Autoimmunity Flashcards by Robert Ellis

Effector machanisms of autoimmunity

autoantibodies

2. autoimmune T cells

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autoimmune diseases arise via

the breakdown of the negative selection processes that remove self reactive t cells and b cells from the lymphocyte reservoire

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Autoimmune responses are NEVER mediated by

IgE

Therefore there are no Immediate Type I autoimmunties

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Caused by ab’s specific for components of cell surfaces pr ECM

Type II autoimmune disease

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Autoimmune hemolytic anemia class

Type II

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Autoimmune hemolytic anemia

IgG and IgM bind to RBC’s

->activate classical compliment pathway
->MAC destroys RBCS
->C3b deposition also leads to phagocyte clearance in the SPLEEN
RESULT IS ANEMA

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test for autoimmune hemolytic anemia

DIRECT coomb’s

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are nucleated cells efficiently cleared through compliment activation

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Neutropenia autoimmune class

Type II

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Nutropenia MOA

WBC’s destroyed via most via phagocytosis

*ab responses directed at WBC surface antigens–> FC receptors recognize Ab’s

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Common tx. for neutropenia

splenectomy

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Goodpasture’s syndrome is what Autoimmune class

Type II

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Goodpasture’s Syndrome MOA

Ab’s specific for ECM autoantigens

specifically alpha chain of Type Iv collagen (basement membranes)
->ab’s deposit on basement membranes of renal glomeruli and tubules eliciting inflammation
->kidney failure

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tx. for Good pastures

removal of self-reactive ab’s and immunosuppression

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255 of all kidney failure are related to

immune system

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Acute rheumatic fever class

type II autoimmunity

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Acute Rheumatic Fever MOA

(MOLECULAR MEMORY)
Ab’s produced in response to Strep Pyogenes (initially for protecton)–> react with self antigens on human heart (become autoantigens)–> inflammation and damage

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Is Rheumastic fever transient or persistent

Transient–> t cells cannot recognize the strep surface antigens, B cells cannot be activate fully–> NO IMMUNOLOGIC MEMORY IS CREATED

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AUTOIMMUNE CLASS FOR GRAVE’S DISEASE

Type II

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Graves’ disease

Ab’s that bind to TSH receptors and mimic binding of TSH–> chronic overproduction of TH that is independent of regulation

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Graves’ disease results in

Hyperthyroidisms–> heat intolerance, nervouseness, irritability, warm moist skin

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Can TSH mimicking antibodies cross placenta

yes– baby can be born with Grave’s

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TSH mimicking antibody in Graves is of what isoform

IgG–> it can cross placenta

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tx. for adult graves

drugs

removal of thyroid

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tx for grave's infants

removal of anti-TSHR ab's via total exchange of blood plasma

Myasthenia gravis Classification

Type II

Myasthenia gravis MOA

signaling from nerve to muscle is impaired | --> Ab's bind to self-ACH recptors and cause them to be internalized--> muscle is then LESS responsive to ACH

Tx. of Myasthenia Gravis

Immunosuppressive agents, or PYROSTIGIMINE which inhibits acetylcholinesterase

Guillian Barre Class?

Type II

Autoimmune Deficiencies that classify as MOLECULAR MIMICRY

1. GBS 2. Acute Rheumatic fever 3. Wegener's Granulomatosis

GBS MOA

mediated by IgG specific for gangliosides--> mediate Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

GBS is most commonly caused by molecular mimickry from which organism

C. jejuni

GBS morphology

Descending--from legs to head (happens very quickly weeks to days)

Miller-Fischer Syndrome

GBS except it progresses from top to bottom

Wegener's is what type of Autoimmune disease

Type II

Wegener's is meadiated by which Ab's

cANCA's: anti-neutrophil cytoplasmic ab's

Wegener's MOA

cross reactive ANCA's(originally formed to bacterial pathogen response) bind to neutrophils--> actiavted them--> they bind to endothelial cells and cause VASCULITIS

