Chapter 6 Flashcards
1
Q
What type of cells are activated in a type I HS?
A
CD4+ TH2 responses
2
Q
What are the roles of TH2 T cells
A
produce cytokines
IL4 and IL13 which lead to increase B cell IgE synthesis
3
Q
what chemokines can cause mast cell degranulation
A
IL8 and adenosine
4
Q
what drugs can cause mast cell degranulation
A
codein, morphine
5
Q
what phsyical stimuli can cause mast cell degranulation
A
sunlight, trauma, heart/cold
6
Q
What are released in the initial response of type I HS
A
histamine, chymase.tryptase
heparin
7
Q
what are released in the delayed phase type I HS
A
lipid mediators LKT B4, C4, D4, E4 Porstaglandin D2 PAF TNF and IL1 IL4 for + feedback
8
Q
What cells are responsible for the Ab-dep cell-mediated cytotoxicity in type II HS
A
NK
9
Q
What are common diseases that are type II HS
A
Good pastures (Ab agaainst alveolar and glomerular BM)
Myasthenia Gravis
DM II
10
Q
What type of HS is SLE
A
III IC
11
Q
What type of HS is post-streptococcal glomerulonephritis
A
III IC
12
Q
What is a disease type III HS from infectious agent
A
polyareteritis nodosa
13
Q
what type of rxn is acute serum sickness
A
type III
14
Q
Describe injury causes by IC deposition
A
activates C’
can have fibrinoid necrosis from protein accumulation
15
Q
What is type IV HS
A
T cell mediated CD8
16
Q
describe the CD4 rxn in type IV HS
A
DTH
TH1 with macrophages
TH17 with neutrophils
17
Q
What causes CD4 switch to TH1
A
IL-12, then TH1 makes IFNy which increases TH1
18
Q
what causes CD4 witch to TH17
A
IL 1,6,23 and TGFb
19
Q
what is the role of IFNyin DTH
A
Macrophage activation increasing TNF and IL1 and IL12
20
Q
what is the role of TH17 cells in DTH
A
recruit, activate neutrophils and monocytes
21
Q
What does a granuloma look like
A
not around central blood vessel and lots of epithelial histiocytes
22
Q
types of type IV HS
A
RA DM I MS Geullain Barre Crohns contact dermatitis
23
Q
describe CTL component of type IV HS
A
granzymes and performs
CTL don’t need prior recognition of tumors or viruses
24
Q
What type of HS reaction is graft rejection
A
type IV DTH
25
What is AIRE
autoimmune regulator, important in central tolerance
26
Where are T cells found in thymus
paracortical regions
27
What is clonal deletion
the method of peripheral tolerance for B and T cells with high affinity to self
28
What cytokines are responsible for anergy
B7-CD28 whcih irreversible inactivate T cells when recognize self
29
What is PTPN-22 encode and what autoimmune diseases are related
tyrosine phosphotase
| DM and RA
30
What is NOD2 endode and what AI disease
sensing IC microbes
| IBD
31
What IL polymorphisms are assoc with MS
2 and 17
32
What are characteristics of SLE
auto Ab to ds DNA, smith Ab and ANAs detected by immunofleuorescence
33
antiphospholiipid Ab to cardiolipin indicates what
+syphilis, seen in SLE
34
how can SLE cause recurrent thrombosis
lupus anticoagulants
35
What C' parts are deficient withs ome SLE
C2, C4, C1q--> IC aggregation
36
What immune cells are targeted in SLE
B cells because self react
37
What are included in criteria for SLE
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neuro disorder, hematologic disorder, immuno disorder and ANAs
38
What happens to kidney in SLE
increase cell infiltration, microvascular thormbosis, vas wall deposition, increased heamturia, proteinuria, HTN, renal insufficiency
39
What is typical of joints in SLE
non-erosive synovitis
40
What type of necrosis occurs in heart in SLE
fibrinoid
41
What are verrucuos, Libman-sacks endocarditis
assoc with SLE
| small vegetations sup and inverior of heart valves
42
What occurs in spleen in SLE
