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Complement deficiency

**Complement deficiency**
*Classification & external resources*
ICD-10	D84.1
ICD-9	279.8
OMIM	217000 120820, 120900, 610102
DiseasesDB	1847 1869, 1873, 7384, 34381
eMedicine	med/419 ped/447

Complement deficiency is a condition of absent or suboptimal functioning of one of the complement system proteins.

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The disorders can be divided into two categories:

Disorders of the proteins which act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema and hemolytic-uremic syndrome.

Disorders of the proteins which act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections.

Because there are redundancies in the immune system, many complement disorders are never diagnosed. A recent study estimated that less than 10% are identified.^[1]

References

^ Sjöholm AG, Jönsson G, Braconier JH, Sturfelt G, Truedsson L (2006). "Complement deficiency and disease: an update". Mol. Immunol. 43 (1-2): 78-85. PMID 16026838.

v • d • e Pathology: Immune disorders (primarily D80-D89, 273, 279)
Antibody defects	X-linked agammaglobulinemia - Selective immunoglobulin A deficiency
Combined immunodeficiencies	Severe combined immunodeficiency (X-SCID) - Adenosine deaminase deficiency - Nezelof syndrome - Purine nucleoside phosphorylase deficiency - Bare lymphocyte syndrome
Other	Wiskott-Aldrich syndrome - DiGeorge syndrome - Hyper-IgE syndrome - Common variable immunodeficiency - Complement deficiency (Angioedema) - Sarcoidosis - Hypergammaglobulinemia - Cryoglobulinemia - Paraproteinemia - Immunodeficiency/Primary immunodeficiency
Autoimmunity	see list of autoimmune diseases