Immune disorder explained

Immune disorder
Synonyms:	Autoimmune disease
Field:	Immunology

An immune disorder is a dysfunction of the immune system.^[1] 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, more than 150 primary immunodeficiency diseases (PIDs) have been characterized.^[2] However, the number of acquired immunodeficiencies exceeds the number of PIDs.^[3]

It has been suggested that most people have at least one primary immunodeficiency.^[4] Due to redundancies in the immune system, though, many of these are never detected.

Autoimmune diseases

See main article: Autoimmune disease.

An autoimmune disease is a condition arising from an abnormal immune response to a normal body part.^[5] There are at least 80 types of autoimmune diseases.^[5] Nearly any body part can be involved. Common symptoms include low-grade fever and feeling tired.^[5] Often symptoms come and go.^[5]

List of some autoimmune disorders

• Lupus
• Scleroderma
• Certain types of hemolytic anemia
• Vasculitis
• Type 1 diabetes
• Graves' disease
• Rheumatoid arthritis
• Multiple sclerosis (although it is thought to be an immune-mediated process)
• Goodpasture syndrome
• Pernicious anemia
• Some types of myopathy
• Lyme disease (Late)
• Celiac disease
• Alopecia Areata ^[1]

Immunodeficiencies

See main article: immunodeficiency. Primary immune deficiency diseases 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.^[6]

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.^[7]

Primary immune deficiencies

• Severe combined immunodeficiency (SCID)
• DiGeorge syndrome
• Hyperimmunoglobulin E syndrome (also known as Job's Syndrome)
• Common variable immunodeficiency (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 disease (CGD): a deficiency in NADPH oxidase enzyme, which causes failure to generate oxygen radicals. Classical recurrent infection from catalase positive bacteria and fungi.
• Wiskott–Aldrich syndrome (WAS)
• Autoimmune lymphoproliferative syndrome (ALPS)
• Hyper IgM syndrome: 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 deficiency (LAD)
• NF-κB Essential Modifier (NEMO) Mutations
• Selective immunoglobulin A deficiency: the most common defect of the humoral immunity, characterized by a deficiency of IgA. Produces repeating sino-pulmonary and gastrointestinal infections.
• X-linked agammaglobulinemia (XLA; also known as Bruton type agammaglobulinemia): characterized by a deficiency in tyrosine kinase enzyme that blocks B cell maturation in the bone marrow. No B cells are produced to circulation and thus, there are no immunoglobulin classes, although there tends to be a normal cell-mediated immunity.
• X-linked lymphoproliferative disease (XLP)
• Ataxia–telangiectasia

Secondary immune deficiencies

• AIDS

Allergies

See main article: allergy. An allergy is an abnormal immune reaction to a harmless antigen.

• Seasonal allergy
• Mastocytosis
• Perennial allergy
• Anaphylaxis
• Food allergy
• Allergic rhinitis^[8]
• Atopic dermatitis

See also

• Disorders of human immunity
• Hypersplenism

Notes and References

1. Eva Y.-H. P. Lee . Erjavec SO, Gelfman S, Abdelaziz AR, Lee EY, Monga I, Alkelai A, Ionita-Laza I, Petukhova L, Christiano AM. Whole exome sequencing in Alopecia Areata identifies rare variants in KRT82. Nat Commun . 13 . 1. 800 . Feb 2022 . 35145093 . 10.1038/s41467-022-28343-3. free . 8831607 . 2022NatCo..13..800E.
2. Geha RS, Notarangelo LD, Casanova JL, etal . Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee . J. Allergy Clin. Immunol. . 120 . 4 . 776–94 . October 2007 . 17952897 . 10.1016/j.jaci.2007.08.053. 2601718.
3. Kumar A, Teuber SS, Gershwin ME . Current perspectives on primary immunodeficiency diseases . Clin. Dev. Immunol. . 13 . 2–4 . 223–59 . 2006 . 17162365 . 2270780 . 10.1080/17402520600800705 .
4. Casanova JL, Abel L . 7287315 . Primary immunodeficiencies: a field in its infancy . Science . 317 . 5838 . 617–9 . August 2007 . 17673650 . 10.1126/science.1142963 . 2007Sci...317..617C .
5. Web site: Autoimmune diseases fact sheet. OWH. 5 October 2016. 16 July 2012. live. https://web.archive.org/web/20161005144045/https://www.womenshealth.gov/publications/our-publications/fact-sheet/autoimmune-diseases.html. 5 October 2016.
6. Web site: Primary Immune Deficiency Diseases. Department of Health and Human Services

   National Institutes of Health

   . 31 August 2011.
7. Web site: Primary Immunodeficiency FAQ. INFO4PI. 31 August 2011. dead. https://web.archive.org/web/20111002091922/http://www.info4pi.org/aboutPI/index.cfm?section=aboutPI&content=faq. 2 October 2011.
8. Web site: Immune Disorders.