Abstract
Background
Post-infectious glomerulonephritis (PIGN) usually follows a benign course, but few children have an atypical, severe presentation, and these exceptional cases have been linked to the dysregulation of the complement alternative pathway (CAP). There is a considerable overlap in the histopathological features of PIGN and C3 glomerulopathy (C3G), which is also associated with CAP dysregulation but has a poorer outcome. We hypothesized that PIGN and C3G define a disease spectrum, and that in the past there may be some children with C3G who were misclassified with PIGN before C3G was described as a separate disease entity.
Methods
Children with PIGN (n = 33) diagnosed between 1985 and 2010 who underwent a renal biopsy due to their unusual course were reviewed and of them, 8 were reclassified into C3G based on the current classification criteria. Outcome was based on the degree of proteinuria, C3 level, and renal function at follow-up.
Results
Sixteen (72.7%) children with typical PIGN recovered completely as compared to only 2 (25%) with C3G. Of note, children with “typical” PIGN had a more severe disease course at onset; however, the outcome at last follow up was favorable.
Conclusions
Our results support the hypothesis that PIGN and C3G form a disease spectrum and have different long-term clinical implications and management strategies.
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Badria Al-Ghaithi and Rahul Chanchlani contributed equally to this work.
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Al-Ghaithi, B., Chanchlani, R., Riedl, M. et al. C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum. Pediatr Nephrol 31, 2079–2086 (2016). https://doi.org/10.1007/s00467-015-3311-3
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DOI: https://doi.org/10.1007/s00467-015-3311-3