Immunodeficiency due to a classical component pathway complement deficiency

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Immunodeficiency due to a classical component pathway complement deficiency

Synonyms: Immunodeficiency due to C1, C4, or C2 component complement deficiency | Immunodeficiency due to an early component of complement deficiency

A rare primary immunodeficiency due to a deficiency in either complement components C1q C1r C1s C2 or C4 characterized by increased susceptibility to bacterial infections particularly with encapsulated bacteria and increased risk for autoimmune disease. Most commonly these include systemic lupus erythematosus (SLE) SLE-like disease Henoch-Schonlein purpura polymyositis and arthralgia. Disease severity is variable and dependent on the complement affected.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version March 2024

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