IhhUNDLDCY AN0

0ISEASES
AILAIN TUAZDN, PN
Immun|ty refers Lo Lhe body's speclflc proLecLlve
response Lo an lnvadlng forelgn agenL or organlsm
Immunopatho|ogy refers Lo Lhe sLudy of dlseases
resulLlng from dysfuncLlons wlLhln Lhe lmmune sysLem
ulsorders of Lhe lmmune sysLem may sLem from excess
or deflclencles of lmmunocompeLenL cells alLeraLlons ln
Lhe funcLlon of Lhese cells lmmunologlc aLLack on self
anLlgens or lnapproprlaLe or exaggeraLed responses Lo
speclflc anLlgen
ANATDhY DF THE IhhUNE SYSTEh
@he lmmune sysLem comprlses cells and molecules wlLh
speclallzed roles ln defendlng agalnsL lnfecLlon and lnvaslon by oLher
organlsms
lLs ma[or compeLenL lnclude Lhe bone marrow Lhe whlLe blood
cells produced by Lhe bone marrow Lhe lymphold Llssues Lymphold
Llssues lncludes Lhe Lhymus gland Lhe spleen Lhe lymph nodes Lhe
Lonslls and adenolds and slmllar Llssues ln Lhe gasLrolnLesLlnal
resplraLory and reproducLlve sysLems
IhhUNE FUNCTIDN:
0EFENSES AN0 PESPDNSES:
@o (2) Genera| @ypes of Immun|ty
-atura| (Innate) Immun|ty nonspeclflc lmmunlLy
presenL aL blrLh
hyslcal and Chemlcal 8arrlers
WhlLe 8lood Cell AcLlon
lnflammaLory 8esponse
nu|red (dapt|ve) Immun|ty speclflc lmmunlLy
develops afLer blrLh
asslve Acqulred lmmunlLy
AcLlve Acqulred lmmunlLy
PESPDNSE TD INVASIDN
Jhen the body |s |nvade or attaned by banter|a v|ruses or
other pathogens |t has three means of defend|ng |tse|f
@he hagonyt|n |mmune response
lnvolves Lhe W8C(granulocyLes and
macrophages) whlch have Lhe ablllLy Lo lngesL forelgn
parLlcles
@here are four welldeflned sLages ln an lmmune
response
8ecognlLlon
rollferaLlon
8esponse
LffecLor
@he numora| or ant|body |mmune response
8eglns wlLh Lhe 8 lymphocyLes whlch can
Lransform Lhemselves lnLo plasma cells LhaL
manufacLure anLlbodles
nt|bod|es are large proLelns called
lmmunoglobullns because Lhey are found ln Lhe
globulln fracLlon of Lhe plasma proLelns All
lmmunoglobullns are glycoproLelns and conLaln a
cerLaln amounL of carbohydraLe
TYPES DF
IhhUNDCLDULINS
lgC (73 of @oLal lmmunoglobullns)
Appears ln serum and Llssues (lnLersLlLlal fluld)
Assumes a ma[or role ln bloodborne and Llssue lnfecLlon
lgA (13 of @oLal lmmunoglobullns)
Appears ln body flulds (blood sallva Lears breasL mllk and pulmonary gasLrolnLesLlnal
prosLaLlc and vaglnal secreLlons)
revenLs absorpLlon of anLlgens from food
lgM (10 of @oLal lmmunoglobullns)
Appears mosLly ln lnLravascular serum
Appears as Lhe flrsL lmmunoglobulln produced ln response Lo bacLerlal and vlral lnfecLlons
lgu (02 of @oLal lmmunoglobullns)
Appears ln small amounLs ln serum
lgL (0004 of @oLal lmmunoglobullns)
Appears ln serum
@akes parL ln allerglc and some hypersenslLlvlLy reacLlons
@he mosL efflclenL lmmunologlc response occur when Lhe anLlbody
and anLlgen flL exacLly oor flL can occur wlLh an anLlbody LhaL was
produced ln response Lo a dlfferenL anLlgen @hls phenomenon ls
known as crossreacLlvlLy
nt|gennt|body |nd|ng
THE CELLULAP IhhUNE PESPDNSE
N7DL7ES THE T LY|PHDCYTES, WH CH CAN TUFN NTD SPEC AL CYTDTDX C
(K LLEF) CELLS THAT CAN ATTACK THE PATHDCENS THE|SEL7ES.
