Pathology · Immunopathology (Hypersensitivity, Autoimmunity, Immunodeficiency, Amyloidosis)

In systemic lupus erythematosus (SLE), defective clearance of apoptotic cells is a key pathogenic mechanism. Which complement protein deficiency is most strongly linked to the development of SLE, and why?

  • A C1q deficiency; C1q normally opsonizes apoptotic blebs for phagocytosis
  • B C3 deficiency; prevents opsonization of apoptotic cell debris
  • C C5 deficiency; terminal complement pathway needed to lyse apoptotic cells
  • D Factor D deficiency; alternative pathway needed to tag nuclear antigens
Correct answer: A. C1q deficiency; C1q normally opsonizes apoptotic blebs for phagocytosis

Explanation

C1q deficiency carries the highest risk for SLE among all complement deficiencies, with approximately 90% of C1q-deficient individuals developing an SLE-like syndrome. C1q normally binds to apoptotic blebs (especially surface-exposed phosphatidylserine and nuclear components) and opsonizes them for phagocytosis by macrophages. Without C1q-mediated opsonization, apoptotic cells accumulate in tissues, undergo secondary necrosis, and release nuclear antigens (histones, DNA, snRNPs), which drive autoantibody production. C1q also directly engages DNase I on the apoptotic cell surface, facilitating nuclear antigen clearance.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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