AUTOIMMUNE AUTOIMMUNE DISORDERS &DISORDERS &

IMMUNE TOLERANCEIMMUNE TOLERANCE

AMR RATEB (MD)AMR RATEB (MD)

IMMUNE INTERACTIONIMMUNE INTERACTION

IMMUNE REACTION AND IMMUNE REACTION AND INFLAMMATIONINFLAMMATION

IMMUNE REACTIONSIMMUNE REACTIONS

TYPE I: IgE, ANAPHYLAXIS & ATOPY.TYPE I: IgE, ANAPHYLAXIS & ATOPY. TYPE II: IgG & IgM, CYTOTOXIC ANTIBODIES.TYPE II: IgG & IgM, CYTOTOXIC ANTIBODIES. TYPE III: IMMUNE COMPLEX, SERUM TYPE III: IMMUNE COMPLEX, SERUM

SICKNESS & ARTHUS REACTION.SICKNESS & ARTHUS REACTION. TYPE IV: DELAYED, T-CELL MEDIATED.TYPE IV: DELAYED, T-CELL MEDIATED. ? TYPE V: STIMULATING ANTIBODIES, LATS ? TYPE V: STIMULATING ANTIBODIES, LATS

& ANCA.& ANCA. ? TYPE VI: Ab- DEPENDENT CELL ? TYPE VI: Ab- DEPENDENT CELL

CYTOTOXICITY, K- CELLS.CYTOTOXICITY, K- CELLS.

IMMUNOCOMPETENT CELLS IMMUNOCOMPETENT CELLS OF THE SKINOF THE SKIN

T-CELLS.T-CELLS. B-CELLS.B-CELLS. LANGERHANS CELLS.LANGERHANS CELLS. NEUTROPHILS,NEUTROPHILS, MAST CELLS.MAST CELLS. EOSINOPHILS.EOSINOPHILS. KERATINOCYTES.KERATINOCYTES.

IMMUNOGLOBULINSIMMUNOGLOBULINS

IMMUNOGLOBULINSIMMUNOGLOBULINS

AUTOANTIBODIESAUTOANTIBODIES

MEDIATORS OF MEDIATORS OF INFLAMMATIONINFLAMMATION

MAST CELL, EOSINOPHIL& NEUTROPHIL MAST CELL, EOSINOPHIL& NEUTROPHIL MEDIATORS.MEDIATORS.

INTERLEUKINS.INTERLEUKINS.

LEUKOTRIENS & PROSTAGLANDINS.LEUKOTRIENS & PROSTAGLANDINS.

COMPLEMENT.COMPLEMENT.

COMPLEMENTCOMPLEMENT

COMPLEMENTCOMPLEMENT

COMPLEMENTCOMPLEMENT

IMMUNOGENETICSIMMUNOGENETICS

MAJOR HISTOCOMPATABILITY COMPLEX MAJOR HISTOCOMPATABILITY COMPLEX (MHC).(MHC).

HISTOCOMPATABILITY LOCUS A,HISTOCOMPATABILITY LOCUS A, HUMAN LEUCOCYTIC ANTIGEN,HUMAN LEUCOCYTIC ANTIGEN, HLA: HLA: - CLASS I : A,B & C- CLASS I : A,B & C…….ALL CELLS..ALL CELLS. - CLASS II: D- CLASS II: D…… IMMUNE- FUNCTIONING IMMUNE- FUNCTIONING CELLS. CELLS.

SELF vs FOREIGNSELF vs FOREIGN

INTRAUTERINE & EARLY INFANCY:INTRAUTERINE & EARLY INFANCY:

- Ag RECOGNITION & TEMPLATE FORMATION .- Ag RECOGNITION & TEMPLATE FORMATION .

- SUPPRESSION OF SELF REACTION (FORBIDDEN - SUPPRESSION OF SELF REACTION (FORBIDDEN CLONE).CLONE).

ANTIGENICITY:ANTIGENICITY:

- CHEMICAL COMPOSITION.- CHEMICAL COMPOSITION.

- SIZE.- SIZE.

- SITE.- SITE.

IMMUNE TOLERANCEIMMUNE TOLERANCE

HOMOESTASIS: HOMOESTASIS: STARTING INTRAUTERINE.STARTING INTRAUTERINE.

CAUSE OF DISEASE: CAUSE OF DISEASE: LEPROMATOUS LEPROSY.LEPROMATOUS LEPROSY.

CURE FROM DISEASE: CURE FROM DISEASE: DESENSITIZATION e.g ALLERGIES & DESENSITIZATION e.g ALLERGIES &

PLE. PLE.

INDUCTION OF TOLERANCEINDUCTION OF TOLERANCE

CONTINUOUS OR REPEATED EXPOSURE OF THE CONTINUOUS OR REPEATED EXPOSURE OF THE PROVACTIVE ANTIGEN IN SUBTHRESHOLD DOSES PROVACTIVE ANTIGEN IN SUBTHRESHOLD DOSES AND/OR VIA AN UNUSUAL ROUTE:AND/OR VIA AN UNUSUAL ROUTE:

- FORMATION OF NEUTRALIZING ANTIBODIES.- FORMATION OF NEUTRALIZING ANTIBODIES.

