Immune disorder
Encyclopedia
An immune disorder is a dysfunction of the immune system
. These disorders can be characterized in several different ways:
According to the International Union of Immunological Societies
, more than 150 primary immunodeficiency diseases (PIDs) have been characterized. However, the number of acquired immunodeficiencies exceeds the number of PIDs.
It has been suggested that most people have at least one primary immunodeficiency. Due to redundancies in the immune system, though, many of these are never detected.
are those caused by inherited genetic mutations. Secondary or acquired immune deficiencies are caused by something outside the body such as a virus
or immune suppressing drugs.
Primary immune diseases are at risk to an increased susceptibility to, and often recurrent ear infections, pneumonia
, bronchitis
, sinusitis
or skin infections. Immunodeficient patients may less frequently develop abscesses of their internal organs, autoimmune or rheumatologic and gastrointestinal problems.
Primary immune deficiencies
Secondary immune deficiencies
Immune system
An immune system is a system of biological structures and processes within an organism that protects against disease by identifying and killing pathogens and tumor cells. It detects a wide variety of agents, from viruses to parasitic worms, and needs to distinguish them from the organism's own...
. These disorders can be characterized in several different ways:
- By the component(s) of the immune system affected
- By whether the immune system is overactive or underactive
- By whether the condition is congenital or acquired
According to the International Union of Immunological Societies
International Union of Immunological Societies
The International Union of Immunological Societies, a member of the International Council for Science, is an organization which serves as an umbrella organization for many national immunological societies. The organization was founded in 1969....
, more than 150 primary immunodeficiency diseases (PIDs) have been characterized. However, the number of acquired immunodeficiencies exceeds the number of PIDs.
It has been suggested that most people have at least one primary immunodeficiency. Due to redundancies in the immune system, though, many of these are never detected.
List of some autoimmune disorders
- LupusLupusLupus most commonly refers to the disease systemic lupus erythematosus.Lupus may also refer to:-Medicine:* Lupus erythematosus, a chronic autoimmune disease with several different forms...
- SclerodermaSclerodermaSystemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
- Certain types of hemolytic anemiaHemolytic anemiaHemolytic anemia is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells , either in the blood vessels or elsewhere in the human body . It has numerous possible causes, ranging from relatively harmless to life-threatening...
- VasculitisVasculitisVasculitis refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis...
- Type one diabetes
- Graves disease
- Rheumatoid arthritisRheumatoid arthritisRheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...
- Multiple sclerosisMultiple sclerosisMultiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
- Goodpasture's syndromeGoodpasture's syndromeGoodpasture’s syndrome is a rare disease characterized by glomerulonephritis and hemorrhaging of the lungs...
- Pernicious anemiaPernicious anemiaPernicious anemia is one of many types of the larger family of megaloblastic anemias...
- Some types of myopathyMyopathyIn medicine, a myopathy is a muscular disease in which the muscle fibers do not function for any one of many reasons, resulting in muscular weakness. "Myopathy" simply means muscle disease...
Immunodeficiencies
Primary immune deficiency diseasesPrimary immunodeficiency
Primary immunodeficiencies are disorders in which part of the body's immune system is missing or does not function properly. To be considered a primary immunodeficiency, the cause of the immune deficiency must not be secondary in nature...
are those caused by inherited genetic mutations. Secondary or acquired immune deficiencies are caused by something outside the body such as a virus
Virus
A virus is a small infectious agent that can replicate only inside the living cells of organisms. Viruses infect all types of organisms, from animals and plants to bacteria and archaea...
or immune suppressing drugs.
Primary immune diseases are at risk to an increased susceptibility to, and often recurrent ear infections, pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
, bronchitis
Bronchitis
Acute bronchitis is an inflammation of the large bronchi in the lungs that is usually caused by viruses or bacteria and may last several days or weeks. Characteristic symptoms include cough, sputum production, and shortness of breath and wheezing related to the obstruction of the inflamed airways...
, sinusitis
Sinusitis
Sinusitis is inflammation of the paranasal sinuses, which may be due to infection, allergy, or autoimmune issues. Most cases are due to a viral infection and resolve over the course of 10 days...
or skin infections. Immunodeficient patients may less frequently develop abscesses of their internal organs, autoimmune or rheumatologic and gastrointestinal problems.
Primary immune deficiencies
- Severe combined immunodeficiencySevere combined immunodeficiencySevere combined immunodeficiency , is a genetic disorder in which both "arms" of the adaptive immune system are impaired due to a defect in one of several possible genes. SCID is a severe form of heritable immunodeficiency...
(SCID) - DiGeorge syndromeDiGeorge syndrome22q11.2 deletion syndrome, which has several presentations including DiGeorge syndrome , DiGeorge anomaly, velo-cardio-facial syndrome, Shprintzen syndrome, conotruncal anomaly face syndrome, Strong syndrome, congenital thymic aplasia, and thymic hypoplasia is a syndrome caused by the deletion of a...
- Hyperimmunoglobinemia E syndrome (also known as Job’s Syndrome)
- Common variable immunodeficiencyCommon variable immunodeficiencyCommon variable immunodeficiency is a group of approximately 150 primary immunodeficiencies , which have a common set of symptoms but which have different underlying causes.Common variable immunodeficiency is the most commonly encountered primary immunodeficiency.-Causes and types:CVID...