follicular hyperplasia (onion skin appearance)
43
What are the top 10 clinical manifestations of SLE
hematologic, arthritis, skin, fever, fatigue, weight loss, renal, neuropshychiatric, pleuritis, myalgia
44
What causes chronic discoid lupus erythematous
deposition of Ig C3 at dermal epidermal junction
45
Ab to SS-A and HLA-DR3 are assoc with what
subacute cutaneous lupus erythematous
46
what drugs can induce SLE
hydralazine procainamide, Isonizid, D-pennicliamine
47
What are the recombination genes for T cells
Rag 1 and 2
48
What are the components of the TCR complex
CD3 and zig
| CD4,8,2,28
49
What R are on B cells that are essential
Fc, C' and CD40
50
Without CD40 what happens
no Ab production because B cells cannot interact with T cells CD40L
hyper IgM
51
What is the R for EBV
CR2 or CD21
52
What type of R are on dedritic cells
TLRs and mannose R for microbes
53
Where are follicular DC found
germinal centers in spleen and lymph nodes
54
What R do follicular DC have
FcR for IgG and R for C3b
55
What is the key effector cell to eliminate IC microbes
macrophages
56
What CD are used to identify Nk cells
16 and 56
57
What R inhibit NK cells
Ig-like, CD94 family of lectins
58
what cytokines do macrophages secrete
TNF IL1
59
What is related with HLA b27
ankylosing spondylitis
60
What locus of HLA are autoimmune related to
DR
61
HLA-A is associ with what disease
hereditary hemachromatosis
62
What are the costimulators for APCs on T cells
B7
63
what are the costimulators for APCs on naive T cells
CD28
64
during earliest response to microbe in cell mediated immunity what cells and IL are released
CD4 and IL2
65
Where does isotype switching of Ig take place
in germinal centers formed by proliferating B cells
66
what Ig activate C'
IgG and IgM
67
What tissues are involved in RA
joints, skin vessels, lungs and heart
68
What is primary form sjogren
sicca which is fibrosis lacrimal and salivary glands
69
What disease is assoc with Ab SS-A Ro and SS-B La
sjogren syndrome, sicca
70
lymphocytic infiltration salivary glands, ductal lining hyperplasia, atrophy acini and hyalinizaiton and drying of corneal epithelium point to what
sicca syndrome
71
what is essential for Dx of sicca, or primary sjogren
lip biopsy
72
what are the clinical features of sjogren syndrome
keratoconjuntivitis, xerostomia, parotid gland enlargement, synovitis, other AI disorders
73
What is systemic sclerosis from
chornic disease with chronic inflmmation from AI with widespread damage and progressive fibrosis
74
What is diffuse scleroderma
widespread skin involvement with rapid progression
75
what is limited scleroderma
skin involvement is on fingers toes, face
| CREST
76
What ANAs are found assoc with diffuse scleroderma
DNA topoisomerase I leading to pulm fibrosis and peripheral vasc disease
77
what Ab are found assoc with CREST
anti centromere
78
What can happen with scleroderma in alimentary track
progressive atrophy with Barret metaplasia and loss of microvilli leading to malabsorption
79
What type of necrosis occurs in kidney with scleroderma
fibrinoid, death from renal failure common
80
can you Dx patient based on CREST symptoms
no, need Ab against centromeres
81
What are examples of inflammatory myopathies
dermatomyositis, polymyositis, inclusion-body myositis
82
how do you Dx inflammatory myopathies
muscle biopsy
83
high titers of Abs to ribonucleoprotein particle containing U1 RNP is assoc with what
mixed CT syndrome
84
if patient has less than 4 criteria SLE than what is high on Ddx
mixed CT
85
mutations on Xq21.22 is probably causing what
well its bruton tyrosine kinase, so failure development B cells.
