The document discusses immunodeficiency disorders, which occur when the immune system is reduced or absent, leading to recurrent infections. It describes primary immunodeficiencies, which are often hereditary, and secondary immunodeficiencies, which can be acquired. The document also covers autoimmune diseases, where the immune system attacks the body's own tissues, classifying them as cytotoxic, immune complex, or cell-mediated based on the pathogenic mechanism.
This presentation is an overview of primary and secondary immunodeficiency disorders with highlights on the genetic basis of primary disorders and associated factors underlying secondary disorders, as well a management of these disorders
Pediatric Home Service Medical Director, Dr. Roy Maynard discusses deficiencies of innate immune system and other well-defined immunodeficiency syndromes.
This presentation is an overview of primary and secondary immunodeficiency disorders with highlights on the genetic basis of primary disorders and associated factors underlying secondary disorders, as well a management of these disorders
Pediatric Home Service Medical Director, Dr. Roy Maynard discusses deficiencies of innate immune system and other well-defined immunodeficiency syndromes.
From Queens Library's expert-led panel, Cancer Awareness: What You Need to Know, featuring professionals from New York Hospital Queens, North Shore LIJ, the American Cancer Society, and the Leukemia and Lymphoma Society
Normally, immune responses eradicate infectious pathogens without serious injury to host tissues.
However, these responses are sometimes
inadequately controlled
inappropriately targeted to host tissues
triggered by commensal microorganisms or environmental antigens that are usually harmless.
In these situations, the normally beneficial immune response is the cause of disease.
Disorders caused by immune responses are called hypersensitivity diseases.
This term , hypersensitivity , arose from the clinical definition of immunity as sensitivity, which is based on the observation that an individual who has been exposed to an antigen exhibits a detectable reaction, or is sensitive, to subsequent encounters with that antigen.
Today, we will describe the pathogenesis of different types of hypersensitivity reactions, with an emphasis on the effector mechanisms that cause tissue injury.
A variety of human diseases are caused by immune responses to non-microbial environmental antigens, and involve the type 2 cytokines interleukin-4 (IL-4), IL-5, and IL-13 produced by Th2 cells and innate lymphoid cells (ILCs), immunoglobulin E (IgE), mast cells, and eosinophils.
The antigens that elicit immediate hypersensitivity are called allergens. Most of them are common environmental proteins, animal products, and chemicals that can modify self proteins.
In the effector phase of these responses, mast cells and eosinophils are activated to rapidly release mediators that cause
increased vascular permeability
Vasodilation
bronchial and visceral smooth muscle contraction
This vascular reaction is called immediate hypersensitivity because it begins rapidly, within minutes of antigen challenge in a previously sensitized individual (immediate), and has major pathologic consequences (hypersensitivity).
Following the immediate response, there is a more slowly developing inflammatory component called the late-phase reaction characterized by the accumulation of neutrophils, eosinophils, and macrophages.
Autoimmunity and autoimmune diseases dr. ihsan alsaimarydr.Ihsan alsaimary
Dr. ihsan edan abdulkareem alsaimary
PROFESSOR IN MEDICAL MICROBIOLOGY AND MOLECULAR IMMUNOLOGY
ihsanalsaimary@gmail.com
mobile : 009647801410838
university of basrah - college of medicine - basrah -IRAQ
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.
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)
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)
15.
16.
17. Type II antibody against cell-surface or matrix
antigens ( syndrome, autoantigen and
consequence )
18. Type III immune complex disease
( syndrome, autoantigen and consequence)