Autoimmunity and A uto-immune diseases By –Yashi jain
INTRODUCTION Autoimmunity is the system of immune responses of an organism against its own healthy cells and tissues Any disease that results from such an aberrant immune response is termed as autoimmune disease Auto immune disease affect 5 percent of North Americans and Europeans, a majority of them being women
TYPES OF AUTO-IMMUNE DISEASES- Organ-specific diseases – are one in which an immune response is directed towards antigens in a single organ Systemic diseases – are those which affect multiple organs or glands
CHRONIC THYROIDITIS Also called Hashimoto’s thyroiditis is a disease of thyroid mainly affects the women of age group 30-50 years Eventual atrophy of the thyroid gland results in hypothyroidism Immune targets in this are thyroid peroxidase, thyroglobulin and second colloid antigen It affect 0.1-5 percent adults
SYMPTOMS Dry skin Puffy face Brittle hair and nails Feeling of being cold
TREATMENT Detection of high levels of antibodies against thyroglobulin and thyroid peroxidase in the patient’s blood
PERNICIOUS ANAEMIA In this the human body does not make enough red blood cells due to lack of vitamin B 12 in the body Usually occurs in individuals who have lost the ability to absorb vitamin B 12 from food It is named pernicious anaemia because it was fatal during ancient times when its cause had not been discovered
SYMPTOMS AND TREATMENT Weakness, loss of appetite, pallor, fatigue and weight loss It is treated with injections of vitamin B 12
AUTOIMMUNE HAEMOLYTIC ANAEMIA It is characterized by the production of autoantibodies that attack red blood cells as if they were foreign to the antibody Divided into two groups depending on their physical properties- warm antibodies and cold agglutinins Warm antibodies react with red blood cells under warm conditions(37°C) while cold agglutinins at below 37ºC
TREATMENT In patient’s with advanced auto immune heamolytic anaemia, a corticosteroid such as prednisone is usually recommended If the patient does not respond to this drug or it causes side affects then splenectomy is often the best option
DRUG- INDUCED HAEMOLYTIC ANAEMIA It occurs when a drug causes the body’s immune system to react against red blood cells Drugs that induces this disease are cephalosporins, quinidine, levodopa, apart from penicillin and methyldopa
THROMBOCYTOPENIC PURPURA It is a life threatening disorder that was first described by Moschcowitz in 1924 It results from the destruction of platelets by autoantibodies For untreated patients, the mortality rate is approximately 95 percent
SYMPTOMS AND TREATMENT Petechia(skin spots due to ruptured blood vessels ) Gingivitis(bleeding gums) Bleeding in the genitourinary tracts In this splenectomy is recommended for treatment
GOODPASTURE SYNDROME It is named after Goodpasture, who first described this disorder in 1919 He reported this as a case of pulmonary haemorrhage during in influenza epidemic It affect lungs and kidneys This disease affects all age groups
SYMPTOMS Kidney and pulmonary damage Pulmonary haemorrhage Heamolysis Death often causes within several months of initiation
INSULIN DEPENDANT DIABETES MELLITUS Also called type 1 diabetes caused by the immunological destruction of the pancreas Immune system targets insulin, hsp60 and glutamic acid decarboxylase of the islets of Langerhans It affects about 1 person out of every 500 in a population
SYMPTOMS AND TREATMENT Patients of type 1 diabetes may lose as much as 6.8 kg weight within two weeks Cardiovascular diseases Kidney problems Treatment is patients require daily insulin injections
GRAVES’ DISEASE It is named after Robert Graves, the physician who first described this form of hyperthyroidism It is often associated with the inflammation, swelling and bulging of the eyes It results from the overproduction of thyroid hormones(thyroxine)
SYMPTOMS Fatigue Nervousness Increased sweating Palpitation Heat intolerance Weight loss
TREATMENT Anti- thyroid drugs such as propylthiouracil or methimazole Surgical or radioactive ablation of the gland is also effective A Graves’ susceptibility gene has recently been localized on chromosome 20
MYASTHENIA GRAVIS This name literally means ‘grave muscle weakness in Greek It’s a chronic disease results from faulty neuromuscular transmission In this, antibodies are produced against the acetylcholine receptors at the neuromuscular junction These Abs destroy the receptors, preventing muscle contraction
CONTINUED… It may be transmitted to the foetus from the mother through the passage of autoantibodies across the placenta The female to male ratio is 3:2
SYMPTOMS Difficulty in chewing and swallowing Difficulty in breathing Eventually death from respiratory failure
SYSTEMIC LUPUS ERYTHEMATOSUS Lupus is a condition characterized by chronic inflammation of body tissues It can be inferred from the name that is it affects many parts of the body The ratio of female to male patients is 10:1 The characteristic feature of SLE is called Malar rash that is butterfly rash on cheeks
CONTINUED… Patients of SLE have 10-15 autoantibodies of different specificities including that against DNA, RNA, RBCs, platelets, thrombin, mitochondria, lysosomes and ribosomes
SYMPTOMS AND TREATMANT Skin rashes Malaise Fever Lethargy Weight loss Non- steroidal anti-inflammatory drugs called aspirin while steroidal called cortisone are useful in treatment
RHEUMATOID ARTHRITIS It is a chronic, inflammatory joint disease affecting mainly women It affects the smaller joints, such as hands, knees or ankles Individuals faces difficulty in carrying out normal activities like standing, walking etc About 2 million people in USA are affected
SYMPTOMS Weight loss Malaise Fever Fatigue Weakness Respiratory systems are also affected
MULTIPLE SCLEROSIS It is an autoimmune neuromuscular disease that affects the central nervous system The name multiple sclerosis refers to the occurrence of several scleroses(lesions) in the white matter of the CNS It affects the neurons in the brain and spinal cord
CONTINUED… The immune system targets multiple myelin antigens, including myelin basic protein, proteolipid protein and myelin oligodendrocyte glycoprotein Usually people between the age of 20-40 are affected
SYMPTOMS Motor weakness Ataxia(uncontrolled movement of limbs) Impaired vision Urinary bladder dysfunction Mental aberration
SCLERODERMA It is a rare autoimmune disease that affects mainly women between the age of 35-54 The name sclero means hard and derma means skin The three major forms are- diffuse type and limited type(systemic forms means affecting multiple organs) whereas linear form is localized to the skin
CONTINUED… Individual of this disease develop CREST syndrome. It is an acronym for five symptoms- Calcinosis-calcium deposits in the skin Reynauld’s phenomenon- abnormal blood flow in response to stress or cold Esophageal dysfunction- involving difficulty in swallowing
CONTINUED… Scelerodactyl- tightening or scaling of the skin Telangiectasia- formation of red spots on the face, lips, palms and forearms
SYMPTOMS Gradual lightening of the skin(hands, feet and face) Systemic form affects multiple organs like kidney, heart, lungs etc
GUILLIAN-BARRE SYNDROME It is named after two French physicians Guillian and Barre who, in 1916 reported the nature of this paralytic illness It commonly occurs after an infectious disease(like measles) or after a vaccination(like influenza) The cause of this disease involves antibody and T-cell mediated damage to nerve tissues
CONTINUED… Most patients recover normal function in 6-10 months It is also known as acute idiopathic polyneuritis
SYMPTOMS AND TREATMENT Fever Malaise Progressive weakness leading to paralysis Plasmapheresis or plasma exchange therapy which exchanges the offending antibodies and immune complex containing plasma with normal or frozen serum, is beneficial