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Comparison of accepted autoimmune diseases
Disease name Aetiology Pathophysiology Epidemiology Tissues affected Treatment Prognosis
Acute disseminated encephalomyelitis[1] Unknown Disturbance of the blood-brain barrier and IL-1β, Il-2, IL-4, IL-5, IL-6, IL-8, IL-10, IFN-γ, TNF-α, and MIP-1β appear to be involved.[1] Children tend to be most often affected. Incidence is <1/100,000. Brain and spinal cord. Corticosteroids and intravenous immunoglobulin. Generally favourable; 1.5% mortality rate.
Antiphospholipid syndrome[2] Unknown Antiphospholipid antibody-mediated assault on phospholipids that make up blood cells.[3] Female predominance, more common in Hispanics and African Americans, it is more common in young-middle aged patients. Blood and foetus Anticoagulants, hydroxychloroquine, cyclophosphamide, prednisone, rituximab and intravenous immunoglobulin.[4][5][6][7][8][9][10][11][12][13][14][15][16] Highly fatal, due to venous thromboembolisms.
Atopic dermatitis Unknown, genes and environment contribute. People with a family or personal history of atopic dermatitis, asthma, food allergies and hay fever are at a heightened risk for the condition. Predominantly TH2-mediated pathology.[17][18][19][20] 15-30% of children are affected. Only 1-3% of adults are affected, although.[21][22] Skin Lukewarm baths, moisturisers, corticosteroids, tacrolimus, ciclosporin, azathioprine, methotrexate, rituximab, infliximab and pimecrolimus.[22][23][24][25][26][27] Generally favourable, although relapses are common.
Autoimmune haemolytic anaemia[28] Unknown; infectious and malignant expected. Immunoglobulin G and Immunoglobulin M involved. Annual incidence is about 1/100,000.[29] Red blood cells. Corticosteroid, azathioprine, cyclophosphamide, ciclosporin, mycophenolate mofetil, rituximab and immunoglobulins.[29] Generally favourable. 10% fatality rate overall.
Autoimmune hepatitis Unknown; genetics and environment likely play a role.[30] T-cell mediated destruction of hepatocytes. Frequency is about 0.015-1.2/100,000.[30][31] Liver Corticosteroids, azathioprine, mycophenolate mofetil, tacrolimus and ciclosporin.[32][33] Generally unfavourable; cirrhosis is present in 30% of cases at the time of diagnosis.[30]
Autoimmune inner ear disease[34] Unknown; infectious agents believed to contribute. Unknown Most commonly affects people between the ages of 20-50, especially females with other autoimmune conditions.[34] Inner ear Corticosteroids, azathioprine, mycophenolate mofetil, cyclophosphamide and methotrexate.[35] Unknown
Autoimmune lymphoproliferative syndrome
[36][37][38][39][40]		 Genetic; mutations in the Fas signalling pathway. Mutations in the fas signalling pathway allows for the accumulation of malfunctioning lymphocytes in the tissues. Most commonly presents in childhood and runs in over 300 families worldwide. Haematopoietic, liver and lymphatics (including spleen). Corticosteroids, cyclophosphamide, ciclosporin, mycophenolate mofetil and immunoglobulins. Favourable. Mortality usually occurs as a result of cytopenias or malignancies.
Autoimmune pancreatitis[41][42] Unknown Type I is mediated by immunoglobulin G produced by plasma cells and increased production of T cells. Type II is mediated by granulocytes. Type I is more common in Asia; type II in Europe/North America. Type I usually occurs in one's 60s or 70s. Type I is 2.85 times more common in men than in women. Type II has no such sexual predilection. Type I has a prevalence of 0.82/100,000 in Japan. Pancreas. Corticosteroids (both types); type I with azathioprine, mercaptopurine, mycophenolate mofetil and rituximab. Favourable for type II; unfavourable for type I. Type I has a high rate of re-occurrence.
Autoimmune polyglandular syndrome[43] Genetic Inflammation, destroying the various exocrine and endocrine glands of the body. Type I: <1/100,000. Type II: 5/100,000. Type I is more common in Iranian Jews (prevalence: 1:600-9,000) and Finns (prevalence: 1:25,000) and usually first presents in children, it has no sexual predilection. Type II occurs predominantly in women, and is more frequent than type I with an incidence of 5/100,000. Various endocrine glands including the pancreas, parathyroid, gonads, pituitary and thyroid; and the exocrine glands, the parotids. Supportive care with hormone replacement therapy. Favourable. With hormone replacement therapy to help with the various symptoms.
Autoimmune thrombocytopenic purpura
[44][45][46][47][48]		 Unknown Immunoglobulin G-mediated platelet lysis. It is more common in males than females when it comes to children, whereas in adults it is more common in females than males, the peak age for it is 1-6 years and 30-40 years. Brain and bone marrow. Corticosteroids, thrombopoietin receptor agonists (e.g. eltrombopag, romiplostim), platelet transfusions, intravenous immunoglobulin G, azathioprine, cyclophosphamide, danazol (mostly postmenopausal women) and rituximab. Haemorrhage is the major cause of death. About 0.9% of kids with it die at presentation; overall >80% of patients will respond favourably to treatment.