- DEPLETION OF EFFECTOR T-CELLS.- DEPLETION OF EFFECTOR T-CELLS.

- DEPLETION OF MEDIATORS OF INFLAMMATION.- DEPLETION OF MEDIATORS OF INFLAMMATION.

INDUCTION OF INDUCTION OF AUTOIMMUNITYAUTOIMMUNITY

RELEASE OF A FORBIDDEN CLONE OF RELEASE OF A FORBIDDEN CLONE OF T- EFFECTOR CELLS.T- EFFECTOR CELLS.

CHANGE OF A SELF PROTEIN: DRUG, CHANGE OF A SELF PROTEIN: DRUG, UVR, VIRAL UVR, VIRAL …….. OR ELSE!!!!.. OR ELSE!!!!

SELF PROTEIN OUTSIDE ITS NATURAL SELF PROTEIN OUTSIDE ITS NATURAL LOCATION: ANTI-NUCLEAR LOCATION: ANTI-NUCLEAR CYTOPLASMIC ANTIBODIES IN LE.CYTOPLASMIC ANTIBODIES IN LE.

PATHOGENEIS OF PATHOGENEIS OF AUTOIMMUNITYAUTOIMMUNITY

LACK OF T-REGULATORY CONTROL.LACK OF T-REGULATORY CONTROL.

OVERPRODUCTION OF AUTOANTIBODIES.OVERPRODUCTION OF AUTOANTIBODIES.

TYPES II, III & V IMMUNE REACTIONS.TYPES II, III & V IMMUNE REACTIONS.

PROPAGATION OF REACTION, SPECIFIC TO PROPAGATION OF REACTION, SPECIFIC TO EACH DISEASE.EACH DISEASE.

AUTOIMMUNE DISEASESAUTOIMMUNE DISEASES

AUTOIMMUNE BULLOUS DISORDERS.AUTOIMMUNE BULLOUS DISORDERS. AUTOIMMUNE COLLAGEN DISORDERS.AUTOIMMUNE COLLAGEN DISORDERS. VITILIGO.VITILIGO. VASCULITIS.VASCULITIS. AUTOIMMUNE URTICARIA.AUTOIMMUNE URTICARIA. OTHERS.OTHERS.

PEMPHIGUSPEMPHIGUS

PEMPHIGOIDSPEMPHIGOIDS

EB ACQUISITAEB ACQUISITA

LUPUS ERYTHEMATOSUSLUPUS ERYTHEMATOSUSAETIOPATHOGENESISAETIOPATHOGENESIS

Altered immune responseAltered immune response::

- - reduced T-suppressor functionreduced T-suppressor function..

- - overproduction of Igsoverproduction of Igs..

- - reduced clearance of immune complexesreduced clearance of immune complexes..

- - complement mediated damagecomplement mediated damage..

- - complement deficiency ( C2 – C4 )complement deficiency ( C2 – C4 )..

LUPUS ERYTHEMATOSUSLUPUS ERYTHEMATOSUS

SCLERODERMASCLERODERMA

SCLERODERMASCLERODERMA

DERMATOMYOSITISDERMATOMYOSITISAETIOPATHOGENESISAETIOPATHOGENESIS

-- HEREDITARY PREDISPOSITIONHEREDITARY PREDISPOSITION..

- -AUTOANTIBODIES IN DMAUTOANTIBODIES IN DM..

- -CYTOTOXIC T-CELLS IN PMCYTOTOXIC T-CELLS IN PM..

? - ? -PPTATED BY VIRAL OR STREPTOCOCCAL PPTATED BY VIRAL OR STREPTOCOCCAL PROTIENSPROTIENS..

? - ? -PPTATED BY INTERNAL MALIGNANCY IN PPTATED BY INTERNAL MALIGNANCY IN ADULTSADULTS . .

DERMATOMYOSITIS (DM)DERMATOMYOSITIS (DM)

EOSINOPHILIC FACIITISEOSINOPHILIC FACIITIS

VASCULITISVASCULITIS PATHOGENESISPATHOGENESIS

IMMUNOLOGICIMMUNOLOGIC::

- - SPECIFIC: types II, III, IV, V & VI SPECIFIC: types II, III, IV, V & VI hypersensitivityhypersensitivity . .

- - NON-SPECIFIC: ComplementNON-SPECIFIC: Complement..

NON-IMMUNOLOGICNON-IMMUNOLOGIC::

- -SCHWARTZMANN PHENOMENONSCHWARTZMANN PHENOMENON..

WEGNERWEGNER’’S S GRANULOMATOSISGRANULOMATOSIS

BALANCE PRESERVES BALANCE PRESERVES FUNCTIONFUNCTION

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