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The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
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Related Experiment Video

Updated: Feb 11, 2026

Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus
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Published on: May 16, 2025

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Complement deficiency in pediatric-onset systemic lupus erythematosus.

Parisa Afzali1,2, Anna Isaeian2, Peyman Sadeghi3

  • 1Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Journal of Laboratory Physicians
|April 26, 2018
PubMed
Summary

Complement deficiencies are common in pediatric-onset systemic lupus erythematosus (pSLE), associated with earlier onset and more severe symptoms. Consider complement testing in pSLE patients with aggressive disease and negative anti-ds-DNA. Keywords: pediatric lupus, complement deficiency, SLE manifestations.

Keywords:
C1q deficiencychildhood-onsetchildrencomplement deficiencysystemic lupus erythematosus

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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Pediatric-onset systemic lupus erythematosus (pSLE) comprises 10-20% of SLE cases.
  • Early complement pathway deficiencies are significant genetic risk factors for SLE development.
  • This study compares clinical and laboratory features of pSLE patients with and without complement deficiencies.

Purpose of the Study:

  • To investigate the clinical and immunological manifestations of pSLE in Iran.
  • To compare pSLE patients with and without complement deficiencies.
  • To identify correlations between complement levels and disease characteristics.

Main Methods:

  • 36 consecutive pSLE patients (onset < 18 years) were studied over 2 years.
  • Complement C1q, C2, C3, and C4 levels were measured using radial immunodifusion and nephelometry.
  • Retrospective evaluation of medical records and statistical analysis (descriptive, t-test, Pearson correlation).

Main Results:

  • 58% of pSLE patients had at least one complement component deficiency.
  • Low C1q (27%) and C2 (30.5%) were most frequent; C3 (25%) and C4 (11%) were also observed.
  • Complement deficiency correlated with earlier disease onset, more frequent/severe cutaneous manifestations, and higher renal morbidity, but less frequent anti-ds-DNA positivity.

Conclusions:

  • Complement deficiency is prevalent in pSLE patients.
  • pSLE with complement deficiency often presents with earlier onset and more severe clinical manifestations.
  • Complement testing is recommended for pSLE patients with aggressive disease and negative anti-ds-DNA results.