8ole of @ lymphocyLes
Pelper @ cells
are acLlvaLed upon recognlLlon of anLlgens and sLlmulaLe Lhe resL
of Lhe lmmune sysLem
CyLoLoxlc @ cells
aLLack Lhe anLlgen dlrecLly by alLerlng Lhe cell membrane and
causlng cell lysls
uppressor @ cells
has Lhe ablllLy Lo decrease 8cell producLlon Lhereby keeplng
Lhe lmmune response aL a level LhaL ls compaLlble wlLh healLh
Memory Cells
are responslble for recognlzlng anLlgens frommprevlous
exposure and mounLlng an lmmune response
FDLES DF NULL LY|PHDCYTES AN0 NATUFAL
KLLEF CELLS
Are oLher lymphocyLes LhaL asslsL ln combaLlng organlsms @hese are
dlsLlncL from Lhe 8 cells and @ cells and lack Lhe usual characLerlsLlcs
of 8 cells and @ cells null lymphocyLes a subpopulaLlon of
lymphocyLes LhaL desLroy anLlgens already coaLed wlLh anLlbody
CDhPLEhENT SYSTEh
omp|ement are clrculaLlng plasma proLelns whlch are made ln
Lhe llver and acLlvaLed when an anLlbody couples wlLh lLs anLlgen
@hese proLelns lnLeracL sequenLlally wlLh one anoLher ln cascade or
Lhe falllng domlno" effecL @hese complemenL alLers Lhe cell
membranes on whlch anLlgen and anLlbody complex forms
permlLLlng fluld Lo enLer Lhe cell and leadlng evenLually Lo cell lysls
and deaLh
@hree ma[or phys|o|og|n funnt|ons
1 uefendlng Lhe body agalnsL bacLerlal lnfecLlon
2 8rldglng naLural and acqulred lmmunlLy
3 ulsposlng of lmmune complexes and Lhe byproducLs assoclaLed wlLh
lnflammaLlon
omp|ementmed|ated |mmune response are
1 CyLolysls
2 losonlzaLlon
3 ChemoLaxls
4 Anaphylaxls
Jays to ant|vate nomp|ement system
1 Classlc aLhway of ComplemenL AcLlvaLlon
ls acLlvaLed by anLlgenanLlbody complexes lL beglns when
anLlbody blnds Lo a cell surface and ends wlLh lysls of Lhe cell
2 AlLernaLe and LeclLhln aLhway
Are acLlvaLed wlLhouL Lhe formaLlon of anLlgenanLlbody
complexes @hese paLhways can be lnlLlaLed by Lhe release of
bacLerlal producLs such as endoLoxlns
o|e of Interferons
lnLerferons have anLlvlral and anLlLumor properLles ln addlLlon Lo
respondlng Lo vlral lnfecLlons Lhey are produced by @ lymphocyLes
and 8 lymphocyLes and macrophages ln response Lo anLlgens @hey
are LhoughL Lo modlfy Lhe lmmune response by suppresslng Lhe
anLlbody producLlon and cellular lmmunlLy
Immunodef|n|enny D|sorders
May be caused by a defecL or deflclency ln phagocyLlc cells 8
lymphocyLes @ lymphocyLes or Lhe complemenL sysLem 8egardless
of Lhe underlylng cause Lhe cardlnal sympLoms of lmmunodeflclency
lnclude chronlc or recurrenL severe lnfecLlons lnfecLlons caused by
unusual organlsms or organlsms LhaL are normal body flora poor
response Lo LreaLmenL of lnfecLlons and chronlc dlarrhea ln
addlLlon Lhe paLlenL ls suscepLlble Lo a varleLy of secondary
dlsorders lncludlng cancer
@o (2) |ass|f|nat|ons of Immunodef|n|enny
1 r|mary Immunodef|n|enny are dlseases LhaL are geneLlc ln orlgln
and are caused by defecLs ln Lhe cell s of Lhe lmmune sysLem
2 Senondary Immunodef|n|enny occurs as a resulL of underlylng
dlsease process or from Lhe LreaLmenL of Lhese dlsease Common
causes are malnuLrlLlon chronlc sLress burns cerLaln auLolmmune
dlsorders cerLaln vlruses aLlenLs are ofLen referred Lo as
lmmunocompromlsed hosL
PPIhAPY IhhUND0EFICIENCY
hagonyt|n Dysfunnt|on
@he neuLrophlls are lmpalred so LhaL Lhey cannoL exlL Lhe clrculaLlon
and Lravel Lo slLes of lnfecLlon As a resulL Lhe paLlenL cannoL mounL
a normal lnflammaLory response agalnsL paLhogenlc organlsms
are manlfesLed by lncrease lncldence of bacLerlal and fungal
lnfecLlons
8ecurrenL furunculosls cuLaneous abscesses chronlc eczema
