Group 4

Autoimmune Disorders: Organ

Nonspecific
Introduction
 Autoimmunization involves the recognition of self
antigens by the immune system and may be considered as
the loss of immunologic tolerance to self antigens.


 Autoimmune diseases occur more frequently in

women and in adults more frequently in children.


 The term autoimmune disease is used when

demonstrable immunoglobulins or cytotoxic cells display a
specificity for self antigens or autoantigens and contribute
to the pathogenesis of the disease.



Nonorgan-specific Disorders

 Many disorders are believed to be

related to immunologic abnormalities, and
the identification of additional diseases
grows continually. Autoimmune diseases
exhibit a full spectrum of tissue reactivity.
At one extreme are diseases that manifest
themselves as nonorgan-specific diseases
such as systemic lupus erythematosus
(SLE) and rheumatoid arthritis (RA).


 Nonorga-specific disorders are

characterized by the presence of both
lesions and autoantibodies not confined to
any one organ.
Factors Influencing the Development of
Autoimmunity

1.Genetic factors
2.Age
3.Exogenous Factors
Features of Non-organ specific AID
 Serological and clinical overlap may occur within families.

 The damage is related to immune complex deposition in
the tissues.

 Well-established tolerance in health to the antigens
concerned makes it difficult to reproduce the disease
experimentally.

 Natural animal models such as NZB ,ice and SLE dogs are
the only way of studying the disease experimentally.

 Treatment has to be immunosupressive therapy and
anitinflammmatory drugs and plasmapheresis.

Rheumatoid arthritis (RA)
 The majority of adult RA patients have IgM
rheumatoid factor (RF) in their serum detectable
by routine laboratory tests with sensitized sheep
cells aor latex agglutination. A portion of juvenile
patients, however, have only IgG-RF, which is not
detectable by these routine tests. They have
milder disease, without rheumatoid nodules and
vasculitis that complicate severe seropositive RA.
Either type of RF, however, can induce
immunecomplex-mediated arthritis.
Signs and Symptoms
 RA is a chronic, usually progressive
inflammatory disorder of the joints. It is
however, a highly variable disease that
ranges from a mild illness of brief duration to
a progressive, destructive polyarthritis
associated with systemic vasculitis.
 Three Distinct Stages:
1.Initiation os synovitis by the primary etiologic
facter.
2.Subsequent immunologic events that
perpetuate the initial inflammatory reaction
3.Transition of an Inflammatory reactuion in the
synovium to a proliferative destructive
process of tissue.
SystemiC lupus erythematosus (SLE)
 This multisystemic disease is caused by over-
reactivity of the T helper and B cell systems due
to suboptimal function of the T suppressor cell
circuit. A wide range of antibodies against
somatic epitopes therefore develop, for instance
against nucleoproteins, lymphocytes, RBC,
platelets, clotting factors, immunoglobulins and
neurones.
 The tissue damage in SLE is either
caused by the immune complex
mechanism, as in skin, kidney, and
joints, or by direct cytotoxic damage,
as in the case of the blood elements
and neurones, resulting in
lymphopenia, hemolytic anemia,
thrombocytopenia, bleeding tendency
and neurological symptoms
respectively. In some cases of SLE anti-
lymphocyte antibodies appear to cross-
react with CNS antigens, causing
neurological symptoms and with
trophoblastic tissues, causing repeated
Immunologic Manifestations

 Patients with SLE are known to produce

multiple autoantibodies. There are two
leading hypothesis as to why so many
different antibodies develop.
 One hypothesis supports the belief that
antibody-forming B lymphocytes are
stimulated in a relatively non-specific
fashion, so-called polyclonal B-cell
activation.
 . The second hypothesis is that the
immune response in SLE is specifically
stimulated by antigens.
Cellular Aspects

 SLE is a disease that results from

defects in the regulatory mechanism
of the immune system. Studies of the
immunopathogenesis of lupus
nephritis have demonstrated a variety
of aberrations in T cell and B cell
function.
 Lymphocyte subset abnormalities are a
major immunologic feature of SLE.
Humoral Aspects

 Patients with SLE exhibit multiple serum

antibodies that react with native or
altered self-antigens.
 Nuclear components
 Cell surface and cytoplasm antigens of
polymorphonuclear and lymphocytic
leukocytes, erythrocytes, platelets, and
neuronal cells.
 Immunoglobulin IgG


Diagnostic evaluation
 The manifestations of SLE expressed in
laboratory findings are numerous.
 Histologic,

 hematologic
 serologic abnormalities
 The tissue damage in SLE is either
caused by the immune complex
mechanism, as in skin, kidney, and
joints, or by direct cytotoxic damage,
as in the case of the blood elements
and neurones, resulting in
lymphopenia, hemolytic anemia,
thrombocytopenia, bleeding tendency
and neurological symptoms
respectively. In some cases of SLE anti-
lymphocyte antibodies appear to cross-
react with CNS antigens, causing
neurological symptoms and with
trophoblastic tissues, causing repeated
References
 Immunology Simplified, 2nd Edition by
Bowry
 Immunology and Serology in Laboratory
Medicine, 2nd Edition by Mary Louise
Turgeon
 Basic and Clinical Immunology by D.P
Stites et. al.