Takayasu arteritis is an autoimmune disorder that resembles temporal arteritis histologically but affects young adults, usually 15 to 20 years of age, with a striking female preponderance (9:1). Takayasu arteritis is rare, having an estimated incidence in the United States of 2.6 cases per million compared with 16 cases per million for temporal arteritis. The incidence in the Orient, particularly Japan, is much higher. An increased incidence of certain HLA markers in affected subjects and occurrence in monozygotic twins suggest disease influence by genetic factors. Multiple antigenic triggers, including mycobacteria and streptococci, have been inconclusively suggested to initiate the disease. Chronic Inflammatory Demyelinating Polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder in which there is swelling of nerve roots and destruction of the covering (myelin sheath) over the nerves. This causes weakness, paralysis, and/or impairment in motor function, especially of the arms and legs. Sensory loss may also be present, causing numbness, tingling, and burning sensations. The motor and sensory impairments are usually found on both sides of the body. The severity of CIDP can vary from mild to severe. CIDP can affect any age group, and the onset may begin anytime throughout life.
The course of CIDP may also vary. Some patients may follow a slow steady pattern of symptoms, while other patients have symptoms that flare and remit. The most severe symptoms usually occur after many months of symptoms that come and go. One characteristic that differentiates this disorder from other similar demyelinating diseases is that there is typically no preceding viral infection at least three to four months prior to the onset of the disorder, such as is the case in Guillain-Barré syndrome. Discoid Lupus
Discoid lupus is a benign, distinctive disc-shaped skin eruption. It affects only the skin and not internal organs. Discoid lupus is neither cancerous nor contagious. The initial lesion is a small or moderate-sized, coin-shaped red patch that arises spontaneously or after mild injury or exposure to sunlight. The disease spreads to other areas, but it may be confined to the exposed areas of the body, such as the face, scalp, ears, chest, and arms. The inflammation occurring on the skin may cause destruction of hair follicles and glands and thinning of the skin, with loss of normal color. These lesions may cause permanent scarring and loss of hair.
The cause of the disease is an autoimmunity. Researchers believe that changes in the skin may be caused by an autoimmune reaction of the immune system, skin injury, or an inherited factor. However, there is no clearly defined predisposition to the disease. Rheumatoid Arthritis
Rheumatoid arthritis is one of the more difficult of the autoimmune rheumatic diseases to control and can do the most damage to the joints. The condition generally starts between the ages of 20 and 50, although it can begin at any age.
Rheumatoid arthritis is an autoimmune disease that usually affects many joints, most commonly the small joints of the hands. Inflammation and thickening of the tissue that lines the joints, which is the result of an autoimmune attack on the tissue, causes pain and swelling and, if uncontrolled, may cause destruction of the bones, deformity and, eventually, disability.
The severity of rheumatoid arthritis varies from person to person. In some cases, the disease may be mild, while in others it can be crippling. Its course is unpredictable. It can flare up suddenly, and just as quickly go into remission. Although emotional stress is not a direct cause of rheumatoid arthritis, it can hasten progression of the disease and make it worse. Behçet's Disease
Behçet's disease is a chronic, multisystem autoimmune disease that involves inflammation of blood vessels throughout the body. It is rare, with an occurrence of one case per 20,000 in the United States. The disease is found worldwide, but it is most common in the Eastern Mediterranean countries and in eastern Asia. In those countries, it is a leading cause of blindness; but it is not a leading cause of blindness in the Western world.
Onset of the disease is usually between the ages of 20 and 30, although some patients have been as young as 4 and as old as 70. Behçet's disease affects twice as many men as women. The central nervous system, heart, and intestinal tract may be involved. Behçet's disease is not infectious, contagious, or sexually transmitted.
Because Behçet's disease is rare and symptoms of this disease overlap symptoms of other diseases, it can be very difficult to diagnose. Spontaneous remission is common for patients with Behçet's disease; this can add to the difficulty in diagnosis. Cardiomyopathy
Cardiomyopathy refers to a number of diseases that weaken the heart muscle. Doctors classify cardiomyopathies by the way they affect the anatomy and function of the heart.
