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Autoimmune diseases 1 Running head: CASE STUDY 1. What is the basis of autoimmune diseases (list 4 diseases). Anthony Kyle Johnson Stacey Elder Chamille Gumprecht Hope Galas Dorota Kent State University Autoimmune diseases 2 Overview As healthy functioning individuals, we have an immune system which protects prevents us from disease and infection. Autoimmune diseases cause the immune system to mistakenly attack healthy cells in the body. These diseases usually run in families and most commonly affect women, especially during child-bearing years. There are over 80 types of autoimmune diseases. These disorders range from diseases in which one organ or one type of tissue is affected to generalized or systemic disorders and they affect the following parts of the body: the endocrine system (i.e. grave’s diseases), skin (i.e. vitiligo), neuromuscular tissue (i.e. multiple scleroris), gastrointestinal system (i.e. Crohn’s disease), eye (i.e. Sjögren's disease), connective tissue (i.e. rheumatoid arthritis), renal system (i.e. immune complex glomerulonephritis), hematologic system (i.e. autoimmune hemolytic anemia) and respiratory system (i.e. goodpasture syndrome) (Abbas, 2005, National Institutes of Health, 2008, Rote, 2002). The causes of these diseases are not entirely understood unknown, but contributing factors include the environment, genetic predisposition, drugs, sunlight and hormones, all of which can alter the immune system. Infections also seem to play a role, a role that is not yet fully understood. Rheumatic heart disease is the one autoimmune disease clearly linked to an infection. Antibodies against streptoccoocal proteins mimic myocardial proteins well enough to cross-react with them, causing myocarditis (Abbas, 2005). Many times, an autoimmune disease develops as a result of preexisting infections (Rote, 2002). Pathophysiology Autoimmune diseases 3 During embryonic development we develop immunologic tolerance which allows our body to tolerate self-antigens. The autoimmune process begins when the mechanisms that regulate immunologic tolerance fail and T cells and antibodies begin to destroy host tissues immune system produces T cells and antibodies which work against self-antigens and destroy host tissues (National Institutes of Health, 2008). This process triggers the body to produce high quantities of autoantibodies (antibodies against self-antigens). The end result is an immune system which lacks tolerance and begins to identify self-antigens as foreign bodies. Types of Autoimmune Diseases Graves Disease Graves disease, an organ specific autoimmune disease, occurs when maternal thyroid autoantibodies stimulate a TSH receptor and therefore causes neonatal hyperthyroidism. As many of the thyroid hormones (TSH, TRH, T3) remain at increased levels, the immune system can no longer respond appropriately which cause hyperfunction of the thyroid gland and the various thyrotoxic manifestations (Rote, 2002). Clinical manifestations of a thyrotoxic crisis are increased heart rate, diarrhea, nausea, vomiting, anxiety, and confusion (Huether, 2002). Thyrotoxic crisis, a life-threatening complication can occur with a worsening of the thyrotoxic state. This must be treated immediately by trying to reduce TSH levels, and provide supportive care (Rote, 2002). Systemic Lupus Erythematosus Systemic Lupus Erythematosus, SLE is a common and serious systemic autoimmune disease characterized by an overactive immune response causing a butterfly Autoimmune diseases 4 rash across the nose and cheeks and other parts of the body, photosensitivity, swelling and damage to various organs such heart, lungs, kidneys, blood vessels, brain, skin and joints (often painful) (National Institutes of Health, 2008). These systems are caused by a variety of autoantibodies that oppose various cellular structures such as nucleic acids, erythrocytes, phospholipids, lymphocytes and other self-components. The tissue damage is caused by release of immune complexes with antibodies against the host DNA. These immune complexes cause inflammatory lesions in the above listed organs and a state of chronic inflammation (Rote, 2002). Anti-Glomerular Basement Membrane Disease (Good Pasture Syndrome) This organ specific disease is an example of rapidly progressive glomerulonephritis and is caused by formation of antibodies against a collagen located in the pulmonary capillaries and glomerular basement membranes. This disease is unique in that it usually affects men in their 20s and 30s or women in their 60s and 70s. The attack of these antibodies results in pulmonary hemorrhage and rapidly progressing glomerulonephritis. This progression results in renal failure with clinical manifestations of hematuria, proteinuria, edema, and hypertension. Due to the pulmonary-renal syndrome, this disease is also referred to as good pasture syndrome (Huether, 2002). Crohn Disease (Regional Enteritis, Regional Ileitis) Crohn disease is an idiopathic autoimmune disorder which affects the gastrointestinal tract. The inflammatory lesions are first seen in the intestinal submucosa and eventually spread to the mucosa and serosa. A typical granuloma appears in the inflamed tissue with cobblestone projections with surrounding areas of ulceration. Skip lesions are common and seen when inflammation affects some haustral areas, leaving the Autoimmune diseases 5 other areas unaffected. The extensive gastrointestinal inflammation can also extend into the lymphoid tissue. These findings contribute to the gastrointestinal symptoms including abdominal pain and diarrhea. Other manifestations include right side abdominal tenderness related to inflammation. If Crohn disease is present for a prolonged period, it increases risk of intestinal adenocarcinoma (Huether, 2002). Multiple Sclerosis Multiple Sclerosis (MS) is an autoimmune disorder involving the central nervous system. It is usually seen in adults ages 20-40. MS is caused by a response to a viral agent with questionable genetic predisposition. The autoimmune response produces antibodies to mylein basic protein, the inflammation causes damage to the myelin sheath and the axons. When the inflammation response starts to resolve plaques and diffuse lesions are present, resulting in the demyelinating process involving the white matter of the CNS. The demyelination causes conduction block throughout the CNS. The progression of the disease can progress over 20 years with remission in the early stage leading to debilitation. Disorders in the immune system results in disease. Unfornately, in autoimmune disorders the body’s immune system attacks not only the bad cells such as bacteria, viruses, and other such harmful invaders but it attacks healthy cell as well resulting in tissue destruction. Autoimmune diseases 6 References: Abbas, A. K. (2005). Diseases of immunity. Robbins and Cotran pathologic basis of disease, 193-267). Philadelphia, Pennsylvania: Elsevier Saunders. National Institutes of Health (2008). Autoimmune Diseases. Retrieved September 2, 2008 from http://www.nlm.nih.gov/medlineplus/autoimmunediseases.html. Huether, S. E. (2002). Alterations of Digestive Function. In KL McCance and SE Huether (Eds.), Pathophysiology: the biologic basis for disease in adults and children, 1261-1313. St. Louis, Missouri: Mosby. Huether, S.E (2002). Alterations of Renal and Urinary Tract Function. In KL McCance and SE Huether (Eds.), Pathophysiology: the biologic basis for disease in adults and children, 1191-1216. St. Louis, Missouri: Mosby. National Institute of Allergy and Infectious Disease (2008). Disorders of the Immune System. Retrieved September 2, 2008 from http://www3.niaid.nih.gov/topics/immuneSystem/disorders.htm. National Institutes of Health (2008). Autoimmune Diseases. Retrieved September 2, 2008 from http://www.nlm.nih.gov/medlineplus/autoimmunediseases.html. Rote N.S. (2002). Infection and Alterations in Immunity and Inflammation. In KL McCance and SE Huether (Eds.), Pathophysiology: the biologic basis for disease in adults and children, 227-271. St. Louis, Missouri: Mosby.