Behcet's disease[49] Unknown; genes and infectious agents are believed to contribute. T cells (especially Th1), NK cells and neutrophils are all involved, as are IL-2, IL-8, IL-12, IL-21, TNF-α and IFN-γ.[50] Turks and Asians appear to be a high-risk group. Turks have an incidence of 420/100,000; Asians 13.5-22/100,000. North American and European frequency is estimated to be 0.2-7/100,000. Mean age is between 20 and 40; most commonly affects males (3-6 times more frequently than females). Joints, eyes and skin are most commonly affected; although, the central nervous system, cardiovascular system, GI tract and genitourinary system may also be affected.[51][52][53] Corticosteroids, azathioprine, cyclophosphamide, methotrexate, chlorambucil, thalidomide, colchicine, ciclosporin, infliximab and adalimumab.[54][55] 5% die; more are permanently disabled by either eye or CNS involvement.
Coeliac disease
[56][57][58]
[59]		 Genes contribute, including HLA-DQ2 and HLA-DQ8.[60] Immunoglobulin A and T cell-mediated response to gluten.[61][62][63] Incidence is fairly constant across the races with a prevalence of about 0.15-1%.[64] Intestines Corticosteroids and gluten avoidance.[65][66][67][68][69][70] Excellent, if gluten is avoided. Increased incidence of cavities, type I diabetes mellitus,[71] inefficacy of hepatitis B vaccination,[72] non-Hodgkin's lymphoma, sepsis, juvenile rheumatoid arthritis and depression.[73][74]
Cold agglutinin disease[75][76][77] Idiophathic or infection/cancer. Unknown Annual incidence: 1/300,000. Blood and liver. Prednisone, cyclophosphamide, rituximab and interferon alfa-2b. Generally favourable. Few have complete remissions, however.
Crohn disease Unknown. Genes, diet, microbial and environment (especially cigarette smoking, contraception and NSAIDs) contribute.[78][79] Th1-mediated chronic assault on the GI tract, TNF-alpha is believed to be involved, along with other cytokine messengers. Granulomas form in the GIT.[80][81] Annual incidence is about 10-150/100,000 in the Europe, with a prevalence of 322/100,000. The prevalence is about 320-511/100,000 in the US, and the annual incidence is about 43/100,000 for children and 201/100,000 for adults. In Asia the annual incidence is about 0.5-4.2/100,000, whereas in South Africa the prevalence is 0.3-2.6/100,000 and in Latin America its prevalence is about 0-0.03/100,000. It is more common in Ashkenazi Jews.[82] GI tract Corticosteroids, aminosalicylates, azathioprine, mercaptopurine, methotrexate, tacrolimus, infliximab, adalimumab and certolizumab.[83][84][85][86][87][88][89][90][91][92] Generally favourable, although it causes significant disability in 25% of patients during the year when the diagnosis is made. Increased risk of lung and colorectal cancers, COPD, liver, genitourinary and GI disease.[93]
Dermatomyositis Unknown, genes are believed to contribute. Humoural assault on the skin. TNF-alfa appears to contribute too.[94][95][96][97][98] Annual incidence is estimated to be 9.63/1,000,000. Peak ages for onset are 5-10 years and 50 years. Lung, skin, joints, skeletal muscle and heart. Glucocorticoid,azathioprine, methotrexate, rituximab, mycophenolate mofetil, tacrolimus, ciclosporin, hydroxychloroquine,cyclophosphamide and intravenous immunoglobulins.[99][100][101][102] Generally unfavourable, only 20-40% of affected individuals achieve a remission and 5% die. 60-80% of persons suffer chronic disease.[103] Malignancies are more common in persons with dermatomyositis than in the general population.[104]
Diabetes mellitus type I Genetic and environmental factors involved.[105] 2-3% of children who's mother has the disease will develop it and 5-6% of children who's father has it will develop it.[106] 85% have antibody-mediated destruction of the islet cells.[106] Fewer than 1% of Chinese/Japanese individuals with diabetes mellitus have type I. Whereas 20% of Scandinavians with diabetes mellitus have type II. In the US the annual incidence is 15-20/100,000.[106] Pancreas Regular insulin injections and dietary changes. Generally positive; irreversible and deadly if ignored. Increased risk of various malignancies has been observed, particularly bladder, gastric and pancreatic cancer.[107][108][109]
IgA nephropathy
[110][111][112][113][114][115]
[116]		 Unknown Immunoglobulin A-mediated reaction.[117] It is significantly more common in Asia and Europe than in North America. The annual incidence is about 0.5-1/100,000 in the US, whereas the incidence in Japan the incidence is about 5-10/100,000. Kidney. Symptomatic (with ACE inhibitors), corticosteroids,cyclophosphamide, azathioprine, mycophenolate mofetil, anticoagulants, omega-3 fatty acids, leflunomide and ciclosporin.[118] The 10-year progression rate to end-stage kidney disease is about 15%, whereas the 20-year progression rate is 20%.
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