A potential pathogenic role of interleukin-6 in a child with ANCA-negative pauci-immune crescentic glomerulonephritis: case report and literature review.

Abstract

BACKGROUND:

Crescentic glomerulonephritis is a disease characterized by severe glomerular injuries that is classified into five different pathological types. Patients with type V disease have pauci-immune crescentic glomerulonephritis (PICGN) that is negative for anti-neutrophil cytoplasmic autoantibodies (ANCAs). There are limited clinical data on the manifestations, treatment, and prognosis of type V crescentic glomerulonephritis, especially in children.

CASE PRESENTATION:

A 13-year-old girl who had an intermittent fever for more than 10 months was admitted to our hospital. She had no gross hematuria, oliguria, edema, or hypertension, but further tests indicated a decreased glomerular filtration rate, hematuria, proteinuria, and an elevated level of IL-6. The antinuclear antibody spectrum test was positive at 1:1000, and the ANCA and anti-glomerular basement membrane antibody tests were negative. A renal biopsy confirmed the diagnosis of ANCA-negative PICGN. We administered methylprednisolone pulse therapy with intravenous cyclophosphamide and oral mycophenolate mofetil. At the 3-month follow-up, her urine protein level was significantly lower, and her serum creatinine level was in the normal range.

CONCLUSIONS:

Fever may be an extrarenal manifestation of ANCA-negative PICGN, and IL-6 may play a role in the pathogenesis of this disease. Early methylprednisolone pulse therapy with an immunosuppressant may reduce symptoms and improve prognosis.

• Authors: Ling Hou, Lu Yin, Yubin Wu, Chengguang Zhao, Yue Du
• Journal: BMC nephrology (BMC Nephrol) Vol. 22 Issue 1 Pg. 285 (08 23 2021) ISSN: 1471-2369 [Electronic] England
• PMID: 34425760 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: © 2021. The Author(s).

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Interleukin-6

Topics

• Adolescent
• Antibodies, Antineutrophil Cytoplasmic (blood)
• Female
• Fever of Unknown Origin (etiology)
• Glomerulonephritis (complications, immunology, pathology)
• Humans
• Interleukin-6 (blood)
• Kidney Glomerulus (pathology, ultrastructure)