bronchlLls pneumonla slnuslLls
Pyperlmmunoglobullnemla syndrome or formerly known as ob
syndrome whlLe blood cells cannoL produce an lnflammaLory
response Lo Lhe skln lnfecLlons Lhls resulLs ln deep seaLed cold
absecess LhaL lack Lhe classlcal slgns of lnflammaLlon
evere neuLropenla may be accompanled by deep and palnful
mouLh ulcers glnglvlLls sLomaLlLls and cellullLls
e|| Def|n|enn|es
@wo Lypes
1 Lack of dlfferenLlaLlon of 8 cells precursosrs lnLo maLure 8
cells
As a resulL plasma cells are lacklng and Lhe germlnal cenLers from all
lymphaLlc Llssues dlsappears leadlng Lo a compleLe lack of anLlbody
producLlon agalnsL lnvadlng bacLerla vlruses and oLher paLhogens
Lx sexllnked aggamaglobullnemla (8ruLon's dlsease)
2 Lack of dlfferenLlaLlon of 8 cells lnLo plasma cells
amp|es
Pypogammaglobullnemla also called common varlable
lmmunodeflclency (Cvlu) lgA are lacklng
anhypoglobullnemla general lack of lmmunoglobulln
@ e|| Def|n|enn|es
ccurs when when Lhe Lhymus falls Lo develop normally durlng
embryogenesls
8ecause @ cells play a regulaLory role ln lmmune funcLlon Lhe loss
of @ cell funcLlon ls usually accompanled by some loss of 8 cell
acLlvlLy
D| George Syndrome or Lhymlc hypoplasla
ls one example of @ cell lmmunodeflclency lL ls a rare congenlLal dlsease
resulLs from absence of several genes on chromosomes 22
Pave hypoparaLhyroldlsm wlLh resulLanL hypocalcemla congenlLal
hearL dlsease faclal feaLures and renal anormallLles
@hese lnfanLs are suscepLlble Lo yeasL fungal proLozoan nad vlral
lnfecLlons parLlcularly chlldhood dlsease llke chlckenpox measles and
rubella)
D omb|ned e|| and @ e|| Def|n|enn|es
amp|es
ta|a te|ang|entas|a auLosomal recesslve dlsorder affecLlng boLh @ and 8
cell lmmunlLy ln 40 of paLlenLs wlLh Lhls dlsease a selecLlve lgA
deflclency exlsLs Along wlLh lgC and lgL defleclencles
Severe nomb|ned |mmunodef|ne|enny (SID) wlde varleLy of congenlLal
and heredlLary lmmunologlc defecLs LhaL are characLerlzed by early onseL
of lnfecLlons defecLs ln boLh 8 and @ cell sysLem
J|sott|dr|nh syndrome has a varlaLlon of LhrombocyLopenla
Def|n|enn|es of the omp|ement System
amp|e
ng|oneurot|n edema ls cause by an lnherlLed deflclency of Lhe
lnhlblLor of C1 esLerase whlch opposes Lhe release of lnflammaLory
medlaLors A deflclency of Lhls lnhlblLor resulLs ln frequenL eplsodes of
urLlcarla and edema ln varlous parLs of Lhe body
SECDN0APY
IhhUND0EFICIENCY
nIV I-@IC- and IDS
ls Lhe mosL common secondary lmmunodeflclency
ls LransmlLLed ln body flulds conLalnlng Plv or lnfecLed Cu4 + @
lymphocyLes @hese flulds lnclude blood semlnal fluld vaglnal
secreLlons amnloLlc fluld and breasL mllk
NTFD0UCTDN
LLlologlc agenL of Acqulred lmmunodeflclency
yndrome (Alu)
ulscovered lndependenLly by Luc MonLagnler of
lrance and 8oberL Callo of Lhe u ln 198384
lormer names of Lhe vlrus lnclude
Human T cell lymphotrophic virus (HTLV-)
Lymphadenopathy associated virus (LAV)
ADS associated retrovirus (ARV)
Plv2 dlscovered ln 1986 anLlgenlcally
dlsLlncL vlrus endemlc ln WesL Afrlca
ne mllllon people lnfecLed ln u 30
mllllon worldwlde are lnfecLed
Leadlng cause of deaLh of men aged
2344 and 4Lh leadlng cause of deaLh
of women ln Lhls age group ln Lhe u
hLLp//wwwcnncom/2003/PLAL@P/condlLlons/11/17/black
shlvap/
MeLhods of Lransmlsslon
Sexual transmission
Exposure to infected blood or blood products.
Sharing contaminated needles (V drug
users).
Transplantation of infected tissues or organs.
Mother to fetus, perinatal transmission
variable, dependent on viral load and
mother's CD 4 count.
