Marie-Sophie Meuleman, Florent Petitprez, Matthew C Pickering, Moglie Le Quintrec, Mikel Rezola Artero, Anna Duval, Marion Rabant, Alyssa Gilmore, Olivia Boyer, Julien Hogan, Aude Servais, François Provot, Vivianne Gnemmi, Maeva Eloudzeri, Anne Grunenwald, David Buob, Jean-Jacques Boffa, Anissa Moktefi, Vincent Audard, Jean-Michel Goujon, Frank Bridoux F, Eric Thervet, Alexandre Karras, Lubka T Roumenina, Véronique Frémeaux- Bacchi, Jean-Paul Duong Van Huyen, Sophie Chauvet
BACKGROUND: C3 glomerulopathy is a rare disease resulting from an overactivation of the complement alternative pathway. Although there is also evidence of terminal pathway activation, its occurrence and consequences on the disease have been poorly studied. METHODS: We retrospectively studied a cohort of 42 patients diagnosed with C3 glomerulopathy. We performed centralized extensive characterization of histological parameters. Kidney C5b-9 staining was performed as a marker of terminal pathway activation, intra-renal immune response was characterised through transcriptomic analysis...
May 6, 2024: Journal of the American Society of Nephrology: JASN