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Related Experiment Videos

Atypical hypocomplementemic vasculitis syndrome in a child.

F B Waldo, P A Leist, C F Strife

    The Journal of Pediatrics
    |May 1, 1985
    PubMed
    Summary

    A rare case of severe acquired C1q deficiency presented in childhood with recurrent urticaria, progressing to end-stage renal disease. This complement disorder highlights potential links between C1q deficiency and glomerulonephritis.

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    Area of Science:

    • Immunology
    • Nephrology
    • Genetics

    Background:

    • Hypocomplementemic vasculitis syndrome (HVS) typically presents later in life.
    • Severe hypocomplementemia and progression to end-stage renal disease (ESRD) are atypical features for HVS.

    Observation:

    • A patient developed recurrent urticaria and angioedema at age 2, severe hypocomplementemic glomerulonephritis at 11, and ESRD at 14.
    • Extremely low serum C1q levels and significantly reduced C4, C2, C3, and C5 were observed.
    • Low C1 inhibitor (C1INH) levels and evidence of C1INH-C1r-C1s complexes suggested consumption.

    Findings:

    • Absence of a circulating complement activator and low C3/C5 suggested a solid-phase activator, potentially on vascular endothelium.
    • The patient's cells did not activate complement with normal serum, ruling out cellular activators.

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  • Family members showed normal complement component levels, indicating an acquired deficiency in the patient.

    • Implications:

    • Severe, prolonged acquired C1q deficiency may predispose individuals to glomerulonephritis, similar to homozygous classical pathway component deficiencies.
    • This case expands the understanding of complement-mediated kidney disease and its potential triggers.
    • Further research into acquired C1q deficiency and its association with autoimmune and renal diseases is warranted.