In dilated cardiomyopathy, the chambers of the heart are large and the heart won't pump blood effectively. In hypertrophic cardiomyopathy, the heart muscle thickens, reducing the size of the left heart ventricle. Usually, the septum (dividing wall) between the ventricles enlarges more than the outside walls of the heart. The ventricles can't expand well and fill with enough blood. In restrictive cardiomyopathy, the inside of the ventricle walls become stiff and unable to expand. Again, enough blood cannot fill the ventricles.
Restrictive cardiomyopathies are the least common of the cardiomyopathies in the western world. Several diseases may cause the ventricle walls to become rigid, although many times the cause is unknown. Diseases include fibrosis (the formation of tough, fibrous tissue in the heart), collagen-vascular autoimmune diseases (connective tissue disorders of the heart), and amyloidosis (the accumulation of protein fibers on the heart).
Dilated cardiomyopathy may be caused by alcohol or infections. Other conditions associated with it include high blood pressure, pregnancy, and cigarette smoking. If the cause cannot be found, doctors call it "idiopathic." Dilated cardiomyopathy is generally found in middle-aged people, more commonly men.
Hypertrophic cardiomyopathy (also called idiopathic hypertrophic subaortic stenosis) is of unknown cause. However, the tendency to develop this disease is inherited in over half the cases. Often times, family members of a patient with hypertrophic cardiomyopathy are also examined by a cardiologist to determine whether they have signs of the disease. Most patients with this disease who experience symptoms are young adults, but hypertrophic cardiomyopathy is found in all ages and equally in men and women. Guillain-Barré
Guillain-Barré (ghee-yan-bah-ray) syndrome is a disorder in which the body's immune system attacks part of the nervous system, causing a neuromuscular autoimmune disease. In GBS the immune system starts to destroy the myelin sheath that surrounds the axons of many nerve cells, or even the axons themselves (axons are long, thin extensions of the nerve cells that carry nerve signals). This acute autoimmune attack on myelin in the peripheral nervous system (PNS) is associated with IgM antibodies to myelin glycolipid. The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances. GBS can affect anybody. It can strike at any age, and both sexes are equally prone to the disorder. The syndrome is fairly rare, afflicting only about one person in every 100,000. It is not known why GBS strikes some people and not others. Nor do we know exactly what sets the disease in motion.
In autoimmune diseases in which the nerve cells' myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to the brain's commands, commands that must be carried through the nerve network. Reiter's Syndrome
Reiter's syndrome was first described by H. Reiter in 1916. This disease consists of three manifestations: arthritis, urethritis, and conjunctivitis. Urethritis usually appears first. A patient who has all three of these manifestations is said to have the complete syndrome. This disease later was shown to have an infectious cause, with chlamydia being the organism most frequently associated with Reiter's syndrome. Patients having an initiating infectious episode and the subsequent arthritis are said to have an incomplete syndrome. By the current definition, as proposed in 1981 by the American Arthritis Association, Reiter's syndrome is a type of arthritis that follows urethritis, cervicitis, or dysentery. Other possible effects are inflammatory eye lesions, oral ulcers, inflammation of the skin covering the end of the penis, and scaling lesions of the palms, soles, penis, area around the nails, and occasionally other areas. Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a disease of inflammation that results in scarring, or fibrosis, of the lungs. In time, this fibrosis can build up to the point where the lungs are unable to provide oxygen to the tissues of the body.
Doctors use the word "idiopathic" (from the Greek "idio" meaning "peculiar" or "unusual" and "pathy" meaning "illness") to describe the disease, because the cause of IPF is unknown. Currently, researchers believe that IPF may result from either an autoimmune disorder, a condition in which the body's immune system attacks its own tissues, or the aftereffects of an infection, most likely a virus.
Whatever the trigger is for IPF, it appears to set off a series of events in which the inflammation and immune activity in the lungs—and, eventually, the fibrosis processes, too—become uncontrollable. In a few cases, heredity appears to play a part, possibly making some individuals more likely than others to get IPF. Juvenile Arthritis
Juvenile chronic arthritis (JCA), by definition, is the onset of a chronic arthritic disease in a child under the age of 16 years; and symptoms last longer than three months. It can usually be divided into four types and may be referred to as Still's disease. No clear evidence of a genetic predisposition for JCA exists, except in the case of familial spondylarthropathies, which may be associated with HLA-B27. However, a genetic predisposition may be considered with autoimmune diseases in general.
