{
  "abstract": "Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with glomerulonephritis (AAV-GN) is classically considered pauci-immune, yet complement activation is a central pathogenic mechanism. Whether intrarenal complement-related transcriptional programs are reflected by conventional serum or immunofluorescence markers, and whether they are clinically informative, remains unclear. Methods: In 369 patients with AAV-GN from 2 independent retrospective cohorts, complement was assessed across circulating, histopathologic, and transcriptomic layers (serum C3/C4, glomerular C1q/C3 deposition, and targeted kidney transcriptomics). Predefined classical and alternative pathway-related expression scores were derived from intrarenal gene expression. Associations with severity and outcomes were evaluated using multivariable Cox models. Public spatial transcriptomic data sets provided validation and localization.\n\nResults: Below-normal serum C3/C4 levels were uncommon. Low serum C3, but not C4, was associated with more severe kidney involvement, kidney failure (adjusted hazard ratio [aHR]: 2.0 [1.1-3.7]), and mortality (aHR: 2.3 [1.3-4.0]). Both glomerular C3 (47%) and C1q (11%) deposition identified more severe pathology and adverse outcomes. Compared with controls, AAV-GN kidneys showed increased complement-related expression-including classical pathway components, proximal alternative pathway genes (C3, C5AR1), and regulatory transcripts-that was only weakly concordant with serum or deposition markers. Low alternative pathway-related expression was independently associated with better kidney (aHR: 0.3 [0.1-0.7]) and overall (aHR: 0.4 [0.2-0.9]) survival. Spatial transcriptomics validated these signatures and suggested contributions from both immune and resident kidney compartments.",
  "abstract_shown": true,
  "affiliations": [
    "Service de Néphrologie-Dialyse-Transplantation, CHU Angers, Angers, France",
    "Nephrology and Internal Medicine Department, Hospital of Valenciennes, Valenciennes, France",
    "Inserm, CNRS, CRCI2NA, Structure Fédérative de Recherche - Interactions Cellulaires et Applications Thérapeutiques (SFR ICAT), University of Angers, Nantes Université, Angers, France",
    "Laboratoire d’Immunologie et Immunogénétique, FHU ACRONIM, Hôpital Pellegrin, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France",
    "Service de Néphrologie-Dialyse, Centre Hospitalier de Cholet, Cholet, France",
    "Service de Néphrologie-Dialyse, Centre Hospitalier de Laval, Laval, France",
    "Service de Néphrologie-Dialyse, Centre Hospitalier du Mans, Le Mans, France",
    "Department of Human Genetics, Victor Phillip Dahdaleh Institute of Genomic Medicine, McGill University, Montreal, Quebec, Canada",
    "Service d'anatomopathologie, Centre Hospitalier Universitaire de Lille, Lille, France"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 21,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Benoît Brilland"
    },
    {
      "affs": [
        0
      ],
      "name": "Victor Milon"
    },
    {
      "affs": [
        1
      ],
      "name": "Cyrille Vandenbussche"
    },
    {
      "affs": [
        2
      ],
      "name": "Nathalie Merillon"
    },
    {
      "affs": [
        0
      ],
      "name": "Eleonore Ourouda"
    },
    {
      "affs": [
        3
      ],
      "name": "Andrea Boizard-Moracchini"
    },
    {
      "affs": [
        3
      ],
      "name": "Maëva Roy"
    },
    {
      "affs": [
        2
      ],
      "name": "Odile Blanchet"
    },
    {
      "affs": [
        2
      ],
      "name": "Emeline Vinatier"
    },
    {
      "affs": [
        2
      ],
      "name": "Céline Beauvillain"
    },
    {
      "affs": [
        1
      ],
      "name": "Thomas Quéméneur"
    },
    {
      "affs": [
        4
      ],
      "name": "Assia Djema"
    },
    {
      "affs": [
        5
      ],
      "name": "Nicolas Henry"
    },
    {
      "affs": [
        6
      ],
      "name": "Giorgina Barbara Piccoli"
    },
    {
      "affs": [
        6
      ],
      "name": "Samuel Wacrenier"
    },
    {
      "affs": [
        2
      ],
      "name": "Yves Delneste"
    },
    {
      "affs": [
        3
      ],
      "name": "Patrick Blanco"
    },
    {
      "affs": [
        7
      ],
      "name": "David Langlais"
    },
    {
      "affs": [
        8
      ],
      "name": "Viviane Gnemmi"
    },
    {
      "affs": [
        2
      ],
      "name": "Marie-Christine Copin"
    },
    {
      "affs": [
        0
      ],
      "name": "Jean-François Augusto"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/6zvd1zns01spa.bib",
  "cited_by": 0,
  "doi": "10.1016/j.ekir.2026.107056",
  "fulltext_url": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13657220/?tool=EBI",
  "id": "6zvd1zns01spa",
  "issns": [
    "2468-0249"
  ],
  "landing_url": "https://europepmc.org/article/PMC/PMC13657220",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "hal",
      "0.sdv"
    ],
    [
      "hal",
      "1.sdv.imm"
    ],
    [
      "openalex",
      "2740|Vasculitis and related conditions"
    ]
  ],
  "openalex": "W7204249169",
  "pdf_url": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13657220/pdf/?tool=EBI",
  "pmcid": "PMC13657220",
  "pmid": null,
  "published": "2026-08-26",
  "publisher": "Elsevier",
  "ris": "https://research.draconitic.com/paper/6zvd1zns01spa.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:17:44Z",
      "native_id": "PMC:PMC13657220",
      "source": "europepmc",
      "url": "https://europepmc.org/article/PMC/PMC13657220"
    },
    {
      "first_seen": "2026-10-30T12:18:00Z",
      "native_id": "hal-05830134",
      "source": "hal",
      "url": "https://u-bordeaux.hal.science/hal-05830134v1"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "hal:1.sdv.imm",
      "subfield": "immunology"
    }
  ],
  "title": "Multimodal Profiling of Complement Expression and Association With Outcomes in ANCA-Associated Vasculitis With Glomerulonephritis",
  "title_text": "Multimodal Profiling of Complement Expression and Association With Outcomes in ANCA-Associated Vasculitis With Glomerulonephritis",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:18:00Z",
  "url": "https://research.draconitic.com/paper/6zvd1zns01spa",
  "venue": "Kidney international reports",
  "versions": []
}