Autoimmune Case Study

Autoimmune disease- any of a large group of diseases characterized by abnormal functioning of the immune system that causes your immune system to produce antibodies against your own tissues (the definition from wordnetweb.pronceton.edu).

As I was doing my research I came across the autoimmune part and totally lost. It’s a breakdown in the immune system. As we know every person produces their own cell types that fight off disease and infection. In order to fight off the bad bacteria our bodies must be able to recognize the bad known as antigens (often proteins) on the surface of the UN wanted invaders. Everyone’s immune system should be able to recognize the bad, and be able to fight it off. In an autoimmunity this is where the immune system is not able to recognize the invaders and fight them off.

Overwhelming, deleterious host immune response to an infection due to release of chemicals to fight the infection 2• 1

Acquired Immune System – Specific for distinct pathogens and the ability to create immunological memory; requires presentation of foreign antigens by antigen presenting cells(Rodriguez, 2014, p. 86).

this occurs in a series of steps, the first of which is incorporation of unidentified antigens by APCs in the epidermis and dermis. This process involves binding of the antigens to the MHC on the APC surface and the APC migrates to the lymph nodes. There, the APC binds reversibly and briefly with naïve or resting T cells through interactions between surface molecules located on both cells. Next, the MHC presents the antigen to a T lymphocyte receptor to begin activation of the T lymphocyte. The second signal for T lymphocyte activation is a non-antigen/ cell-cell interaction known as costimulation. If costimulation does not occur, the T lymphocyte will either undergo apoptosis or become unresponsive. Costimulation involves pairing of receptor with ligand on the T cell; these pairs include (LFA)-3 interacting with CD2, B7 interacting with CD28, and ICAM-1 interacting with LFA-1 (Lebwohl, 2003).

A. An autoimmune disease is a disorder in which the body’s immune system attacks the body’s own cells and organs.

A disorder where the body is attacking itself is known as an autoimmune disorder. In an autoimmune disorder the body cannot decipher between a pathogen, bacteria, or infection attacking the body and normal healthy body tissue. The majority of autoimmune disorders are long lasting or even permanent. An example of a chronic autoimmune disorder is multiple sclerosis (MS).

To understand autoimmune diseases, the general characteristic of autoimmunity must be addressed. Autoimmunity is defined, in short, as “ misdirected immune response”. A healthy person is equipped with the mechanisms necessary to defend the body from pathogens within the immune system. When autoimmunity is present in an individual, something within the immune system is

Lupus is a chronic inflammatory disease marked by its effect on various parts of the body, including the joints, skin, blood, and kidneys. It is a condition in which the body's immune system attacks its own cells and tissues, resulting in pain, inflammation, and often damage to organs. Lupus involves the immune system. The immune system makes antibodies that work to protect the body against foreign substances like viruses and bacteria. Such foreign bodies are called antigens. When a person has lupus, his or her body is unable to determine the difference between antigens and the individual's cells and body tissues. As such, the immune system creates antibodies against the individual's own tissues. These antibodies are called autoantibodies. Depending on the type of lupus, a wide range of symptoms may be experienced, from rashes, hair loss, and achy, swollen joints to fever, anemia, and abnormal blood clotting. Though the disease can affect many parts of the body, individuals usually experience symptoms in only a few organs. There is no known cure for lupus. However,

Autoimmune Lymphoproliferative Syndrome (ALPS) is a heritable genetic disease, marked by mis-regulated lymphoproliferation, due to an inoperative FAS/FASL apoptotic pathway in lymphocytes (Sneller et al.1992). An immune response generates large amounts lymphocytes, to contend with pathogen insults, and the subsequent dispatch of the pathogen. The immune response then requires suppression of the activated lymphocytes, terminating the immune response. This suppression is facilitated by the cell-mediated process of apoptosis. During the suppression of the immune response, T lymphocytes express FAS and other B and T lymphocytes generate FASL (Nagata & Golstein 1995). The expression of FAS and FASL are the prerequisite components of FAS/FASL apoptosis, and any dysfunction of this pathway negates the ability of the immune system to regulate the suppression of activated lymphocytes. Mis-regulatiuon, of the activated lymphocytes, causes an inordinate population of T lymphocytes, which inappropriately target host tissues leading to ALPS. There are several subcategories of the ALPS disease, each is distinguished by an individual gene mutation, producing several diverse proteins that constitute the FAS/FASL apoptotic pathway. This brief synopsis will focus on the ALPS attributed mutation of the FAS gene, classified as ALPS 1A, which constitutes 75% proportion of ALPS cases (Bleesing et al. 2005).

Immunology basically involves understanding the immune system and how it responds to various disease conditions. the immune system consists of a number of components. Traditionally, it is divided into humoral and cellular immune responses. It can also be distinguished into innate and adaptive immunity. The innate immunity can discriminate between normal tissues , self and newly encountered non-self-proteins while the adaptive immunity is the more complex system aimed at the eradication of intracellular pathogens. To do this, antigen derived from such pathogens that are often new to the host organism, need to be recognised by receptor-bearing specialised immune cells which respond to a complex system of stimulatory and costimulatory signals. Better understanding of the human immune system has led to the identification of a number of tumor-associated antigens in the 1980s and the development of various immunotherapeutic approaches. In recent years, identification of the specific antigenic MHC class I epitopes, advancements in genetic engineering, gene delivery, and cell-based therapeutic approaches allowed development of the novel immunotherapeutics.

With autoimmune diseases, the underlying issue is similar. The body's immune system becomes unable to recognize healthy cells from diseased cells. Consequently, the immune’s own defense mechanism produces antibodies that attack the normal tissues and cells. Autoimmune diseases

Scientists who research the epidemiology of autoimmune diseases have experienced many complications in their work. In a field where one plus one rarely equals two, researchers have been work fervently to determine which cell types are responsible for, or at least involved in, autoimmune dysfunction. The main subject of study by Esposito et al. is the deleterious effects of chronic activation of proinflammatory cytokines. To this end, this team has sought to characterize and quantify the cells found in various loci in mice plagued with relapsing-remitting experimental autoimmune encephalomyelitis (RR-EAE), an induce murine model of human multiple sclerosis (MS). Another question of paramount importance is how to elucidate the mechanisms of these cells and their specific cell markers. For example, are certain cells present only in the target organ? Or did these cells migrate to that location? Or were there sequential stages of differentiation that occurred? Or is it a combination of the aforementioned postulates?

When the pathogen replicates different components of immune system evolves to protect against various types of pathogens. However infection of an organism does not necessarily shows diseases, disease are only present when the bolus of infection, in terms of when immunity is comprised. All components of

Our immune system is the second most complex system in our body. It is made up of organs, cells and proteins that work together to protect our bodies from harmful bacteria, viruses or other microorganisms that can cause diseases. Usually we don’t notice our immune system defending us against pathogens, but if the pathogen (harmful microorganism) is aggressive or if our body hasn’t ever come into contact with it, we can get sick. The jobs of our immune system are to recognise pathogens, as well as neutralise and remove them from our body. Our immune system also has to fight our own cells if they have changed due to an illness, for example, cancer. (1)