Juvenile rheumatoid arthritis (JRA) is a rheumatic autoimmune disease characterized by chronic inflammation of the synovial tissue found in joints. It affects between 65,000 to 70,000 children in the United States and comprises about ten percent of JCA. The disease may develop at any age during childhood, and girls are more often affected than boys. There are several subgroups of JRA. JRA has been described as a childhood rheumatic illness that has many characteristics of adult onset autoimmune diseases, such as rheumatoid arthritis and lupus. Stiff-Man Syndrome
Stiff-man syndrome, which may also be referred to as Moersch-Woltmann syndrome, is a rare, severe autoimmune neurologic disease involving the central nervous system. The disease is characterized by a progressive rigidity or stiffness of the body musculature. The stiffness is caused by a diffuse hypertonia (excess muscular tension or pressure) that involves the voluntary muscles of the neck, shoulders, trunk, arms and legs. Painful muscle spasms may also be present. Unlike many autoimmune diseases which have a higher incidence in women, stiff-man syndrome is found more frequently in men. Approximately 70 percent of those afflicted are men.
While the exact cause of stiff-man syndrome is not clear, autoimmunity is thought to be a factor. Genetic factors have not been established, although familial occurrences of the disease and/or other autoimmune disorders appear to point in this direction. The frequent occurrence of insulin-dependent diabetes mellitus (IDDM), also referred to as type 1 diabetes, in stiff-man syndrome supports this hypothesis. Uveitis
The eye is shaped much like a tennis ball, hollow inside with three different layers of tissue surrounding a central cavity. The outermost layer is the sclera (white coat of the eye), and the innermost layer is the retina (image-fathering tissue in the back of the eye much like the film in a camera). The middle layer between the sclera and retina is called the uvea, from the Greek word "uva" meaning grape. In the laboratory, it looks much like a "peeled grape." When the uvea becomes inflamed, the condition is called uveitis (pronounced U'VE-I-TIS).
The uvea contains many of the blood vessels which nourish the eye. Inflammation of the uvea can affect the cornea, the retina, the sclera, and other vital parts of the eye. Since the uvea borders many important parts of the eye, inflammation of this layer may be sight- threatening and more serious than the more common inflammations of the outside layers of the eye.
Uveitis has many different causes. It may result from a virus (such as shingles, mumps, or herpes), a fungus (such as histoplasmosis), or a parasite (such as toxoplasmosis). In most cases, the cause remains unknown.
Uveitis can also be related to autoimmunity or come as a consequence of injury to the eye. Inflammation in one eye can result from a severe injury to the opposite eye (sympathetic uveitis). Primary Agammaglobulinemia
Primary agammaglobulinemia is an immune disorder related to antibody deficiency (hypogammaglobulinemia) and is manifested in a variety of immune deficiency disorders in which the immune system is compromised. This group of immune deficiencies may be the consequence of an inherited condition, an impaired immune system from known or unknown causes, a relation to autoimmune diseases, or a malignancy.
Immunoglobulin deficiencies may be referred to by many different names, as there are several variables within the separate but related immune disorders; and there are also many subgroups. Antibody deficiency, immunoglobulin deficiency, and gamma globulin deficiency are all synonyms for hypogammaglobulinemia. Essential Mixed Cryoglobulinemia
Essential mixed cryoglobulinemia is a rare autoimmune disorder that may involve the blood and various other tissues and organs. The major symptoms include unusual response to cold, skin abnormalities, blood disorders and generalized weakness. There may also be joint pain, inflamed blood vessels, and kidney problems.
The exact cause of essential mixed cryoglobulinemia is not known. However, it is considered to be an autoimmune disorder. (Autoimmune disorders are caused when the body's immune system, which is meant to defend the body against bacteria, viruses, and any other foreign product, malfunctions and produces antibodies against healthy tissue, cells and organs.) In this condition, the immune system appears to be triggered by cold temperatures. Cryoglobulins are proteins in the blood that become apparent when the blood is cooled. These cryoglobulins can affect many different bodily systems, causing pain and dysfunction. It is important to note that a link between cryoglobulinemia and hepatitis B infection is possible. Insulin-dependent Diabetes
Insulin-dependent diabetes melitis (IDDM) is a chronic autoimmune endocrine disease that usually begins in childhood or young adulthood and is more common in females than in males. It is not the most common form of diabetes. IDDM accounts for only 5 percent or less of diabetes in this country. Often, though, IDDM has a much greater impact on a person's life than the more common adult-onset form of diabetes, known as non-insulin-dependent diabetes (NIDDM). Insulin-dependent diabetes may be referred to as type I diabetes, juvenile diabetes, and diabetes mellitus.
