Clinical and laboratory evaluation of complement deficiency.

Area of Science:

• Immunology
• Innate Immunity
• Complement System Biology

Background:

• The complement system is a crucial part of innate immunity, working alongside antibody-mediated immunity.
• It functions as a proinflammatory cascade, amplifying responses through limited proteolysis.
• Key functions include opsonization, cell lysis, and inflammatory peptide release.

Purpose of the Study:

• To review the clinical manifestations and pathophysiology of complement deficiency diseases.
• To present a diagnostic algorithm for complement deficiencies.
• To discuss current and future therapeutic strategies for complement-related disorders.

Main Methods:

• Literature review of complement system function, deficiencies, and treatments.
• Summarization of clinical presentations associated with complement deficiencies.
• Outline of a diagnostic approach and therapeutic options.

Main Results:

• Complement deficiencies predispose individuals to infections and autoimmune syndromes, particularly Systemic Lupus Erythematosus (SLE).
• While total deficiencies are rare, increased incidence is observed in specific patient groups.
• The review synthesizes information on congenital and acquired forms of these deficiencies.

Conclusions:

• Complement deficiencies have significant clinical implications, impacting susceptibility to infections and autoimmunity.
• Accurate laboratory diagnosis is essential for effective management.
• Advances in understanding offer promising therapeutic avenues for complement deficiency diseases.