS pts die of bacterial infections?

A

B-cell humoral immunity remains intact.

106
Q

What are the two HIV types?

What is most common in the US?

A

HIV-1 and HIV-2
HIV-1 in US
HIV-2 in Africa

107
Q

Pneumocystis in AIDS patients is caused by what?

A

a fungus

108
Q

What blood product used by hemophiliacs transmitted HIV?

A

Factor 8

109
Q

What is the risk of accidental seroconversion with HIV?

Hep B?

A

0.3%

30%

110
Q

HIV preferentially infects T-helper lymphocytes, but also infects what other cells?

A 
Monocytes (macrophage)
Dendritic cells (fixed macrophage)
Microglial cells (brain macrophage)
111
Q

What is a common fatal tumor in AIDS patients?

A

B-cell Non-Hodgkin’s Lymphoma

from brain

112
Q

Kaposi Sarcoma is caused by an ______ that is activated in endothelial cells in the small bv’s.

A

Oncogene

HIV and EBV may also act as oncogenes

113
Q

A disease affecting the lymph nodes is…

A

Lymphadenopathy

114
Q

Why does the last stages of AIDS almost never contain granulomas?

A

No immune cells

115
Q

What is the only organ that shows HIV-specific changes?

A

Brain

AIDS Encephalopathy - microglial response

116
Q

What presents as dementia in AIDS patients?

A

HIV Subacute Encephalitis

117
Q

What is the disease of the spinal cord found in 20-30% of AIDS pts resembling B12 deficiency?

A

Vacuolar Myelopathy

118
Q

What are the 2 neurologic manifestations of AIDS in children?

A

Microencephaly

Spasticity

119
Q

DiGeorge Syndrome, aka…

A

Thymic Hypoplasia

120
Q

What is the chromosome and gene affected in DiGeorge?

A

22
TBX1

note: this is 90% cases, other 10% autosomal dominant

121
Q

What is a treatment for DiGeorge’s?

A

Thymic or Marrow transplant

122
Q

What causes Chronic Mucocutaneous Candidiasis?

A

Congenital lack of T-cell function

123
Q

T/F

Only males have Agammaglobulinemia of Bruton

A

True

*Remember, this is a B-cell disorder

124
Q

What is the gene and chromosome defect in Agammaglobulinemia of Bruton?

A

Xq21 (Tyrosine Kinase ATK)

X chromo

125
Q

What is the most common primary immunodeficiency?

A

Selective IgA

126
Q

What disease results in hypogammaglobulinemia from failure of B-cells to mature (by defect or improper T-cell signaling)?

A

Common Variable Immunodeficiency (CVID)

127
Q

What is the classic triad of Wiskott-Aldrich Syndrome?

A

Thrombocytopenia
Infections
Eczema

128
Q

Describe platelets in Wiskott-Aldrich Syndrome

A

Small in number

Small in morphology

129
Q

What antibody is absent or low in WAS?

What cures WAS in 90% cases?

A

IgM

Marrow transplant

130
Q

What is the Boy in the Bubble disease?

What enzyme is lacking?

Treatment?

A

Severe Combined Immunodeficiency

ADA - adenosine deaminase

Bone marrow transplantation

131
Q

What does a titer measure?

A

The dilution at which detectable

132
Q

What is the second most common autoimmune disorder that destroys the lacrimal and salivary glands?

A

Sjogren Syndrome

133
Q

What antibodies are present in Sjogren’s?

A

SS-A
SS-B

also Rheumatoid factor

134
Q

If diagnosed with Sjogren’s Syndrome, the risk of _____ increases 40x.

A

Lymphoma

135
Q

What combines some features from SLE, Scleroderma, and RA?

A

Mixed Connective Tissue Disease

MCTD

136
Q

What ANA’s are present in Mixed Connective Tissue Disease?

A

UI-RPN

Uridine-rich ribonucleoprotein

Tim's Cards (140 decks)

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