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Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body attacks its own cells. Autoimmune diseases are a major cause of immune-mediated diseases.

Today there are more than 40 human diseases classified as either definite or probable autoimmune diseases, and they affect 5% to 7% of the population. Almost all autoimmune diseases appear without warning or apparent cause, and most patients suffer from fatigue. Few examples: Alopecia Areata, Allergic granulomatosis and angiitis, Acute disseminated encephalomyelitis, Anklosing spondylitis, Autoimmune hemolytic anemia, Autoimmune retinopathy, Behcet's syndrome, Bullus pemphigoid, Dermatitis herpetiformis, Discoid lupus erythematosus, Epidermolysis bullosa, Graves' Disease, Gullain-Barre syndrome, Hashimoto's thyroiditis, Kawasaki disease (KD), Meniere's Disease, Myasthenia gravis, Neutropenia, Orchitis, Primary biliary Cirrhosis, Raynaud's phenomenon

Gender influence

Women tend to be affected more often by autoimmune disorders; nearly 79% of autoimmune disease patients in the USA are women. Also they tend to appear during or shortly after puberty. It is not known why this is the case, although hormone levels have been shown to affect the severity of some autoimmune diseases such as multiple sclerosis.[1] Other causes may include the presence of fetal cells in the maternal bloodstream. [2]

Autoimmune diseases

Diseases with a complete or partial autoimmune etiology:

Accepted Autoimmune diseases

The "Mesh" column lists those conditions that are classified as autoimmune by the MeSH system.