No one knows exactly what causes IDDM. What is clear is that the body's own immune or disease-fighting system for some reason turns against the body's own tissues (autoimmunity).
Research shows that most, if not all, people with IDDM may inherit traits that put them at risk for IDDM. However, not everyone who inherits these traits develops IDDM. One or more factors in the environment are believed to trigger the immune system to destroy the insulin-producing cells. In some cases, the trigger may be a viral infection. Scientists have, in a few cases, been able to link the onset of diabetes with a virus. In most cases, however, the trigger for diabetes is unknown. Raynaud's Phenomenon
Raynaud's phenomenon (RP) is considered to be a common condition. It occurs in 1-10 percent of the general population and in 25 percent of young women. Raynaud's patients usually report "cold fingers" accompanied by color changes of the skin (white, blue or red). Most persons with RP note cold-induced numbness (pins and needles sensation) of the fingers and toes and occasional discomfort (achy dull pain) with a sense of hand clumsiness. It usually occurs as repeated attacks lasting an average of 15-20 minutes following rewarming. Although cold is the main trigger of an attack, rapidly changing temperature can be a prime cause of attack even in a warm environment. Blood flow studies have shown that during a Raynaud's attack there is no blood flowing to the arteries or the small capillaries of the fingers. This is probably caused by an exaggeration of a normal mechanism which maintains one's central body temperature by shunting blood away from the hands and feet when we are cold. The cause of this exaggerated response is not totally understood but likely is secondary to a failure of the normal chemical and neural control of the dynamic flow of blood in one's fingers.
Raynaud's phenomenon either can occur in normal individuals or can be associated with a disease process. Often, RP is an initial symptom of an underlying rheumatic disease. Approximately 40 percent of patients with systemic lupus erythematosus (SLE or lupus), 50 percent of patients with dermatomyositis, and 90 percent of patients with scleroderma have Raynaud's phenomenon. Normal individuals who have RP usually have mild, less frequent attacks compared to patients with scleroderma, who frequently develop painful ischemic ulcers or loss of their finger tips. Most patients with SLE also have mild RP unless their RP is associated with inflammation of the blood vessel wall (vasculitis). Most studies show that SLE patients with RP are not different from other SLE patients; however, some reports have suggested that those with RP have milder SLE but are more likely to develop bone complications (aseptic necrosis of bone) while on corticosteroid. IgA Nephropathy
Primary IgA nephropathy is an autoimmune kidney disease marked by IgA glomerulonephritis due to the glomerular immune deposit formation in the kidney.
IgA nephropathy usually occurs in adolescents or young adults between the ages of 15 and 35. Males are affected two or three times more often than females. It occurs significantly more often in native Americans than in any other ethnic group tested. It is more prevalent in Caucasians than in African Americans, and it is one of the leading causes of acute nephritis in young people in the United States, Europe, and Japan. One study of young men in the military showed an annual occurrence of 94 cases of IgA nephropathy out of 100,000 male inductees. Hashimoto's Thyroiditis
Hashimoto's thyroiditis, also referred to as autoimmune thyroiditis and chronic lymphocytic thyroiditis, is a chronic inflammatory glandular autoimmune disease. An autoimmune reaction to proteins in the thyroid is the underlying cause of Hashimoto's thyroiditis. There is evidence of a genetic predisposition in the development of Hashimoto's thyroiditis. It is not uncommon for persons with autoimmune thyroid disease to have other coinciding autoimmune disorders. Approximately 25 percent of patients with Hashimoto's may develop pernicious anemia, diabetes, adrenal insufficiency, or other autoimmune diseases.
Another thyroid related autoimmune disease is Graves' disease. The disease process can eventually destroy the thyroid, resulting in hypothyroidism; but usually the person has an enlarged thyroid gland with normal or mildly abnormal thyroid function tests. Persons with Hashimoto's thyroiditis have autoantibodies against several different proteins in their thyroid gland. A family history of thyroid disease is not unusual.
