Phenotypic characterization of patients with ANCA-associated vasculitis and diffuse alveolar hemorrhage: a data-driven analysis of a multicentre, retrospective, nationwide cohort
Abstract
Background
Severe diffuse alveolar hemorrhage (DAH) is a potentially fatal complication of ANCA-associated vasculitides (AAV). This study aimed to identify distinct clinical phenotypes among patients to the ICU, and to examine their association with mortality.Methods
We conducted an ancillary analysis of the national multicenter retrospective PLEXHIA cohort, including 197 patients with severe AAV-related DAH, defined by respiratory symptoms with new infiltrates consistent with alveolar hemorrhage, along with oxygen saturation ≤85%, high-flow oxygen or mechanical ventilation requirement. Kohonen self-organizing maps were applied to predefined clinical variables at ICU admission, excluding treatment and outcomes. Clusters were interpreted and compared regarding organ involvement, severity, management, and mortality at 30 and 90 days.Results
Between 2012 and 2024, 197 patients (66 years old, 52% female) were included, revealing marked clinical heterogeneity. Three phenotypes emerged: The MPO pulmonary-renal (MPO-PR) cluster included patients with anti-MPO ANCA, renal involvement and high oxygen requirements. The mild-respiratory (Mild-R) cluster included predominantly female patients with less severe respiratory compromise and mild renal involvement. The PR3 multi-organ (PR3-MO) cluster involved younger patients with anti-PR3 ANCA, severe acute respiratory failure and multiorgan failure. Renal replacement therapy was most frequent in the MPO-PR group (73%), while invasive mechanical ventilation was most common in the MPO-PR and PR3-MO groups (71% and 68%). Thirty-day mortality differed significantly: 23% (MPO-PR), 4% (mild-R cluster), and 14% (PR3-MO cluster).Conclusion
This large ICU cohort highlights three clinically relevant phenotypes of AAV-related DAH, which could inform the development of phenotype-driven immunosuppressive strategies.
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BibTeX
@article{gendreau2026phenotypic,
title = {{Phenotypic characterization of patients with ANCA-associated vasculitis and diffuse alveolar hemorrhage: a data-driven analysis of a multicentre, retrospective, nationwide cohort}},
author = {Segolene Gendreau and Anne-Claire Sanna and Cyrielle Desnos and Romain Lombardi and Jolan Malherbe and Dimitri Titeca-Beauport and Florian Reizine and Raphael Porcher and Benjamin Terrier and Nicolas de Prost},
journal = {Annals of Intensive Care},
year = {2026},
month = oct,
doi = {10.1016/j.aicoj.2026.100180},
url = {https://u-picardie.hal.science/hal-05795617v1},
note = {CC BY},
}
RIS
TY - JOUR
TI - Phenotypic characterization of patients with ANCA-associated vasculitis and diffuse alveolar hemorrhage: a data-driven analysis of a multicentre, retrospective, nationwide cohort
AU - Segolene Gendreau
AU - Anne-Claire Sanna
AU - Cyrielle Desnos
AU - Romain Lombardi
AU - Jolan Malherbe
AU - Dimitri Titeca-Beauport
AU - Florian Reizine
AU - Raphael Porcher
AU - Benjamin Terrier
AU - Nicolas de Prost
PY - 2026
DA - 2026/10/05
JO - Annals of Intensive Care
DO - 10.1016/j.aicoj.2026.100180
UR - https://u-picardie.hal.science/hal-05795617v1
AB - Background Severe diffuse alveolar hemorrhage (DAH) is a potentially fatal complication of ANCA-associated vasculitides (AAV). This study aimed to identify distinct clinical phenotypes among patients to the ICU, and to examine their association with mortality.Methods We conducted an ancillary analysis of the national multicenter retrospective PLEXHIA cohort, including 197 patients with severe AAV-related DAH, defined by respiratory symptoms with new infiltrates consistent with alveolar hemorrhage, along with oxygen saturation ≤85%, high-flow oxygen or mechanical ventilation requirement. Kohonen self-organizing maps were applied to predefined clinical variables at ICU admission, excluding treatment and outcomes. Clusters were interpreted and compared regarding organ involvement, severity, management, and mortality at 30 and 90 days.Results Between 2012 and 2024, 197 patients (66 years old, 52% female) were included, revealing marked clinical heterogeneity. Three phenotypes emerged: The MPO pulmonary-renal (MPO-PR) cluster included patients with anti-MPO ANCA, renal involvement and high oxygen requirements. The mild-respiratory (Mild-R) cluster included predominantly female patients with less severe respiratory compromise and mild renal involvement. The PR3 multi-organ (PR3-MO) cluster involved younger patients with anti-PR3 ANCA, severe acute respiratory failure and multiorgan failure. Renal replacement therapy was most frequent in the MPO-PR group (73%), while invasive mechanical ventilation was most common in the MPO-PR and PR3-MO groups (71% and 68%). Thirty-day mortality differed significantly: 23% (MPO-PR), 4% (mild-R cluster), and 14% (PR3-MO cluster).Conclusion This large ICU cohort highlights three clinically relevant phenotypes of AAV-related DAH, which could inform the development of phenotype-driven immunosuppressive strategies.
SN - 2110-5820
ER -