Autoimmune Thrombocytopenic Purpura is in what Class

Type II

ITP cause

IgG mediated inhibition of an enezyme responsible for cleavage of VWF-- (ROBBIN;s has this as ADAMSt13 Leads to thrombocytopenia, neurological symptoms and microangiopathic hemolytic anema

Microangiopathic hemolytic anemia

rbc's get cleaved (schistocytes) as they are pushed through microscopic clots

Scleroderma (Type II) cause

destrcution of vascular endothelium and smooth muscle cells--. replacement with abundant collagen from fibroblasts

Scleroderma is associated with what Ab's

``` Anti topo 1 (Diffuse) anti centromere (CREST) ```

Findings of CREST Systemic Scleroderma

``` *Milder form calcinosis Raynaud's esophageal dysmotility Sclerodactyly Telangeictasia ```

Anti centromer Ab's are specific to

Limited Scleroderma: CREST

Anti Topo 1 ab's are specific to

diffuse scleroderma

Pemphigus Vulgaris (Type II autoimmunity)

autoimmune condition specific for 2 proteins Desmoglein 1 and 3 *cohesion of kratinoctyes--> painful and chronic blistering of the skin

tx for pemphisus bulgaris

rituximab

SLE is what type of autoimmune

III

Ab's present in SLE

ANA's (non diagnostic) Anti-histone (non diagnostic) Anti-SMith--> diagnostic Anti-dsDNA--> diagnostic

SLE pt.'s usually die from failure of what organs

KIDNEY | BRAIN

Post Strep Glomerulonephritis

immune compex disorder that follows infections with GAS--> Ab-GAS complexes get lodged in the glomeruli resulting in inflammation that hinders kidney fxn

Pt.s with Insulin Dependent TYPE 1 diabetes have what autoimmune process going on

T cell mediated autoimmune response that desstroys beta cells in islets of langerhans

IDDM is what class of Autoimmune disease

TYPE IV--> T CELL MEDIATED

CTL's in IDDM do what

bind to undefined antigen on Beta cells and kill them over time--> decreased insulin production

CD4 and Ab's cells in IDDM do what

involved in producing specific for products of B cells (anti-insulin Ab's and anti-glutamate dehydrogenase)

RA is what type of autoimmunity

Type IV

What is rehumatoid factor

IgM, IgA, IgG AUTOANTIBODIES that are specific to the FC recgions of human IgG molecules--> this si not required for tissue damage, but helps diagnose

Test more specific for RA

anti-CCP : cyclic citrullinated proteins

Cells found in infiltrates of all ppl with RA

CD4 CD8 T cells, B cells, Plasma cells, neutrophils, macrophages

Tx for RA

TNF-Alpha blockers

Multiple Sclerosis is what type

Cause of MS

immune response directed at myelin sheath | *causes sclerotic plaques of demyelinated tissue in white matter of CNS--> loss of coordination and spasticity

Cell type responsible for demyelination in MS

TH1 *produce IFN gamma that activated macrophages in sclerotic plaques--> mac's release proteases and cytokines--> demyelination

Tpe Iv autoimmune disease that attacks exocrine glands

Sjogren Syndrome

Sjogren's is usally found

in conjucntion with another Autoimmune condition

Ab's specific for Sjogren's

Anti-SS A/RO and Anti SS B/LA

APECED is caused by a defect in...

AIRE gene--> impaired negative selection of t cells in the thymus (therefore b cells are affected too)

PPL that express HLA-DR2 are

16 times more likely to develop goodpasture's syndrome than those that do NOT express HLA-DR2

Immunologically priveledges dites

Anterior chamber of the eye uterus testes

Sympathetic ophthalmia | an alteration on what is believed to be a immune preveledged site

is a bilateral diffuse granulomatous uveitis (a kind of inflammation) of both eyes following trauma to one eye. It can leave the patient completely blind. Symptoms may develop from days to several years after a penetrating eye injury.