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Auto Immune Disease

Auto Immune Disease
• An antigen (Ag) is defined as a substance, usually protein in nature, which when introduced

An antigen (Ag) is defined as a substance, usually protein in nature, which when introduced into the tissues stimulates antibody production.

Hapten is a non-protein substance which has no antigenic properties, but on combining with a protein can form a new antigen capable of forming antibodies.

An antibody (Ab) is a protein substance produced as a result of antigenic stimulation. Circulating antibodies are immunoglobulins(Igs) of which there are 5 classes: IgG, IgA, IgM, Ig E and Ig D.

TYPES OF IMMUNITY

TYPES OF IMMUNITY • . Broadly speaking, immunity or body defense mechanism is divided into 2

. Broadly speaking, immunity or body defense mechanism is divided into 2 types, each with humoral and cellular components:

Natural or innate immunity is non-specific and is considered as the first line of defense without antigenic specificity. It has 2 major components:

  • a) Humoral:comprised by complement.

  • b) Cellular: consists of neutrophils, macrophages, and natural killer (NK)

cells.

Specific or adaptive immunity is specific and is characterized by antigenic specificity.

It has 2 main components:

  • a) Humoral: consisting of antibodies formed by B cells.

  • b) Cellular: mediated by T cells.

Types of Immunity

Types of Immunity • Active Immunity - Naturally-Acquired Active Immunity - Artificially-Acquired Active Immunity • Passive

Active Immunity

  • - Naturally-Acquired Active Immunity

  • - Artificially-Acquired Active Immunity

Passive Immunity

  • - Naturally-Acquired Passive Immunity

  • - Artificially-Acquired Passive Immunity

STRUCTURE OF IMMUNE SYSTEM

STRUCTURE OF IMMUNE SYSTEM • ORGANS OF IMMUNE SYSTEM • Although functioning as a system, the

ORGANS OF IMMUNE SYSTEM

Although functioning as a system, the organs of immunesystem are distributed at different places in the body. These are as under:

a) Primary lymphoid organs:

i) Thymus ii) Bone marrow b) Secondary lymphoid organs:

i) Lymph nodes ii) Spleen

iii) MALT (Mucosa-Associated Lymphoid Tissue located inthe respiratory tract and GIT).

CELLS OF IMMUNE SYSTEM

CELLS OF IMMUNE SYSTEM i) Lymphocytes ii) Monocytes and macrophages iii) Mast cells and basophils iv)

i) Lymphocytes ii) Monocytes and macrophages iii) Mast cells and basophils iv) Neutrophils v) Eosinophils

Cells of the Immune System and their Functions.

Cells of the Immune System and their Functions.

HLA SYSTEM AND MAJORHISTOCOMPATIBILITY COMPLEX

HLA SYSTEM AND MAJORHISTOCOMPATIBILITY COMPLEX • HLA system is described here as it is considered important

HLA system is described here as it is considered important in the regulation of the immune system.

HLA stands for Human Leucocyte Antigens because these antigens or genetic proteins in the body which determine ones own tissue from non-self(histocompatibility) were first discovered on the surface of leucocytes.

• Subsequently, it was found that HLA are actually gene complexes of proteins on the surface

Subsequently, it was found that HLA are actually gene complexes of proteins on the surface of all nucleated cells of the body and platelets.

Since these complexes are of immense importance in matching donor and recipient for organ transplant,

they are called major histocompatibilitycomplex (MHC) or HLAcomplex.

Depending upon the characteristics of MHC, they havebeen divided into 3 classes

Depending upon the characteristics of MHC, they havebeen divided into 3 classes • Class I MHC

Class I MHC antigens have loci as HLA-A, HLA-B and HLA-C. CD8+(i.e. T suppressor) lymphocytes carry receptors for class I MHC and these cells are used to identify class I antigen on them.

Class II MHC antigens have single locus as HLA-D. These antigens have further 3 loci: DR, DQ and DP. Class II MHC is identified by B cells and CD4+ (i.e. T helper) cells.

Class III MHC antigens are some components of the complement system (C2 and C4) coded on HLA complex but are not associated with HLA expression and are not used in antigen identification.

ROLE OF HLA COMPLEX

ROLE OF HLA COMPLEX • The HLA complex is significant in a number of ways: •

The HLA complex is significant in a number of ways:

1. Organ transplantation. Historically, the major importance of HLA system is in matching donor and recipient for tissue transplantation.

The recipients immune system can recognize the histocompatibility antigens on the donor organ and accordingly accept it or reject it.

Both humoral as well as cell-mediated immune responses are involved in case of genetically non-identical transplants.

