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Related Experiment Videos

Crescentic glomerulonephritis in hyper IgD syndrome.

M Tsimaratos1, I Koné-Paut, L Daniel

  • 1Unité de Néphrologie Pédiatrique, Service de Pédiatrie Multidisciplinaire, Hôpital d'Enfants de la Timone, Marseille, France. gpicon@aphml.ap-hm.fr

Pediatric Nephrology (Berlin, Germany)
|May 6, 1999
PubMed
Summary

Hyperimmunoglobulinemia D syndrome (HIDS), a systemic inflammatory condition, can lead to end-stage renal failure. Early kidney biopsy is recommended for HIDS patients presenting with renal disease to diagnose crescentic glomerulonephritis.

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Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • Hyperimmunoglobulinemia D syndrome (HIDS) is a systemic autoinflammatory disorder.
  • HIDS typically presents with recurrent fever, rash, and gastrointestinal symptoms.
  • Amyloidosis and nephropathy are considered rare complications of HIDS.

Observation:

  • A case report of a patient with Mediterranean ancestry presenting with HIDS and end-stage renal failure.
  • Kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN).

Findings:

  • The patient exhibited typical HIDS symptoms alongside severe renal impairment.
  • Histopathological examination of the kidney demonstrated crescentic glomerulonephritis.

Implications:

Related Experiment Videos

  • This case suggests that crescentic glomerulonephritis may be an underrecognized complication of HIDS.
  • Cytokine network activation in HIDS might contribute to glomerular injury.
  • Early renal biopsy is crucial for timely diagnosis and management of renal involvement in HIDS patients.