Glossary

Asthma

Asthma is a condition in which your airways narrow and swell and produce extra mucus. This can make breathing difficult and trigger coughing, wheezing and shortness of breath.

For some people, asthma is a minor nuisance. For others, it can be a major problem that interferes with daily activities and may lead to a life-threatening asthma attack.

Asthma can’t be cured, but its symptoms can be controlled. Because asthma often changes over time, it’s important that you work with your doctor to track your signs and symptoms and adjust treatment as needed.

Autoimmune Angioedema

Angioedema may be due to hereditary forms of Cl-Inh deficiency, but recently an autoimmune form of angioedema has been described in which the mechanism is novel. While the peripheral blood monocytes of patients with autoimmune angioedema produce a normal, functionally active, 105 KD Cl-Inh in normal quantities, the Cl-Inh isolated from the patient’s plasma exists in a dysfunctional lower molecular weight (96 KD) performance. Rather than bind and biologically inactivate the enzyme, a relatively common phenomenon in autoimmune disease, the autoimmune angioedema cleave the Cl-Inh molecule. The following sequence of events is proposed: structural and functionally normal Cl-Inh is synthesised and secreted, this secreted inhibitor is complexed by autoantibody and following enzyme interaction, denatured 96 KD Cl-Inh is proposed. This process depletes the pool of normal, functional Cl-Inh to critical levels and predisposes patients to episodes of oedema.

Autoimmune Aplastic Anemia

Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. The condition, as the name indicates, involves both aplasia and anemia. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets, termed pancytopenia.

Autoimmune Dysautonomia

Dysautonomia is a broad term that describes any disease or malfunction of the Autonomic Nervous System (ANS). The autonomic nervous system controls a number of functions in the body, such as heart rate, blood pressure, digestive tract peristalsis, sweating, amongst others. Dysfunction of the ANS can involve any of these functions.

A number of conditions are forms of dysautonomia: postural orthostatic tachycardia syndrome, inappropriate sinus tachycardia,vasovagal syncope, pure autonomic failure, neurocardiogenic syncope, neurally mediated hypotension, orthostatic hypotension, orthostatic hypertension, autonomic instability and a number of lesser-known disorders such as cerebral salt-wasting syndrome. Dysautonomia may occur as the result of other diseases, such as diabetes mellitus, multiple system atrophy, Guillain-Barré syndrome and a number of other conditions that may affect the nervous system.

Autoimmune Hepatitis

Autoimmune Hepatitis is a disease of the liver that occurs when the body’s immune system attacks cells of the liver. Anomalous presentation of human leukocyte antigen (HLA) class II on the surface of hepatocytes, possibly due to genetic predisposition or acute liver infection, causes a cell-mediated immune response against the body’s own liver, resulting in autoimmune hepatitis. This abnormal immune response results in inflammation of the liver, which can lead to further complications, including cirrhosis.

Autoimmune hepatitis has an incidence of 1-2 per 100,000 per year, and a prevalence of 10-20/100,000. As with most other autoimmune diseases, it affects women much more often than men (70%). Liver enzymes are elevated, as may be bilirubin.

Autoimmune Hyperlipidemia

In the antilipoprotein type of autoimmune hyperlipidemia (AIH), the immunoglobulins (Ig) are bound to lipoproteins by their antibody site and circulate as immune Ig-Lp complexes. In the earlier studies, the specific antibody activities were demonstrated in vitro by specific but rather sophisticated methods which were not suitable for the screening of antilipoprotein AIH in large populations. In the Ig-Lp test described here, the immunoglobulins bound to the low density lipoproteins (Ig-Lp) are detected by floating the complexes at D 1.10 in the ultracentrifuge in a physiological saline sucrose density gradient; delipidating them by ether in the presence of 0.2 M urea, and assaying the protein by radial immunodiffusion and laser immunonephelometry with antisera specific for IgG, IgA, IgM, low density lipoproteins and albumin. Radial immunodioffusion and immunonephelometry gave similar results. This Ig-Lp test was positive in 5 myelomas associated with hyperlipidemia, which were previously classified as AIH with specific methods. And the test was specific for the Ig type of the monoclonal antibody involved in each case (3 IgA, 1 IgG and 1 IgM). It was negative in 6 normolipidemic myelomas and also in 40 sera from healthy blood donors and one normal serum taken 4 hours after a fat meal. Although the Ig-Lp test is not specific for antilipoprotein antibodies, the results of this study allow to use if for the screening for AIH.

Autoimmune Immunodeficiency

Immunodeficiency disorders — also called primary immune disorders or primary immunodeficiency — weaken the immune system, allowing repeated infections and other health problems to occur more easily.

Many people with primary immunodeficiency are born missing some of the body’s immune defenses, which leaves them more susceptible to germs that can cause infections. Some forms of primary immunodeficiency are so mild they may go unnoticed for years. Other types of primary immunodeficiency are severe enough that they are discovered almost as soon as an affected baby is born.

Treatments can boost the immune system for many types of primary immunodeficiency disorders.

Autoimmune Inner Ear Disease

Autoimmune inner ear disease is a suspected autoimmune disease characterized by rapidly progressive bilateral sensorineural hearing loss. It occurs when the body’s immune systemattacks cells in the inner ear that are mistaken for a virus or bacteria.

