komplementhiányokban

Komplementhiányokban refers to conditions characterized by a deficiency in components of the complement system, a crucial part of the innate immune system. The complement system is a cascade of proteins that work together to defend the body against pathogens, clear immune complexes, and regulate inflammation. Deficiencies can occur in any of the many proteins that make up the system, including C1, C2, C3, C4, C5, C6, C7, C8, C9, or regulatory proteins like Factor H or Factor I.

These deficiencies can be inherited, meaning they are caused by genetic mutations passed down from parents, or acquired, developing later in life due to other medical conditions or treatments. The clinical manifestations of complement deficiencies vary widely depending on which component is lacking and the severity of the deficiency. Generally, deficiencies in early components of the classical pathway (C1, C4, C2) are associated with an increased risk of autoimmune diseases, particularly systemic lupus erythematosus. Deficiencies in later components of the common pathway (C3) or the terminal pathway (C5-C9) lead to a significantly heightened susceptibility to infections, especially by encapsulated bacteria such as Neisseria meningitidis and Streptococcus pneumoniae.

Diagnosis typically involves laboratory testing to measure the levels and functional activity of specific complement proteins. Management strategies focus on preventing and treating infections, often with prophylactic antibiotics, and managing associated autoimmune conditions. While there is no cure for inherited complement deficiencies, individuals can lead relatively normal lives with appropriate medical care and monitoring.