Immune disorder

Immune disorder
Classification and external resources
ICD-10	D80-D89
ICD-9-CM	273, 279
MeSH	D007154

An immune disorder is a dysfunction of the immune system. 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.^[1] However, the number of acquired immunodeficiencies exceeds the number of PIDs.^[2]

It has been suggested that most people have at least one primary immunodeficiency.^[3] Due to redundancies in the immune system, though, many of these are never detected.

List of some autoimmune disorders

Main article: autoimmune disorders

Lupus
Scleroderma
Certain types of hemolytic anemia
Vasculitis
Type one diabetes
Graves disease
Rheumatoid arthritis
Multiple sclerosis (although it is thought to be an immune-mediated process)
Goodpasture's syndrome
Pernicious anemia
Some types of myopathy
Lyme disease (Late)

Immunodeficiencies

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.^[4]

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.^[5]

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

Main article: allergy

An allergy is an abnormal immune reaction to a harmless antigen.

References

↑ Geha RS, Notarangelo LD, Casanova JL, et al. (October 2007). "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. doi:10.1016/j.jaci.2007.08.053. PMC 2601718. PMID 17952897.
↑ Kumar A, Teuber SS, Gershwin ME (2006). "Current perspectives on primary immunodeficiency diseases". Clin. Dev. Immunol. 13 (2–4): 223–59. doi:10.1080/17402520600800705. PMC 2270780. PMID 17162365.
↑ Casanova JL, Abel L (August 2007). "Primary immunodeficiencies: a field in its infancy". Science 317 (5838): 617–9. doi:10.1126/science.1142963. PMID 17673650.
↑ "Primary Immune Deficiency Diseases". Department of Health and Human Services: National Institutes of Health. Retrieved 31 August 2011.
↑ "Primary Immunodeficiency FAQ". INFO4PI. Retrieved 31 August 2011.
↑ http://www.merckmanuals.com/home/immune_disorders.html

External links

Immune disorder at DMOZ

Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4)

Primary

Antibody/humoral (B)

Hypogammaglobulinemia	X-linked agammaglobulinemia Transient hypogammaglobulinemia of infancy

Dysgammaglobulinemia	IgA deficiency IgG deficiency IgM deficiency Hyper IgM syndrome (1 2 3 4 5) Wiskott-Aldrich syndrome Hyper-IgE syndrome

Other	Common variable immunodeficiency ICF syndrome

T cell deficiency (T)

thymic hypoplasia: hypoparathyroid (Di George's syndrome)
euparathyroid (Nezelof syndrome
Ataxia telangiectasia)

peripheral: Purine nucleoside phosphorylase deficiency

Hyper IgM syndrome (1)

Severe combined (B+T)

x-linked: X-SCID
autosomal: Adenosine deaminase deficiency
Omenn syndrome
ZAP70 deficiency
Bare lymphocyte syndrome

Acquired

AIDS

Leukopenia:
Lymphocytopenia

Idiopathic CD4+ lymphocytopenia

Complement deficiency

Immunoproliferative immunoglobulin disorders (D89, 273)

PCDs/PP	Plasmacytoma Multiple myeloma (Plasma cell leukemia) MGUS IgM (Macroglobulinemia/Waldenström's macroglobulinemia) heavy chain (Heavy chain disease) light chain (Primary amyloidosis)

Other hypergammaglobulinemia	Cryoglobulinemia

Hypersensitivity and autoimmune diseases (279.5–6)

Type I/allergy/atopy
(IgE)

Foreign	Atopic eczema Allergic urticaria Allergic rhinitis (Hay fever) Allergic asthma Anaphylaxis Food allergy common allergies include: Milk Egg Peanut Tree nut Seafood Soy Wheat Penicillin allergy

Autoimmune	Eosinophilic esophagitis

Type II/ADCC

- IgM
- IgG

Foreign

Hemolytic disease of the newborn

Autoimmune

Cytotoxic	Autoimmune hemolytic anemia Immune thrombocytopenic purpura Bullous pemphigoid Pemphigus vulgaris Rheumatic fever Goodpasture's syndrome Guillain–Barré syndrome

"Type V"/receptor	Graves' disease Myasthenia gravis Pernicious anemia

Type III
(Immune complex)

Foreign	Henoch–Schönlein purpura Hypersensitivity vasculitis Reactive arthritis Farmer's lung Post-streptococcal glomerulonephritis Serum sickness Arthus reaction

Autoimmune	Systemic lupus erythematosus Subacute bacterial endocarditis Rheumatoid arthritis

Type IV/cell-mediated
(T cells)

Foreign	Allergic contact dermatitis Mantoux test

Autoimmune	Diabetes mellitus type 1 Hashimoto's thyroiditis Multiple sclerosis Coeliac disease Giant-cell arteritis Postorgasmic illness syndrome Reactive arthritis

GVHD	Transfusion-associated graft versus host disease

Unknown/
multiple

Foreign	Hypersensitivity pneumonitis Allergic bronchopulmonary aspergillosis Transplant rejection Latex allergy (I+IV)

Autoimmune	Sjögren's syndrome Autoimmune hepatitis Autoimmune polyendocrine syndrome APS1 APS2 Autoimmune adrenalitis Systemic autoimmune disease

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