Name MeSH?  ICD-10  Description
Acute disseminated encephalomyelitis (ADEM) yes G04.0 is a form of encephalitis caused by an autoimmune reaction and typically occurring a few days or weeks after a viral infection or a vaccination.
Addison's disease yes E27 is often caused by autoimmune destruction of the adrenal cortex.
Ankylosing spondylitis yes M08.1, M45. is a chronic, painful, progressive inflammatory arthritis primarily affecting spine and sacroiliac joints, causing eventual fusion of the spine.
Antiphospholipid antibody syndrome (APS) yes D68.8 affects the blood-clotting process. It causes blood clots to form in veins and/or arteries.
Aplastic anemia no D60 is often caused by an autoimmune attack on the bone marrow.
Autoimmune hepatitis no K75.9 is a disorder wherein the liver is the target of the body's own immune system.
Autoimmune Oophoritis no N70 is a disorder in which the immune system attacks the female reproductive organs.
Celiac disease no K90.0 is a disease characterized by chronic inflammation of the proximal portion of the small intestine caused by exposure to certain dietary gluten proteins.
Crohn's disease no K50 is a form of inflammatory bowel disease characterized by chronic inflammation of the intestinal tract. Major symptoms include abdominal pain and diarrhea. There is also a theory that Crohn's Disease is an infectious disease caused by Mycobacterium avium paratuberculosis.
Diabetes mellitus type 1 yes E10 when it is characterized by a deficiency or absence of insulin production (Type I), is often the consequence of an autoimmune attack on the insulin-producing beta cells in the islets of Langerhans of the pancreas.
Gestational pemphigoid no O26.4 is a pregnancy-related blistering condition where autoantibodies are directed against the skin.
Goodpasture's syndrome yes M31.0 is a disease characterised by rapid destruction of the kidneys and haemorrhaging of the lungs through autoimmune reaction against an antigen found in both organs.
Graves' disease yes E05.0 is the most common form of hyperthyroidism, and is caused by anti-thyroid antibodies that have the effect of stimulating (agonist) the thyroid into overproduction of thyroid hormone.
Guillain-Barré syndrome (GBS) yes G61.0 is an acquired immune-mediated inflammatory disorder of the peripheral nervous system (i.e., not the brain and spinal column). It is also called acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis and Landry's ascending paralysis.
Hashimoto's disease yes E06.3 is a common form of hypothyroidism, characterised by initial inflammation of the thyroid, and, later, dysfunction and goiter. There are several characteristic antibodies (e.g., anti-thyroglobulin).
Idiopathic thrombocytopenic purpura yes D69.3 is an autoimmune disease where the body produces anti-platelet antibodies resulting in a low platelet count
Kawasaki's Disease no M30.3 is often caused by an autoimmune attack on the arteries around the heart.
Lupus erythematosus yes L93, M32 is a chronic (long-lasting) autoimmune disease wherein the immune system, for unknown reasons, becomes hyperactive and attacks normal tissue. This attack results in inflammation and brings about symptoms. This is a "Non-organ-specific" type of autoimmune disease.
Mixed Connective Tissue Disease     has features of other connective tissues diseases — lupus, rheumatoid arthritis, scleroderma and polymyositis. The presence of a specific antibody — called U1-RNP is needed for diagnosis.
Multiple sclerosis yes G35 is a disorder of the central nervous system (brain and spinal cord) characterised by decreased nerve function due to myelin loss and secondary axonal damage.
Myasthenia gravis yes G70.0 is a disorder of neuromuscular transmission leading to fluctuating weakness and fatigue. Weakness is caused by circulating antibodies that block (antagonist) acetylcholine receptors at the neuromuscular junction.
Opsoclonus myoclonus syndrome (OMS) n/a n/a is a neurological disorder that appears to the result of an autoimmune attack on the nervous system. Symptoms include opsoclonus, myoclonus, ataxia, intention tremor, dysphasia, dysarthria, mutism, hypotonia, lethargy, irritability or malaise. About half of all OMS cases occur in association with neuroblastoma.
Optic neuritis no H46 is an inflammation of the optic nerve that may cause a complete or partial loss of vision.
Ord's thyroiditis n/a n/a is a thyroiditis similar to Hashimoto's disease, except that the thyroid is reduced in size. In Europe, this form of thyroid inflammation is more common than Hashimoto's disease.
Pemphigus yes L10 is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes.
Pernicious anaemia no D51.0 is an autoimmune disorder characterised by anaemia due to malabsorption of vitamin B12
Polyarthritis in dogs n/a n/a is an immune reaction severely affecting the joints of dogs. Although rare and of unknown cause it can render a dog immobile even at a very young age. Treatment includes cortisone-type drugs.
Primary biliary cirrhosis no K74.3 appears to be an autoimmune disease that affects the biliary epithelial cells (BECs) of the small bile duct in the liver. Although the cause is yet to be determined, most of the patients (>90%) appear to have auto-mitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC), an enzyme that is found in the mitochondria.
Rheumatoid arthritis yes M05-M06 is an autoimmune disorder that causes the body's immune system to attack the bone joints.
Reiter's syndrome no M02 seems to be an autoimmune attack[citation needed] on various body systems in response to a bacterial infection and the body's confusion over the HLA-B27 marker
Sjögren's syndrome yes M35.0 is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva.
Takayasu's arteritis no M31.4 is a disorder that results in the narrowing of the lumen of arteries.
Temporal arteritis (also known as "giant cell arteritis") yes M31.5 is an inflammation of blood vessels, most commonly the large and medium arteries of the head. Untreated, the disorder can lead to significant vision loss.
Warm autoimmune hemolytic anemia n/a D59.1 is a disorder characterized by IgM attack against red blood cells
Wegener's granulomatosis no M31.3 is a form of vasculitis that affects the lungs, kidneys and other organs.

 

Suspected

Diseases suspected or theorized to be linked to autoimmunity are:

 