(CVID): B-cell levels are normal in circulation but with decreased production of IgG throughout the years, so it is the only primary immune disorder that presents onset in the late teens years. - Chronic granulomatous diseaseChronic granulomatous diseaseChronic granulomatous disease is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds used to kill certain ingested pathogens...
(CGD): a deficiency in NADPH oxidaseNADPH oxidaseThe NADPH oxidase is a membrane-bound enzyme complex. It can be found in the plasma membrane as well as in the membrane of phagosome.-Subunits:It is made up of six subunits...
enzyme, which causes failure to generate oxygen radicals. Classical recurrent infection from catalase positive bacteria and fungi. - Wiskott-Aldrich syndromeWiskott-Aldrich syndromeWiskott–Aldrich syndrome is a rare X-linked recessive disease characterized by eczema, thrombocytopenia , immune deficiency, and bloody diarrhea . It is also sometimes called the eczema-thrombocytopenia-immunodeficiency syndrome in keeping with Aldrich's original description in 1954...
(WAS) - Autoimmune lymphoproliferative syndromeAutoimmune lymphoproliferative syndromeAutoimmune lymphoproliferative syndrome is a form of lymphoproliferative disorder. It affects lymphocyte apoptosis. It is a RASopathy.-Introduction:...
(ALPS) - Hyper-Immunoglobulin M syndrome (Hyper-IgM): X-linked disorder that causes a deficiency in the production of CD40 ligand on activated T-cells. This increases the production and release of IgM into circulation. The B-cell and T-cell numbers are within normal limits. Increased susceptibility to extracellular bacteria and opportunistic infections.
- Leukocyte adhesion deficiencyLeukocyte adhesion deficiencyLeukocyte adhesion deficiency , is a rare autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections. Leukocyte adhesion deficiency is divided into at least two subtypes: LAD1 and LAD2.-Characteristics:...
(LAD) - NF-κB Essential Modifier (NEMO) Mutations
- Selective immunoglobulin A deficiencySelective immunoglobulin A deficiencySelective immunoglobulin A deficiency is a relatively mild genetic immunodeficiency. People with this deficiency lack immunoglobulin A , a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is defined as an undetectable...
: the most common defect of the humoral immunity, characterized by a deficiency of IgA. Produces repeating sino-pulmonary and gastrointestinal infections. - X-linked agammaglobulinemiaX-linked agammaglobulinemiaX-linked agammaglobulinemia is a rare X-linked genetic disorder that was discovered in 1952 which affects the body's ability to fight infection. XLA is an X-linked disorder, and therefore is more common in males...
(XLA; also known as Bruton type agammaglobulinemia): characterized by a deficiency in tyrosine kinaseTyrosine kinaseA tyrosine kinase is an enzyme that can transfer a phosphate group from ATP to a protein in a cell. It functions as an "on" or "off" switch in many cellular functions....
enzyme that blocks B-cell maturation in the bone marrowBone marrowBone marrow is the flexible tissue found in the interior of bones. In humans, bone marrow in large bones produces new blood cells. On average, bone marrow constitutes 4% of the total body mass of humans; in adults weighing 65 kg , bone marrow accounts for approximately 2.6 kg...
. No B-cells are produced to circulation and thus, there are no immunoglobulin classes, although there tends to be a normal cell-mediated immunityCell-mediated immunityCell-mediated immunity is an immune response that does not involve antibodies but rather involves the activation of macrophages, natural killer cells , antigen-specific cytotoxic T-lymphocytes, and the release of various cytokines in response to an antigen...
. - X-linked lymphoproliferative diseaseX-linked lymphoproliferative diseaseX-linked lymphoproliferative disease is a lymphoproliferative disorder.-XLP1:There is a mutation on the X-chromosome that has been found to be associated with a T- and NK-cell lymphoproliferative disorder. The mutation is on the long arm of the chromosome, at position 25, which is denoted as Xq25...
(XLP) - Ataxia-telangiectasia
Secondary immune deficiencies
- AIDSAIDSAcquired immune deficiency syndrome or acquired immunodeficiency syndrome is a disease of the human immune system caused by the human immunodeficiency virus...
Allergies
An allergy is an abnormal immune reaction to a harmless antigen.- Seasonal allergy
- MastocytosisMastocytosisMastocytosis is a group of rare disorders of both children and adults caused by the presence of too many mast cells and CD34+ mast cell precursors in a person's body.- Classification :Mastocytosis can occur in a variety of forms:...
- Perennial allergy
- AnaphylaxisAnaphylaxisAnaphylaxis is defined as "a serious allergic reaction that is rapid in onset and may cause death". It typically results in a number of symptoms including throat swelling, an itchy rash, and low blood pressure...
- Food allergyFood allergyA food allergy is an adverse immune response to a food protein. They are distinct from other adverse responses to food, such as food intolerance, pharmacological reactions, and toxin-mediated reactions....
- Allergic rhinitisAllergic rhinitisAllergic rhinitis, also known as pollenosis or hay fever, is an allergic inflammation of the nasal airways.It occurs when an allergen, such as pollen, dust or animal dander is inhaled by an individual with a sensitized immune system...
- Atopic dermatitisAtopic dermatitisAtopic dermatitis is an inflammatory, chronically relapsing, non-contagious and pruritic skin disorder...
See also
- Disorders of human immunity
- Hypersplenism