Low factor H-related 5 levels contribute to infection-triggered haemolytic uraemic syndrome and membranoproliferative glomerulonephritis

• Published in: Clinical kidney journal Vol. 14; no. 2; pp. 707 - 709
• Main Authors: Gómez Delgado, Irene, Gutiérrez-Tenorio, Josué, Fraga Rodríguez, Gloria M, Cavero, Teresa, Arjona, Emilia, Sánchez-Corral, Pilar
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01-02-2021
• Subjects: Bacterial pneumonia; Exceptional Cases; Glomerulonephritis; Health aspects; Hepatitis C virus; Kidneys; Liver; Pneumonia; Transplantation; Spain; Streptococcus; HUS; pneumoniae; CFHR5; HCV; complement; MPGN
• ISSN: 2048-8505; 2048-8513
• DOI: 10.1093/ckj/sfaa004

Dysregulation of the alternative complement pathway is a major pathogenic mechanism in two rare renal diseases: atypical haemolytic uraemic syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN). We report on a 66-year-old male with chronic hepatitis C virus (HCV) infection and a combined liver-kidney transplant that was diagnosed with MPGN at the age of 63 years and a 5-year-old boy who presented with aHUS at the age of 21 months following a
infection. Both patients carried similar frameshift variants in the complement
gene that segregate with reduced levels of factor H-related 5 (FHR-5). We conclude that low FHR-5 levels may predispose to viral and bacterial infections that then trigger different renal phenotypes.