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Updated: Aug 8, 2026

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
06:29

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells

Published on: January 29, 2014

Glomerulonephritis associated with complete deficiency of the fourth component of complement. Response to intravenous

T R Welch1, A J McAdams, L S Beischel

  • 1Children's Hospital Research Foundation, Cincinnati, Ohio, USA.

Arthritis and Rheumatism
|September 1, 1995
PubMed

Insights

A teen with C4 deficiency and lupus developed nephritis. Intravenous immunoglobulin therapy improved symptoms and reduced kidney inflammation, suggesting alternative treatments for complement-deficient patients.

Area of Science:

  • Immunology
  • Nephrology
  • Genetics

Background:

  • Complete C4 deficiency is a rare genetic condition.
  • Individuals with C4 deficiency have an increased risk of autoimmune disorders, including lupus.
  • Lupus nephritis is a serious complication characterized by immune complex deposition in the kidneys.

Observation:

  • A 15-year-old female with C4 deficiency and a lupus-like disorder presented with nephritis.
  • Renal biopsy confirmed immune complex glomerulonephritis with significant deposits.
  • The patient had normal renal function at presentation.

Findings:

  • Treatment with monthly intravenous immunoglobulin (IVIG) for six months was well-tolerated.
  • IVIG therapy led to the resolution of cutaneous lupus manifestations (rash) and hematuria.
  • Follow-up renal biopsy revealed reduced glomerular proliferation and fewer immune deposits.

Implications:

  • This case highlights the potential efficacy of IVIG in managing lupus nephritis in complement-deficient patients.
  • The findings suggest that non-immunosuppressive therapies may be viable alternatives for glomerulonephritis in this population.
  • Given the heightened infection risk in complement deficiency, avoiding traditional immunosuppression is crucial.

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