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