CHAFACTEFSTCS DF
H7
Plv belongs Lo a group of reLrovlrus
@hese reLrovlruses carry Lhelr geneLlc
maLerlal ln Lhe form on 8lbonuclelc Acld
(8nA) raLher Lhan ueoxyrlbonuclelc Acld
(unA)
ConslsLs of vlral core conLalnlng Lhe vlral
8nA" surrounded by an envelope
conslsLlng of proLrudlng glycoproLelns (C)
@he membrane of Lhe vlral envelope musL
be fused wlLh Lhe plasma membrane of a
cell a process medlaLed by Lhe envelope
C of Plv
LnLry of Plv Lo a person's body
nce lnslde Lhe hosL Plv aLLaches Lo Lhe LargeL cell membrane by way of lLs
recepLor molecule Cu4+
vlral 8nA ls Lranscrlbed lnLo unA
@he vlrus ls uncoaLed and Lhe 8nA enLers Lhe cell
8everse LranscrlpLase ls released
FAIHOFHYSIOLOGY
newly creaLed unA moves lnLo Lhe nucleus
and Lhe unA of Lhe cell
rovlrus ls creaLed
@he hosL cell dles
Plv lnfecLs oLher cells
vlral buddlng
Plv LargeLs Cu4+ @ helper cells
uecreased ln Cu4+ @ Cell counL
8ody loses lLs ablllLy Lo malnLaln a consLanL sLaLe
of healLh
CaLegory A
CaLegory 1 CreaLer Lhan
or equal Lo 300 Cu4+
cells
CaLegory 2 200 499
Cu4+ cells
CaLegory 3 less Lhan
200 Cu4+ cells
CaLegory 8 CaLegory C
ueaLh
Alu
CATECDFY A:
AsympLomaLlc Plv lnfecLlon
erslsLenL generallzed
lymphadenopaLhy
rlmary Plv lnfecLlon
CATECDFY 8:
@he condlLlons are aLLrlbuLed Lo Plv lnfecLlon or
are lndlcaLlve of a defecL ln cell medlaLed
lmmunlLy
@he condlLlons are consldered by physlclans Lo
have a cllnlcal course or managemenL LhaL ls
compllcaLed by Plv lnfecLlon
..CDNT
8acLerlal LndocardlLls menlnglLls
pneumonla or sepsls
Candldlasls vulvovaglnal
Cervlcal uysplasla severe or
carclnoma
lever or dlarrhea lasLlng more Lhan 1
monLh
Palry leukoplakla oral
Perpes zosLer
ldlopaLhlc LhrombocyLopenlc purpura
LlsLerlosls
erlpheral neuropaLhy
CATECDFY C:
Candldlasls of bronchl Lrachea lungs esophagus
Cervlcal cancer
CyLomegalovlrus dlsease
LncephalopaLhy
Perpes slmplex
PlsLoplasmosls
apos|'s Sarnoma
Mynobanter|um tubrenu|os|s
neumonyst|s nar|n|| pneumon|a
almonella sepLlcemla
WasLlng syndrome
ln 1990 Lhe World PealLh rganlzaLlon (WP)
grouped Lhese lnfecLlons and condlLlons
LogeLher by lnLroduclng a sLaglng sysLem for
paLlenLs lnfecLed wlLh Plv1 MosL of Lhese
condlLlons are opporLunlsLlc lnfecLlons LhaL are
easlly LreaLable ln healLhy people
Lage l Plv lnfecLlon ls asympLomaLlc and noL
caLegorlzed as Alu
Lage ll lncludes mlnor mucocuLaneous manlfesLaLlons
and recurrenL upper resplraLory LracL lnfecLlons
Lage lll lncludes unexplalned chronlc dlarrhea for longer
Lhan a monLh severe bacLerlal lnfecLlons
and pulmonary Luberculosls
Lage lv lncludes Loxoplasmosls of
Lhe braln candldlasls of
Lhe esophagus Lrachea bronchl or lungs and kaposls
sarcoma Lhese dlseases are lndlcaLors of Alu
PPDCPESSIDN DF HIV (4
STACES)
DPPDFTUNSTC
NFECTDNS
MosL of Lhe paLhogens responslble for ls are ublqulLous ln
naLure LhaL ls Lhey are all around us
Lg neumocysLls carlnll ls an organlsm LhaL ls ln Lhe alr we
breaLhe @he reason mosL people do noL geL slck from Lhls
organlsm ls LhaL Lhelr lmmune sysLems are lnLacL
PNEU|DCYSTS CAFN
PNEU|DNA
neumocysLls carlnll ls an organlsm LhaL ls alrborne and can be
found ln Lhe lungs of humans and anlmals
8eacLlvaLlon
Coughlng ls a frequenL flrsL sympLom whlch beglns as
nonproducLlve progresslng Lo producLlve cough
lever dyspnea on exerLlon dyspnea aL resL
aLlenLs wlLh Plv lnfecLlon have subsLanLlally lncreased
lncldence of several mallgnanL cancers @hls ls prlmarlly due
Lo colnfecLlon wlLh an oncogenlc unA vlrus
especlally LpsLeln8arr vlrus (L8v) kaposls sarcoma
assoclaLed herpesvlrus (kPv) and human paplllomavlrus
kaposls sarcoma (k) ls Lhe mosL common Lumor ln Plv
lnfecLed paLlenLs @he appearance of Lhls Lumor ln young
homosexual men ln 1981 was one of Lhe flrsL slgnals of Lhe
