Academic Journal
C3 glomerulopathy associated with anti-factor H autoantibodies in a child with inflammatory bowel disease.
| Τίτλος: | C3 glomerulopathy associated with anti-factor H autoantibodies in a child with inflammatory bowel disease. |
|---|---|
| Συγγραφείς: | Ban M; Division of Pediatric Nephrology, Dialysis and Transplantation, Department of Pediatrics, University Hospital Center Zagreb, Zagreb, Croatia., Matković H; Division of Pediatric Nephrology, Dialysis and Transplantation, Department of Pediatrics, University Hospital Center Zagreb, Zagreb, Croatia., Aničić MN; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University Hospital Center Zagreb, Zagreb, Croatia.; Department of Pediatrics, University of Zagreb School of Medicine, Zagreb, Croatia., Ćorić M; Department of Pathology and Cytology, University Hospital Center Zagreb, Zagreb, Croatia.; Department of Pathology, University of Zagreb School of Medicine, Zagreb, Croatia., Prohászka Z; Department of Internal Medicine and Hematology, Semmelweis University, Budapest, Hungary., Lamot L; Division of Pediatric Nephrology, Dialysis and Transplantation, Department of Pediatrics, University Hospital Center Zagreb, Zagreb, Croatia. lovro.lamot@kbc-zagreb.hr.; Department of Pediatrics, University of Zagreb School of Medicine, Zagreb, Croatia. lovro.lamot@kbc-zagreb.hr. |
| Πηγή: | Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2026 Nov; Vol. 41 (11), pp. 3899-3902. Date of Electronic Publication: 2026 Jun 16. |
| Τύπος έκδοσης: | Journal Article; Case Reports |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Springer International Country of Publication: Germany NLM ID: 8708728 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-198X (Electronic) Linking ISSN: 0931041X NLM ISO Abbreviation: Pediatr Nephrol Subsets: MEDLINE |
| Imprint Name(s): | Publication: Berlin : Springer International Original Publication: Berlin : Springer International, c1987- |
| Ιατρικοί όροι (MeSH): | Autoantibodies*/immunology , Autoantibodies*/blood , Complement C3*/immunology , Complement C3*/analysis , Complement C3*/metabolism , Complement Factor H*/immunology , Glomerulonephritis, Membranoproliferative*/immunology , Glomerulonephritis, Membranoproliferative*/drug therapy , Glomerulonephritis, Membranoproliferative*/pathology , Inflammatory Bowel Diseases*/immunology , Inflammatory Bowel Diseases*/complications , Inflammatory Bowel Diseases*/drug therapy , Acute Kidney Injury*/immunology , Acute Kidney Injury*/drug therapy , Acute Kidney Injury*/etiology , Glomerulonephritis*/immunology , Glomerulonephritis*/drug therapy , Glomerulonephritis*/pathology, Glucocorticoids/therapeutic use ; Humans ; Male ; Child ; Biopsy |
| Περίληψη: | Complement dysregulation may occur secondary to systemic immune activation, but its relationship with inflammatory bowel disease (IBD) remains poorly defined. We report a 10-year-old boy presenting with severe anemia and newly diagnosed IBD who developed acute kidney injury with microscopic hematuria and proteinuria. Complement studies showed markedly reduced C3 and CH50 with normal C4. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis with dominant C3 deposition and subendothelial immune complexes, consistent with C3 glomerulopathy. Additional complement testing revealed factor H depletion, high titers of anti-factor H autoantibodies, and elevated soluble C5b-9, while C3 nephritic factor and genetic testing of complement genes were negative. Multiple systemic autoantibodies were simultaneously present. Treatment with glucocorticoids resulted in rapid clinical improvement with normalization of kidney function, complement parameters, and disappearance of anti-factor H antibodies during follow-up. This case illustrates how transient systemic immune activation associated with intestinal inflammation may induce acquired complement dysregulation and trigger C3 glomerulopathy. (© 2026. The Author(s), under exclusive licence to International Pediatric Nephrology Association.) |
| Competing Interests: | Declarations. Consent for publication: The authors declare that they have obtained written informed consent from the patient's parents and the patient discussed in the report. Conflict of interest: The authors declare that they have no financial or personal relationships that could inappropriately influence (bias) their work. In accordance with ICMJE guidelines, all authors report no conflicts of interest relevant to the content of this manuscript. |
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| Contributed Indexing: | Keywords: Anti-factor H autoantibodies; C3 glomerulopathy; Complement-targeting therapy; Inflammatory bowel disease; Pediatric kidney disease; Systemic inflammation |
| Substance Nomenclature: | 0 (Autoantibodies) 0 (Complement C3) 80295-65-4 (Complement Factor H) 0 (Glucocorticoids) |
| Entry Date(s): | Date Created: 20260616 Date Completed: 20260925 Latest Revision: 20261008 |
| Update Code: | 20261008 |
| DOI: | 10.1007/s00467-026-07415-2 |
| PMID: | 42301434 |
| Βάση Δεδομένων: | MEDLINE |
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