References

  1. ^ A Gender Gap in Autoimmunity. Retrieved on 2007-10-19.
  2. ^ JAMA -- Abstract: Microchimerism: An Investigative Frontier in Autoimmunity and Transplantation, March 3, 2004, Adams and Nelson 291 (9): 1127. Retrieved on 2007-10-19.
  3. ^ Hyland KV, Engman DM (2006). "Further thoughts on where we stand on the autoimmunity hypothesis of Chagas disease". Trends Parasitol. 22 (3): 101-2; author reply 103. doi:10.1016/j.pt.2006.01.001. PMID 16446117. 
  4. ^ Gleicher N, el-Roeiy A, Confino E, Friberg J (1987). "Is endometriosis an autoimmune disease?". Obstetrics and gynecology 70 (1): 115-22. PMID 3110710. 
  5. ^ Clinical Trial: Etanercept in Hidradenitis Suppurativa. Retrieved on 2007-07-08.
  6. ^ Kárpáti F, Dénes L, Büttner K (1975). "[Interstitial cystitis=autoimmune cyatitis? Interstitial as a participating disease in lupus erythematosus]" (in German). Zeitschrift für Urologie und Nephrologie 68 (9): 633-9. PMID 1227191. 
  7. ^ Takehara K, Sato S (2005). "Localized scleroderma is an autoimmune disorder". Rheumatology (Oxford, England) 44 (3): 274-9. doi:10.1093/rheumatology/keh487. PMID 15561734. 
  8. ^ Maddison P (2006). "Neuromyotonia". Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology 117 (10): 2118-27. doi:10.1016/j.clinph.2006.03.008. PMID 16843723. 
  9. ^ Carlander, B., Eliaou J.F., Billiard M. (1993). "Autoimmune hypothesis in narcolepsy.". Neurophysiol. Clin. 23. 
  10. ^ Eaton WW, Byrne M, Ewald H, et al (2006). "Association of schizophrenia and autoimmune diseases: linkage of Danish national registers". The American journal of psychiatry 163 (3): 521-8. doi:10.1176/appi.ajp.163.3.521. PMID 16513876. 
  11. ^ Jones AL, Mowry BJ, Pender MP, Greer JM (2005). "Immune dysregulation and self-reactivity in schizophrenia: do some cases of schizophrenia have an autoimmune basis?". Immunol. Cell Biol. 83 (1): 9-17. doi:10.1111/j.1440-1711.2005.01305.x. PMID 15661036. 
  12. ^ Strous RD, Shoenfeld Y (2006). "Schizophrenia, autoimmunity and immune system dysregulation: a comprehensive model updated and revisited". J. Autoimmun. 27 (2): 71-80. doi:10.1016/j.jaut.2006.07.006. PMID 16997531. 
  13. ^ Questions and Answers about Vitiligo. Retrieved on 2007-08-06.
  14. ^ A New Gene Linked to Vitiligo and Susceptibility to Autoimmune Disorders - Journal Watch Dermatology. Retrieved on 2007-08-06.

 

Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as self, which results in an immune response against its own cells and tissues. Any disease that results from such an aberrant immune response is termed an autoimmune disease. Prominent examples include Coeliac disease, diabetes mellitus type 1 (IDDM), systemic lupus erythematosus (SLE), Sjögren's syndrome, multiple sclerosis (MS), Hashimoto's thyroiditis, Graves' disease, idiopathic thrombocytopenic purpura, and rheumatoid arthritis (RA). See List of autoimmune diseases.

The misconception that an individual's immune system is totally incapable of recognising self antigens is not new. Paul Ehrlich, at the beginning of the twentieth century, proposed the concept of horror autotoxicus, wherein a 'normal' body does not mount an immune response against its own tissues. Thus, any autoimmune response was perceived to be abnormal and postulated to be connected with human disease. Now, it is accepted that autoimmune responses are vital to the development and functioning of vertebrate immune systems, and central to the development of immunological tolerance to self-antigens. The latter concept has been termed natural autoimmunity. Autoimmunity should not be confused with alloimmunity.

Low-level autoimmunity

While a high level of autoimmunity is unhealthy, a low level of autoimmunity may actually be beneficial. First, low-level autoimmunity might aid in the recognition of neoplastic cells by CD8+ T cells, and thus reduce the incidence of cancer.

Second, autoimmunity is likely to have a role in allowing a rapid immune response in the early stages of an infection when the availability of foreign antigens limits the response (i.e., when there are few pathogens present). In their study, Stefanova et al. (2002) injected an anti-MHC Class II antibody into mice expressing a single type of MHC Class II molecule (H-2b) to temporarily prevent CD4+ T cell-MHC interaction. Naive CD4+ T cells (those that have not encountered any antigens before) recovered from these mice 36 hours post-anti-MHC administration showed decreased responsiveness to the antigen pigeon cytochrome C peptide, as determined by Zap-70 phosphorylation, proliferation, and Interleukin-2 production. Thus Stefanova et al. (2002) demonstrated that self-MHC recognition (which, if too strong may contribute to autoimmune disease) maintains the responsiveness of CD4+ T cells when foreign antigens are absent.[1] This idea of autoimmunity is conceptually similar to play-fighting. The play-fighting of young cubs (TCR and self-MHC) may result in a few scratches or scars (low-level-autoimmunity), but is beneficial in the long-term as it primes the young cub for proper fights in the future.



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