Alu epldemlc Caused by a gammaherpes vlrus
called kaposls sarcomaassoclaLed herpes vlrus (kPv) lL
ofLen appears as purpllsh nodules on Lhe skln buL can affecL
oLher organs especlally Lhe mouLh gasLrolnLesLlnal LracL
and lungs
kaposl's sarcoma (k)
kaposl's sarcoma (shown) ls
a rare cancer of Lhe blood
vessels LhaL ls assoclaLed
wlLh Plv lL manlfesLs as
blulshred ovalshaped
paLches LhaL may evenLually
become Lhlckened Leslons
may appear slngly or ln
clusLers
LA8DFATDFY
E7ALUATDN
CompleLe 8lood CounL
necessary Lo ldenLlfy anemla LhrombocyLopenla leukopenla and developlng
lnfecLlons
@uberculln kln @esL Cx8
ldenLlfles pulmonary problems
lor women ap mear
creenlng for syphllls gonorrhea chlamydla
Cu4+ cell counLs raLlos percenLages
@o deLermlne Lhe degree of lmmunodeflclency
vlral load LesLlng
@o calculaLe Lhe amounL of vlral acLlvlLy
LLlA
Lnzyme llnked lmmunosorbenL assay
ueslgned Lo screen blood or plasma for Lhe presence of anLlbodles of human
@lymphocyLe vlrus
WesLern 8loL @esL
ConflrmaLory LesL for Alu
NUFSNC |ANACE|ENT
PealLh educaLlon @he healLhcare worker musL
know Lhe paLlenL
Avold [udgmenLal and morallsLlc messages
8e conslsLenL and conclse
use poslLlve sLaLemenL
Clve pracLlcal advlce
racLlce unlversal/sLandard precauLlon
@here ls a need for a Lhorough medlcal handwashlng
afLer every conLacL wlLh paLlenL and afLer removlng
Lhe gown and gloves and before leavlng Lhe room of
anAlu suspecL or known Alu paLlenL
use of unlversal barrler or ersonal roLecLlve
LqulpmenL (L) eg cap mask gloves Cu gown
face shleld/goggles are very necessary
revent|on
Care should be Laken Lo avold accldenLal prlcks
from sharp lnsLrumenLs conLamlnaLed wlLh
poLenLlally lnfecLlous maLerlals form Alu paLlenL
Cloves should be worn when handllng blood
speclmens and oLher body secreLlons as well as
surfaces maLerlals and ob[ecLs exposed Lo Lhem
8lood and oLher speclmens should be labeled wlLh
speclal warnlng Alu recauLlon"
8lood spllls should be cleaned lmmedlaLely uslng
common household dlslnfecLanLs llke chlorox"
needles should noL be benL afLer use buL should be
dlsposed lnLo a puncLurereslsLanL conLalner
ersonal arLlcles llke razor or razor blades
LooLhbrush should noL be shared wlLh oLher
members of Lhe famlly 8azor blades may be
dlsposed ln Lhe same manner as needles are
dlsposed
aLlenLs wlLh acLlve Alu should be lsolaLed
|ANACE|ENT DF
DPPDFTUNSTC
NFECTDN
Larly ldenLlflcaLlon
AssessmenL for presence of slgns and
sympLoms of dlsorders
cllenL educaLlon ln slgns and sympLoms Lo
seek consulLaLlon wlLh Lhelr healLh care
Leam
ApproprlaLe use of prophylaxls
ConsulLaLlon wlLh approprlaLe proLocols and
Lhe healLh care Leam Lo conLlnue versus
LermlnaLe prophylaxls Lherapy
LvaluaLlon of LreaLmenL reglmens
AssessmenL of cllenL response and Lolerance
of reglmens Lo manage opporLunlsLlc
lnfecLlons
TFEAT|ENT
no cure
LffecLlve complex expenslve drug
LreaLmenLs are avallable
MalnLenance of healLhy llfesLyle
lmproves quallLy of llfe
sychologlcal famlly plannlng
economlc and soclal counsellng are very
lmporLanL
@he our s |n the Management of
nIV]IDS
1 omp||anne glvlng of lnformaLlon and counsellng Lhe
cllenL whlch resulLs Lo Lhe cllenL's successful LreaLmenL
prevenLlon and recommendaLlon
2 ounse||ng]edunat|on
a Clvlng lnsLrucLlon abouL Lhe LreaLmenL
b ulssemlnaLlng lnformaLlon abouL Lhe dlsease
c rovldlng guldance on how Lo avold conLracLlng @u agaln
d harlng facLs abouL Plv and Alu
3 ontant tran|ng
a @raclng ouL and provldlng LreaLmenL or parLners
4 ondoms
a romoLlng Lhe use of condom glvlng lnsLrucLlons
abouL lLs use and glvlng away avallable condoms
GI @IC- hys|o|og|n Cverv|e
An allerglc reacLlon ls a manlfesLaLlon of Llssue ln[ury resulLlng
from lnLeracLlon beLween an anLlgen and an anLlbody ||ergy ls an
