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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