Although men and women of any age can develop this disease, it is most common in women between the ages of 30 or 50 where the ratio of female to male is fifty to one. Polyglandular Syndromes
Polyglandular syndromes involve a group of symptoms and signs of disordered function related to one another by some anatomic, physiologic, or biochemical peculiarity affecting many glands. These are referred to as associated endocrinopathies, that is, diseases resulting from disorder of an endocrine gland or glands. Three syndromes of associated endocrinopathies have been defined as the polyglandular syndromes. Type I Syndrome
This syndrome involves an infection of the skin or mucous membrane with any special species of Candida, chiefly Candida albicans, and associated endocrinopathies (for example, Addison's disease and hypoparathyroidism). These diseases begin in early childhood. Patients initially develop candidiasis and hypoparathyroidism, but more than half of the patients also develop Addison's disease.
Associated disorders include ovarian failure, alopecia, malabsorption, and chronic hepatitis.
Patients have organ-specific autoantibodies and poorly defined defects in cell-mediated immunity. The specific origin of this disease is unknown, and there is no HLA association. Pemphigus Vulgaris
Pemphigus is a group of chronic autoimmune skin diseases characterized by blister formations in the outer layer of the skin and the mucous membranes. Pemphigus vulgaris begins with blister formations (bullae) occurring in the mouth and on the scalp. The blisters are soft and are easily broken. The blistering can also affect the esophagus, rectum, nose or the lining of the eyelids. These bullae heal without scarring.
Pemphigus vulgaris predominately occurs in middle-aged patients of Jewish or Mediterranean descent. Pemphigus vulgaris has been associated with other autoimmune diseases, such as myasthenia gravis and systemic lupus.
Diagnosis of an autoimmune bullous disease should be suspect when there is no clear history of exposure to a drug or a contact allergen or when other studies for infectious origins, such as herpes or impetigo, are negative. To differentiate these diseases, a careful history and physical examination are important. A skin biopsy is often helpful. Celiac Sprue-Dermatitis
GSE (Gluten Sensitive Enteropathy) includes celiac sprue (CS), dermatitis herpetiformis (DH), and transient gluten intolerance. In these hereditary immune system disorders, protein fractions in wheat, rye, oats and barley set off a chain of events which lead to tissue damage. Celiac sprue involves destruction of the lining of the small intestine and includes a skin disorder where immune deposits trigger itchy, blistering skin eruptions. Transient gluten intolerance is a poorly understood condition resembling celiac sprue which appears in children under the age of 2 years.
A wide range of symptoms may be present depending on the severity of damage to the lining of the small intestine. Usually, those with celiac sprue have the most severe damage. Common symptoms include: diarrhea, bloating, weight loss, anemia, chronic fatigue, weakness, bone pain, and muscle cramps. Symptoms may also include constipation, or constipation alternating with diarrhea. Overweight persons may also have undiagnosed celiac sprue. Children may show growth failure, have diarrhea, projectile vomiting and a bloating abdomen. Itchy, blistering lesions of dermatitis herpetiformis (DH) can appear almost anywhere on the skin, but they are most common on the elbows, knees and buttocks. Both sides of the body are usually involved. Fibromyalgia-Fibromyositis
Fibromyalgia has only recently gained recognition as a distinct clinical entity, with the establishment of diagnostic criteria in 1990. This common disorder, which superficially resembles other disorders such as rheumatoid arthritis, has often been dismissed as an imaginary or psychiatric problem or a form of malingering. Patients commonly complain of widespread pain and tenderness, fatigue, and exhaustion after minimal effort. Often they feel tired after sleep, and sleep laboratory tests are usually normal. A diagnosis can be confirmed by a characteristic pattern of specific points on the body that are exquisitely tender to palpitation. Fibromyalgia has not been identified as an autoimmune disorder itself. However, it is well known that it often accompanies other endocrine and rheumatic autoimmune disorders.
Fibromyalgia is a common and perplexing clinical condition. In the United States, 3 to 6 million people may be afflicted with its symptoms; and an estimated 15 percent to 20 percent of patients (90 percent of them women) seen in rheumatology practice have fibromyalgia.
Fibromyalgia is characterized by the constant presence of widespread pain so severe that it is often incapacitating; it is also characterized by the total absence of any definable pathophysiologic or laboratory abnormality, even under that most intense scrutiny.
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