lnapproprlaLe and ofLen harmful response of Lhe lmmune sysLem Lo
normally harmless subsLances ln Lhls case Lhe subsLance ls Lermed
an allergen topy refers Lo allerglc reacLlons characLerlzed by Lhe
acLlon of lgL anLlbodles and a geneLlc predlsposlLlon Lo allerglc
reacLlons
When Lhe body ls lnvaded by an ant|gen usually a proLeln LhaL Lhe
body's defense recognlzes as forelgn a serles of evenLs occurs ln an
aLLempL Lo render Lhe lnvader harmless desLroy lL and remove lL
from Lhe body When lymphocyLes respond Lo Lhe anLlgen
ant|bod|es (proLeln subsLance LhaL proLecL agalnsL anLlgen) are
produced
AnLlbodles reacL wlLh anLlgens ln a varleLy of ways
8y coaLlng Lhe anLlgens surfaces lf Lhey are parLlcular subsLances
8y neuLrallzlng anLlgens lf Lhey are Loxlc
8y preclplLaLlng Lhe anLlgens ouL of a soluLlon lf Lhey are dlssolved
unnt|on and rodunt|on of Immunog|obu||ns
Crouped lnLo flve classes (lgL lgu lgC lgM and lgA) anLlbodles can be
found ln Lhe lymph nodes Lonslls appendlx and eyer's paLches of Lhe
lnLesLlnal LracL or clrculaLlng ln Lhe blood and lymph
Lach anLlbody molecule ls composed of Lwo ldenLlcal heavy (P) chalns and
Lwo ldenLlcal llghL (L) chalns Lach chaln conLalns one varlable reglon and
one or more consLanL reglons
@he consLanL reglons deLermlne Lhe class (lgL lgu) of each anLlbody and
allow each class of anLlbody Lo lnLeracL wlLh speclflc effecLor cells and
molecules @he varlable reglons conLaln anLlgen blndlng slLes
lmmunoglobullns of Lhe lgL class are lnvolved ln allerglc dlsorders @wo or more
lgL molecules blnd LogeLher Lo an allergen and Lrlgger masL cells or basophlls Lo
release chemlcal medlaLors whlch produces allerglc skln reacLlons
AnLlbodles comblne wlLh anLlgens ln a speclal way llkened Lo keys flLLlng lnLo a
lock AnLlgens (keys) only flL cerLaln anLlbodles (locks) Pence Lhe Lerm
speclflclLy refers Lo Lhe speclflc reacLlon of an anLlbody Lo an anLlgen @he
sLrengLh wlLh whlch one anLlgen blndlng surface of an anLlbody blnds Lo one
ep|tope an lmmunologlcally acLlve slLe on an anLlgen ls known as Lhe afflnlLy of
lnLeracLlon
AnLlbody molecules are blvalenL LhaL ls Lhey have Lwo comblnlng slLes
@herefore Lhe anLlbody easlly becomes a crossllnk beLween Lwo anLlgen
groups causlng Lhem Lo clump LogeLher (aggluLlnaLlon) 8y Lhls acLlon forelgn
lnvaders are cleared from Lhe bloodsLream
o|e of e||s
ls programmed Lo produce one speclflc anLlbody
on encounLerlng a speclflc anLlgen 8 cell sLlmulaLes producLlon of plasma
cells Lhe slLe of anLlbody producLlon
o|e of @ ne||s
asslsL 8 cells ln produclng anLlbodles @ cells secreLe subsLance known as
lymphoklnes LhaL encourage cell growLh promoLe cell acLlvaLlon dlrecL Lhe
flow of cell acLlvlLy desLroy LargeL cells and sLlmulaLe Lhe macrophages
Macrophages presenL Lhe anLlgen Lo Lhe @ cells and lnlLlaLe Lhe lmmune
response @hey also dlgesL anLlgens and asslsL ln removlng cells and oLher
debrls
unnt|on of nt|gens
AnLlgens are dlvlde lnLo Lwo groups
CompleLe proLeln anLlgens (ex anlmal dander pollen horse serum)
sLlmulaLe a compleLe humoral response
Low molecular welghLs subsLances (ex medlcaLlons) funcLlons as
hapLens (lncompleLe anLlgens) blndlng Lo Llssue or serum proLelns Lo
produce a carrler complex LhaL lnlLlaLes an anLlbody response
unnt|on of hem|na| Med|ators
MasL cells whlch have a ma[or role ln lgL medlaLed lmmedlaLe
hypersenslLlvlLy are locaLed ln Lhe skln and mucous membranes
When masL cells are sLlmulaLed by anLlgens powerful chemlcal
medlaLors are released LhaL cause a sequence of physlologlc evenLs
resulLlng ln sympLoms of lmmedlaLe hypersenslLlvlLy
@o types of nhem|na| med|ators
a r|mary med|ators
n|stam|ne ls released ln masL cell granules where lL ls sLored
