1. Immunodefisiency &
Autoimmune

2. 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.

3. Classification
Primary secundary

4. 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)

5. 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

6. The International Union of Immunological Societies
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

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

8. 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)

11. Autoimmune

12. Autoimmune Diseases
• Loss of self-tolerance leads to production of
antibodies or T cells that react against one’s
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

13. Loss of Tolerance in Autoimmune Disease
Susceptibility genes Triggering factors (probably
(usually multiple) environmental)
Loss of tolerance
Auto reactive T cells Auto reactive B cells Inadequate regulatory
mechanism
Persistent pathogenic auto antibodies
Persistent pathogenic immune complexes
Persistent damaging auto reactive T cell

14. 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)

17. Type II antibody against cell-surface or matrix
antigens ( syndrome, autoantigen and
consequence )

18. Type III immune complex disease
( syndrome, autoantigen and consequence)

19. Type IV cell-mediated disease
(syndrome, autoantigen and consequence)

20. Autoantibodies against cell-surface receptors