• 2 . Regulation of the immune system . Class I and II histocompatibility antigens play

2. Regulation of the immune system. Class I and II histocompatibility antigens play a role in regulating both cellular and humoral immunity:

Class I MHC antigens regulate the function of cytotoxic T cells (CD8+ e.g. in virus infections. Class II MHC antigens regulate the function of helper T cells (CD4+)

AUTOIMMUNE DISEASES

AUTOIMMUNE DISEASES • Autoimmunity is a state in which the body ’ s immune system fails

Autoimmunity is a state in which the bodys immune system fails to

distinguish between selfand non-selfand reacts by formation of autoantibodies against ones own tissue antigens.

In other words,there is loss of tolerance to ones own tissues autoimmunity is the opposite of immune tolerance.

• Immune tolerance is a normal phenomenon present since foetal life and is defined as the

Immune tolerance is a normal phenomenon present since foetal life and is defined as the ability of an individual to recognize self tissue and antigens. Normally, the immune system of the body is able to distinguish self from non-self antigens by the following mechanisms:

• 1 . Clonal elimination . According to this theory, during embryonic development, T cells maturing

1. Clonal elimination. According to this theory, during embryonic

development, T cells maturing in the thymus acquire the ability to distinguish self from non-self.

These T cells are then eliminated by apoptosis for the tolerant individual.

2. Concept of clonal anergy . According to this mechanism, T lymphocytes which have acquired the

2. Concept of clonal anergy. According to this mechanism, T

lymphocytes which have acquired the ability to distinguish self from

non-self are not eliminated but instead become non-responsive and

inactive.

3. Suppressor T cells . According to this mechanism, the tolerance is achieved by a population

3. Suppressor T cells. According to this mechanism, the tolerance is

achieved by a population of specific suppressor T cells which do not

allow the antigen-responsive cells to proliferate and differentiate.

PATHOGENESIS (THEORIES) OF AUTOIMMUNITY

PATHOGENESIS (THEORIES) OF AUTOIMMUNITY • The mechanisms by which the immune tolerance of the body is

The mechanisms by which the immune tolerance of the body is broken causes autoimmunity. These mechanisms or theories of autoimmunity may be immunological, genetic, and microbial, all of which may be interacting.

1. Immunological factors. Failure of immunological mechanisms of tolerance initiates autoimmunity. These mechanisms are as follows:

i) Polyclonal activation of B cells. B cells may be directly activated by stimuli such as infection with microorganisms and their products leading to bypassing of T cell tolerance.

ii) Generation of self-reacting B cell clones may also lead to bypassing of T cell tolerance.

ii) Generation of self-reacting B cell clones may also lead to bypassing of T cell tolerance.

iii) Decreased T suppressor and increased T helper cell activity. Loss of T suppressor cell and increase in T helper cell. activities may lead to high levels of auto-antibody production by B cells contributing to auto-immunity.

2. Genetic factors . There is evidence in support of genetic factors in the pathogenesis of

2. Genetic factors. There is evidence in support of genetic factors in the pathogenesis of autoimmunity as under:

i) There is increased expression of Class II HLA antigens on tissues involved in autoimmunity. ii) There is increased familial incidence of some of the autoimmune disorders.

3. Microbial factors . Infection with microorganisms, particularly viruses (e.g. EBV infection), and less often bacteria

3. Microbial factors. Infection with microorganisms,

particularly viruses (e.g. EBV infection), and less often

bacteria (e.g. streptococci, Klebsiella) and mycoplasma, has

been implicated in the pathogenesis of autoimmune diseases.

TYPES AND EXAMPLES OF AUTOIMMUNE DISEASES

TYPES AND EXAMPLES OF AUTOIMMUNE DISEASES • Depending upon the type of autoantibody formation, the autoimmune

Depending upon the type of autoantibody formation, the autoimmune diseases are broadly classified into 2 groups:

1. Organ specific diseases. In these, the autoantibodies formed react specifically against an organ or target tissue component and cause its chronic inflammatory destruction. The tissues affected are endocrine glands (e.g. thyroid, pancreatic islets of Langerhans, adrenal cortex), alimentary tract, blood cells and various other tissues and organs.

2. Organ non-specific (Systemic) diseases

2. Organ non-specific (Systemic) diseases • These are diseases in which a number of autoantibodies are

These are diseases in which a number of autoantibodies are formed which react with antigens in many tissues and thus cause systemic lesions. The examples of this group are various systemic collagen diseases. However, a few autoimmune diseases overlap between these two main categories.

AMYLOIDOSIS

AMYLOIDOSIS Amyloidosis is the term used for a group of diseases characterised by extracellular deposition of

Amyloidosis is the term used for a group of diseases characterised by extracellular deposition of fibrillar proteinaceous substance called amyloid having common morphological appearance, staining properties and physical structure but with variable protein (or biochemical) composition.

• First described by Rokitansky in 1842, the substance was • subsequently named by Virchow as

First described by Rokitansky in 1842, the substance was subsequently named by Virchow as amyloidunder the mistaken belief that the material was starch-like (amylon =starch). This property was demonstrable grossly on the cut surface of an organ containing amyloid which stained brown