Autoimmune inner ear disease (AIED) is the name used to describe the variety of disorders in which the ear is the sole target of an inappropriate attack by the immune system. This disorder differs from other vestibular disorders because medical treatment can succeed when given early and aggressively. An early diagnosis is important because treatment can not only stop the disease progression but in some cases can reverse the damage.

The standard treatments for autoimmune reactions are drugs that reduce immune function (immuno-suppression), and they have body-wide effects. Their use requires diligence on the part of the patient and the provider. They include steroids, chemotherapy agents, anti-transplant rejection drugs, and the newer anti-tumor, necrosis-factor drugs. The physician must be both knowledgeable and experienced with these drugs and their side effects, and the patient must follow the physician’s instructions closely. Because a rheumatologist has extensive training and experience in these treatments, nearly all otolaryngologists and neurotologists have them manage the drug treatment.

Autoimmune Myocarditis

Myocarditis is an inflammatory disorder of the myocardium with necrosis of the cardiomyocytes and associated inflammatory infiltrate. It is usually caused by viruses which have tropism for myocardiocytes such as adenovirus and coxsackie B viruses. These viruses are therefore frequently associated with viral myocarditis. Myocarditis generally results in a decrease in myocardial function, with accompanying cardiac enlargement and an increase in the end-diastolic volume due to increased preload. Normally, the heart compensates for dilation with an increase in contractility, but because of inflammation and muscle damage, the heart is unable to respond to the increase in volume.

Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment withcorticosteroids, particularly prednisone. It is increasingly regarded as a form of hyper-IgG4 disease.

Autoimmune Thyroid Disease

Autoimmune Thyroiditis, or Chronic Autoimmune Thyroiditis, is a disease in which the body interprets the thyroid glands and its hormone products T3, T4 and TSH as threats, therefore producing special antibodies that target the thyroid’s cells, thereby destroying it. It presents with hypothyroidism or hyperthyroidism and the presence or absence of goiters. Specialists clinically separate autoimmune thyroiditis into two categories. If goiters are present, it is understood as Hashimoto’s Thyroiditis. On the other hand, if the thyroid is atrophic, but does not present goiters, it is denominated Atrophic Thyroiditis. If the symptoms of thyroiditis appear in women after birth, it is attributed to such and therefore called Postpartum Thyroiditis. The effects of this disease are not permanent but transient. Symptoms may come and go depending on whether the patient receives treatment, and whether the treatment is effective.

Autoimmune Urticaria

Autoimmune Urticaria, nettle , commonly referred to as hives, is a kind of skin rash notable for pale red, raised, itchy bumps. Hives might also cause a burning or stinging sensation. Hives are frequently caused by allergic reactions; however, there are many nonallergic causes. Most cases of hives lasting less than six weeks (acute urticaria) are the result of an allergic trigger. Chronic urticaria is rarely due to an allergy. The majority of chronic hives cases have an unknown cause. In perhaps as many as 30 to 40% of patients with chronic idiopathic urticaria, it is caused by an autoimmune reaction. Acute viral infection is another common cause of acute urticaria (viral exanthem). Less common causes of hives include friction, pressure, temperature extremes, exercise, and sunlight.

Axonal & Neuronal Neuropathies

Axonal Neuropathy is a condition in which the nerve cells begin to function abnormally because the axons are degenerating. The effects of the condition can be felt as tingling, burning, weakness, numbness, or as a loss of motor function. Several descriptions are used for various symptom profiles including axonal peripheral neuropathy or motor axonal neuropathy, and different versions of the condition may be chronic or acute in nature. It can be caused by a number of diseases and disorders such as diabetes or a genetic neurological disorder called giant axonal neuropathy. The options for treatment depend on the initial cause of the neuropathy.

Symptoms can occur in various combinations and depend partly on the extent of the damage. Tingling, pricking, and burning sensations are often the earliest symptoms, but they can continue as the condition advances. Numbness can be felt with or without an effect on motor function; likewise, loss of motor function or weakness may present with or without loss of feeling. A patient may also lose some sensitivity to temperature in the affected area.

Axonal Neuropathy is different from demyelinating neuropathies in that it involves degeneration of the entire axon rather than only degeneration of the myelin sheath around the axon. If demyelinating neuropathies are not caught and treated early enough, however, they may lead to degeneration of the axons as well. Axonal peripheral neuropathy begins in the nerves of the extremities like the hands and feet and may involve either sensory or motor signal interruption or both. Motor axonal neuropathy specifically affects the movement of the muscle tissue and may or may not include changes in sensory information.

Balo Disease

Balo Disease is a rare and progressive variant of multiple sclerosis. It usually first appears in adulthood, but childhood cases have also been reported. While multiple sclerosis typically is a disease that waxes and wanes, Balo Disease is different in that it tends to be rapidly progressive. Symptoms may include headache, seizures, gradual paralysis, involuntary muscle spasms, and cognitive loss.

Behcet’s Disease

Behcet’s (beh-CHETS) disease, also called Behcet’s syndrome, is a rare disorder that causes chronic inflammation in blood vessels throughout your body. The exact cause of Behcet’s is unknown, but it may be an autoimmune disorder, which means the body’s immune system mistakenly attacks some of its own healthy cells. Both genetic and environmental factors may be responsible for Behcet’s disease.

The inflammation of Behcet’s disease leads to numerous symptoms that may initially seem unrelated. The signs and symptoms of Behcet’s disease – which may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores – vary from person to person and may come and go on their own.

Treatment aims to reduce the signs and symptoms of Behcet’s disease and to prevent serious complications, such as blindness.