86
What type of infections are people with Burtons Agammaglobulinemia prone to
respiratory
| viral--> giardia resisted usually IgA
87
what are histological appearance of burtons agammaglobulinemia
underdevelopled germinal centers of lymph nodes, peyers patches, appendix tonsils
no plasma cells
88
what type of inheritance is burtons agammaglobulinemia
x linked recessive
89
Normal # B cells, no plasma cells, recurrent sinopulmonary infections, recurrent herpes, persistent diarrhea
hyperplastic B cell areas
probably what?
common variable immunodeficiency
90
what descent is common to have isolated IgA deficiency
european
91
what type of infections occur with isolated IgA deficiency
sinopulmonary and diarrhea
| but usually asymptomatic
92
What can cause hyper IgM syndrome
X linked CD40L on Xq26
| auto recessive mutations in CD40 or cytosine deaminase
93
What occurs clincally in hyper IgM syndrome
recurrent pyogenic infections
| autoimmune hemolytic anemia, thrombocytopenia, neutropenia
94
What is the result of a deletion 22q11
thymic hypoplasia, DiGeorge syndrome
| lack of parathyroid, congenital heart defects, abnormal facies
95
What causes x linked SCID
common y-chain subunit cytokine R deficient IL17 and 15
96
what causes autosomal recessive SCID
adenosine deaminase deficiency
97
what are histo findings in types of SCID
x linked: thymus has epithelial cells resembling fetal thymus
ADA deficiency has Hassall's corpuscles
98
```
decreased IgM
normal IgG
increased IgA IgE
thrombocytopenia and eczema
non-hodgkin B cell lymphoma
```
wiskott aldrich syndrome WASP Xp11.23
99
What occurs with C3 deficiency
serious, pyogenic infections
| incidence of IC mediated glomerulonephritis
100
What are the types of amyloid
AL in plasma cell tumors
AA in secondary amyliodosis
B-amyloid protein in alzheimers
101
What type of amyloid is a problem with hemodialysis
B2-microglobulin MHC class I
102
what does disease does primary amyloidosis occur with
multiple myeloma
103
bence jones proteins are assoc with what
primary amyloidosis
104
What conditions are commonly assoc with reactive system amyloidosis
RA, heroin abusers
renal cell carcinoma
hodkin lymphoma
105
what is familial mediterranean fever
autoinflamm
increased IL1 production
attachs of fever and inflammation of serosal surfaces via AA proteins
106
what areas are assoc with familial med fever
armenian, sephardic jewish and arabic
107
what is a sago spleen vs lauraceous spleen
sago: tapioca like granules on gross inspection
lauraceous: red pulp in splenic sinuses
108
where do amyloids deposit in liver
space of Disse-- hepatic parenchymal cells and sinusoids then kupffer cells
109
what are clinical features amyloidosis
proteinuria
| insidious CHF and arrythmias, chronic constrictive pericarditis, diarrhea
110
What is classifcation A HIV
asymptomatic, acute. sometimes persistent generalized lymphadenopathy
A1 >500 cell A3 <200
111
What is calssication B HIV
not A or C, symptomatic
| B1 >500 B3<200
112
what is classification C HIV
AIDS indicator conditions: constitutional disease, neuro disease or neoplasm
113
Ab marker for drug induced SLE
antihistone
114
Ab marker for polymyositis/dermatomyositis
Jo-1
115
Ab mixed CT disease
Anti-U1-ribonucleotide protein
116
x lnked SCID is mutations in what
y light chain of cytokine R
117
most serious complication of diffuse scleroderma
malignant hypertension
118
what cell is responsible for fibrotic chances in limited scleroderma
CD4
119
HIV patients most at risk for what neoplasm
non-hodgkin B cell lymphoma
120
what condition is most likely in someone with polymyositis, or Jo-1 Ab titers
myocarditis
121
what other condition besides ankylosing spondylitis is assoc with HLA b27
psoriatic arthropathy
122
what mutation in RNA HIV virus accounts for drug R
reverse transcriptase
123
what pneumoniae is common in AIDS
pneumocystitis jiroveci
124
what mutation is with SCID auto recessive
adenosine deaminase deficiency
125
What Ab is in Goodpasture syndrome
type IV collagen so attacks BM
126
What Ab assoc with sjogrens
SS-B
127
what is a common cause senial cardiac amyloidosis
deposition transtheyretin