Maxlmum lnLenslLy ls reached wlLhln abouL 13 mlnuLes afLer anLlgen
conLacL @he effecLs of hlsLamlne release lnclude eryLhema locallzed
edema prurlLus conLracLlon of bronchlal smooLh muscle resulLlng ln
wheezlng dllaLlon of small venule and consLrlcLlon of large vessels
lncreased secreLlon of gasLrlc and mucosal cells resulLlng ln dlarrhea
n2 reneptors found on gasLrlc parleLal cells n1 reneptors are found
predomlnanLly ln bronchlal and vascular smooLh muscle cells
os|noph|| hemotant|n antor of naphy|a|s affecLs movemenL
of eoslnophlls Lo Lhe slLe of allergen ls released Lo lnhlblL Lhe acLlon
of hlsLamlne
|ate|et nt|vat|ng antor ls responslble for lnlLlaLlng plaLeleL
aggregaLlon aL slLes of lmmedlaLe hypersenslLlvlLy reacLlon lL cause
bronchoconsLrlcLlon and lncreased vascular permeablllLy
rostag|and|ns composed of unsaLuraLed faLLy aclds produce
smooLh muscle conLracLlon as well as vasodllaLlon and lncreased
caplllary permeablllLy @hus resulL wlLh fever and paln
b Senondary Med|ators
euotr|enes Lhey collecLlvely make up whaL was once Lermed
slow reacLlng subsLance of anaphylaxls" (8A) lL can cause
smooLh muscle conLracLlon bronchlal consLrlcLlon mucus secreLlon
ln Lhe alrways and Lhe yplcal wheal and flare reacLlon of Lhe skln
Compared wlLh hlsLamlne leukoLrlenes are 100 Lo 1000 Llmes more
poLenL ln causlng bronchospasm
rady|n|n ls a polypepLlde wlLh Lhe ablllLy Lo cause lncreased
vascular permeablllLy LhaL resulLs ln edema vasodllaLlon
hypoLenslon and conLracLlon of many Lypes of smooLh muscle such
as Lhe bronchl
Seroton|n ls released durlng plaLeleL aggregaLlon acLlng as a poLenL
vasoconsLrlcLor
HYPEPSENSITIVITY:
ALLEPCIC 0ISDP0EPS
@ype I
n anaphy|ant|n reant|on |s nharanter|zed by vasod||at|on |nnreased nap|||ary
permeab|||ty smooth musn|e nontrant|on and eos|noph|||a System|n reant|ons may
|nvo|ve |aryngea| str|dor ang|oedema hypotens|on and bronnh|a| GI or uter|ne
spasm |ona| reant|ons are nharanter|zed by h|ves amp|es of type I reant|ons |nn|ude
etr|ns|n asthma a||erg|n rh|n|t|s system|n anaphy|a|s and reant|ons to |nsent st|ngs
@ype II
s|de to to|n reant|on h|nh |nvo|ves the b|nd|ng of e|ther the |gG or |gM ant|body to
a ne|| bound ant|gen may |ead to eventua| ne|| and t|ssue damage @he reant|on |s the
resu|t of m|staen |dent|ty hen the system |dent|f|es a norma| nonst|tuent of the
body as fore|gn and ant|vates the nomp|ement nasnade amp|es of type to
reant|ons are myasthen|a grav|s Goodpasture's syndrome pern|n|ous anem|a
haemo|yt|n d|sease of the neborn transfus|on reant|on and thrombonytopen|a
@ype III
n |mmune nomp|e reant|on |s mared by anute |nf|ammat|on resu|t|ng from
format|on and depos|t|on of |mmune nomp|ees @he [o|nts and |dneys are
part|nu|ar|y susnept|b|e to th|s |nd of reant|on h|nh |s asson|ated |th
system|n |upus and erythematosus serum s|nness nephr|t|s and rheumato|d
arthr|t|s Some s|gns and symptoms |nn|ude urt|nar|a [o|nt pa|n fever rash
and adenopathy (so||en g|ands)
@ype IV
de|ayed or ne||u|ar reant|on onnurs 1 to 3 days after eposure to an ant|gen
@he reant|on h|nh resu|ts |n t|ssue damage |nvo|ves ant|v|ty ||e
|ympho|nes manrophages and |ysozymes rythema and |tnh|ng are
nommon a fe eamp|es |nn|ude nontant dermat|t|s graftversushost
d|sease nash|moto's thyro|d|t|s and sarno|dos|s
0IACNDSTIC EVALUATIDN
omp|ete |ood ount |th D|fferent|a|
os|noph|| ount
@ota| Srerum Immunog|obu||n eve|s
S|n @ests
rovonat|ve @est|ng
ad|oa||ergosorbent @est
hE0ICAL hANACEhENT
vo|danne @herapy
harmano|og|n @herapy
nt|h|stam|nes
drenerg|n gents
Mast e|| Stab|||zers
ort|nostero|ds
Immunotherapy
ommon|y na||ed f(|nf|ammat|on of a
[o|nt) and thought of as one nond|t|on
More than 100 d|fferent types of d|sorders that
pr|mar||y affents se|eta| musn|es bones
