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Transcript
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.