Immunodefisiency &

Autoimmune
dr. Isbandiyah, SpPD

Introduction
Immunodeficiency disorders occur
when the body's immune response is
reduced or absent
T or B cell lymphocytes (or both) do
not work as well as they should, or
when your body doesn't produce
enough antibodies.

Classification

Primar
y

secundar
y

Primary
immunodeficiency (PID)
Many of these disorders are hereditary
and are autosomal recessive or X-linked.
Gx: recurrent or persistent infection
Other signs include:
Poor response to treatment
Delayed or incomplete recovery from illness
Certain types of cancers (such as
Kaposi's sarcoma or non-Hodgkin's lymphoma)
Certain infections (including some forms of
pneumonia or recurrent fungal yeast
infections)

Diagnosis
full blood count (including accurate
lymphocyte and granulocyte counts)
immunoglobulin levels (the three
most important types of antibodies:
IgG, IgA and IgM).
Complement levels

The
International Union of Immunological So
cieties
recognises eight classes of primary
immunodeficiencies

Combined T- and B-cell immunodeficiencies

Antibody deficiencies
Well-defined syndromes
Immune dysregulation diseases
Phagocyte disorders
Innate immunity deficiencies
Autoinflammatory disorders
Complement deficiencies

Treatment of primary
immunodeficiencies
intravenous immunoglobulin (IVIG) or
subcutaneous immunoglobulin (SCIG)
in antibody deficiencies
hematopoietic stem cell transplantatio
n
(for SCID and other severe
immunodeficiences)
Reduction of exposure to pathogens,
and in many situations prophylactic
antibiotics may be advised.

Acquired immunodeficiency
"secondary" or "acquired"
immunodeficiency
Common causes for secondary
immunodeficiency are malnutrition, aging
and particular medications (e.g.
chemotherapy,
disease-modifying antirheumatic drugs,
immunosuppressive drugs, after organ
transplants, glucocorticoids).
cancer, particularly those of the bone
marrow and blood cells (leukemia,
lymphoma, multiple myeloma), and certain
chronic infections. Immunodeficiency is also
the hallmark of acquired immunodeficiency
syndrome (AIDS)

Autoimmune

Autoimmune Diseases
Loss of self-tolerance leads to
production of antibodies or T cells
that react against ones own
antigens.
Immune system response to self
antigens causes damage to
organs.
The major factors that contribute to
the development of autoimmunity are
genetic susceptibility and
environmental triggers

Loss of Tolerance in Autoimmune Disease

Triggering factors (probably
environmental)

Susceptibility genes
(usually multiple)
Loss of tolerance

Auto reactive T cells

Auto reactive B cells

Persistent pathogenic auto antibodies

Persistent pathogenic immune complexes
Persistent damaging auto reactive T cell

Inadequate regulatory
mechanism

Classification
Autoimmune diseases may be either
systemic or organ specific
Three types of autoimmune
disorders:
Cytotoxic (Type II reactions)
Immune complex (Type III
reactions)
Cell-mediated (Type IV reactions)

Type II antibody against cell-surface or

matrix antigens ( syndrome, autoantigen
and consequence )

Type III immune complex disease

( syndrome, autoantigen and consequence)

Type IV cell-mediated disease

(syndrome, autoantigen and
consequence)

Autoantibodies against cell-surface

receptors