nart||age ||gaments tendons and [o|nts of
ma|es and fema|es of a|| ages
heumat|n D|seases
group of d|sorders that are nhron|n |n nature and nharanter|zed by
d|ffuse |nf|ammat|on and degenerat|on |n the nonnent|ve t|ssues
@hey are thought to be the resu|t of |mmuno|og|n abnorma||t|es
D|ffuse nonnent|ve @|ssue D|seases |nn|udes
4
4 S
4 Sn|eroderma
4 o|ymyos|t|s
4 o|ymya|g|a rheumat|na
DIDS C--@IV @ISSD DISSS
resenLaLlon of
anLlgen Lo @ cells
resenLaLlon of
anLlgen Lo @ cells
@ and 8Cell
prollferaLlon
Anglogenesls
ln synovlal
llnlng
@ and 8Cell
prollferaLlon
Anglogenesls
ln synovlal
llnlng
wollen ln small [olnLs assoclaLed
wlLh paln sLlffness and faLlgue
wollen ln small [olnLs assoclaLed
wlLh paln sLlffness and faLlgue
neuLrophll accumulaLlon ln
synovlal fluld Cell prollferaLlon
no carLllage lnvaslon
neuLrophll accumulaLlon ln
synovlal fluld Cell prollferaLlon
no carLllage lnvaslon
Warm swollen effuslons paln and
decreased moLlon wlLh posslble
rheumaLold nodules
Warm swollen effuslons paln and
decreased moLlon wlLh posslble
rheumaLold nodules
ynovlLls Larly pannus
lnvaslon ChondrocyLes
acLlvaLlon uegradaLlon
of carLllage by
proLelnase
ynovlLls Larly pannus
lnvaslon ChondrocyLes
acLlvaLlon uegradaLlon
of carLllage by
proLelnase
lncrease ln severlLy of physlcal slgns
and sympLoms
lncrease ln severlLy of physlcal slgns
and sympLoms
ubchondral bone eroslon
annus lnvaslon of carLllage
ChondrocyLe prollferaLlon
LaxlLy of llgamenLs
ubchondral bone eroslon
annus lnvaslon of carLllage
ChondrocyLe prollferaLlon
LaxlLy of llgamenLs
!olnL lnsLablllLy conLracLures
decreased 8M sysLemlc
compllcaLlons
!olnL lnsLablllLy conLracLures
decreased 8M sysLemlc
compllcaLlons
-heumato|drthr|t|s
athophys|o|ogy
-System|n upus rythematosus
resu|t of d|sturbed |mmune regu|at|on that nauses an
eaggerated produnt|on of ant|bod|es @h|s |mmuneregu|atory
d|sturbanne |s brought about by some nomb|nat|on of genet|n hormona|
(as ev|denned by usua| onset dur|ng the nh||dbear|ng years) and
env|ronmenta| fantors (sun||ght therma| burns)
WSn|eroderma ("hard s|n")
e|at|ve|y rare d|sease that |s poor|y understood
@he nause |s unnon
Its |nn|denne |s 18 to 20 persons per m||||on per year
-o|ymyos|t|s
Group of d|seases termed |d|opath|n |nf|ammatory myopath|es
-o|ymya|g|a heumat|na
Degenerat|ve o|nt D|sease
-Csteoarthr|t|s
@he most nommon and freuent|y d|sab||ng of the [o|nt d|sorders
oth over d|agnosed and tr|v|a||zed
|t |s freuent|y over treated or undertreated
-|ass|f|ed as
r|mary (|d|opath|n) |th no pr|or event or d|sease re|ated to the
osteoarthr|t|s
Senondary resu|t|ng from prev|ous [o|nt |n[ury or |nf|ammatory
d|sease
-Spondy|oarthropath|es
It |nn|udes any|os|ng spondy||t|s reant|ve arthr|t|s (e|ter's
syndrome) and psor|at|n arthr|t|s
It |s asson|ated |th |nf|ammatory boe| d|seases sunh as reg|ona|
enter|t|s (nhron's d|sease) and u|nerat|ve no||t|s
-ny|os|ng Spondy||t|s
ffents the nart||ag|nous [o|nts of the sp|ne and surround|ng t|ssues
D|agnosed |n the senond or th|rd denade of ||fe
eant|ve rthr|t|s (e|ter's Syndrome)
@h|s d|sease proness |s na||ed reant|ve benause the arthr|t|s onnurs
fo||o|ng an |nfent|on
ffents young adu|t ma|es and |s nharanter|zed pr|mar||y by urethr|t|s
arthr|t|s and non[unnt|v|t|s Dermat|t|s and u|nerat|ons of the mouth and
pen|s may a|so be present o ban pa|n |s nommon
-sor|at|n rthr|t|s
haranter|zed by synov|t|s po|yarthr|t|s and spondy||t|s
Metabo||n and ndonr|ne D|seases sson|ated |th heumat|n
D|sorders
1 Gout
neterogenous group of nond|t|ons re|ated to a genet|n defent of
pur|ne metabo||sm resu|t|ng |n hyperur|nem|a
2 |bromya|g|a
ommon syndrome that |nvo|ves nhron|n fat|gue genera||zed
musn|e anh|ng and st|ffness
ourna|
Sept|n shon M|ne sho
ay to a ne treatment
@PAnk ?u Anu Cu 8LL