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Immunodefsiency &
Autoimmune
dr. Isbandiyah, SpPD
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Introduction
Immunodefciency disorders occurwhen the body's immune responseisreducedor absent
T or B cell lymphocytes or both! donot wor" as well as they should, orwhen your body doesn't produce
enou#h antibodies.
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$lassifcation
Primary
secundary
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Primaryimmunodefciency (PID)
%any o these disorders are hereditaryand are autosomal recessieor ()lin"ed.
*+ recurrent or persistent inection
-ther si#ns include Poor response to treatment
Delayed or incomplete recoery rom illness
$ertain types o cancers such as
aposi's sarcomaor non)/od#"in's lymphoma! $ertain inections includin# some orms o
pneumonia or recurrent un#al yeastinections!
http://en.wikipedia.org/wiki/Genetic_disorderhttp://en.wikipedia.org/wiki/Autosomal_recessivehttp://en.wikipedia.org/wiki/Sex_linkagehttp://en.wikipedia.org/wiki/Infectionhttp://www.nlm.nih.gov/medlineplus/ency/article/000661.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/000661.htmhttp://en.wikipedia.org/wiki/Infectionhttp://en.wikipedia.org/wiki/Sex_linkagehttp://en.wikipedia.org/wiki/Autosomal_recessivehttp://en.wikipedia.org/wiki/Genetic_disorder -
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Dia#nosis
ull blood countincludin# accuratelymphocyteand #ranulocytecounts!
immuno#lobulinleels the threemost important types o antibodiesI#*, I#Aand I#%!.
$omplement leels
http://en.wikipedia.org/wiki/Full_blood_counthttp://en.wikipedia.org/wiki/Lymphocytehttp://en.wikipedia.org/wiki/Granulocytehttp://en.wikipedia.org/wiki/Immunoglobulinhttp://en.wikipedia.org/wiki/Immunoglobulin_Ghttp://en.wikipedia.org/wiki/Immunoglobulin_Ahttp://en.wikipedia.org/wiki/Immunoglobulin_Mhttp://en.wikipedia.org/wiki/Immunoglobulin_Mhttp://en.wikipedia.org/wiki/Immunoglobulin_Ahttp://en.wikipedia.org/wiki/Immunoglobulin_Ghttp://en.wikipedia.org/wiki/Immunoglobulinhttp://en.wikipedia.org/wiki/Granulocytehttp://en.wikipedia.org/wiki/Lymphocytehttp://en.wikipedia.org/wiki/Full_blood_count -
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eInternational 0nion o Immunolo#ical Societies
reco#nises ei#ht classes o primaryimmunodefciencies
$ombined T) and B)cell immunodefciencies
Antibody defciencies
1ell)defned syndromes Immune dysre#ulation diseases
Pha#ocyte disorders
Innate immunity defciencies Autoin2ammatory disorders
$omplement defciencies
http://en.wikipedia.org/wiki/International_Union_of_Immunological_Societieshttp://en.wikipedia.org/wiki/International_Union_of_Immunological_Societieshttp://en.wikipedia.org/wiki/International_Union_of_Immunological_Societieshttp://en.wikipedia.org/wiki/International_Union_of_Immunological_Societies -
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Acquired immunodefciency5secondary5 or 5ac6uired5
immunodefciency $ommon causes or secondary
immunodefciency are malnutrition, a#in#and particular medications e.#.chemotherapy,
disease)modiyin# antirheumatic dru#s,immunosuppressie dru#s, ater or#antransplants, #lucocorticoids!.
cancer, particularly those o the bonemarrow and blood cells leu"emia,lymphoma, multiple myeloma!, and certainchronic inections. Immunodefciency is alsothe hallmar" o ac6uired immunodefciencysyndrome AIDS!
http://en.wikipedia.org/wiki/Malnutritionhttp://en.wikipedia.org/wiki/Aginghttp://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Disease-modifying_antirheumatic_drughttp://en.wikipedia.org/wiki/Disease-modifying_antirheumatic_drughttp://en.wikipedia.org/wiki/Chemotherapyhttp://en.wikipedia.org/wiki/Aginghttp://en.wikipedia.org/wiki/Malnutrition -
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Autoimmune
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Autoimmune DiseasesAutoimmune Diseases
Loss o sel-tolerance leads toproduction o antibodies or T cellsthat react against ones o!nantigens"
Immune system response to selantigens causes damage toorgans"
The ma7or actors that contribute tothe deelopment o autoimmunity are#enetic susceptibility and
enironmental tri##ers
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Loss of Tolerance in Autoimmune Disease
Susceptibility genes
(usually multiple)
Triggering factors (probably
environmental)
Loss of tolerance
Auto reactive T cells Auto reactive B cells Inadequate regulatory
mechanism
ersistent pathogenic auto antibodies
ersistent pathogenic immune comple!es
ersistent damaging auto reactive T cell
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$lassifcation
Autoimmune diseases may be eithersystemic or or#an specifc
Three types o autoimmunedisorders#
$ytoto%ic (Type II reactions)
Immune comple% (Type IIIreactions)
$ell-mediated (Type I& reactions)
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Type II antibody against cell-surace ormatri% antigens ( syndrome' autoantigen
and consequence )
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Type III immune comple% disease( syndrome' autoantigen and consequence)
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Type I& cell-mediated disease
(syndrome' autoantigen andconsequence)
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Autoantibodies against cell-suracereceptors