Phenotypic characterization of patients with ANCA-associated vasculitis and diffuse alveolar hemorrhage: a data-driven analysis of a multicentre, retrospective, nationwide cohort - 05/10/26

Doi : 10.1016/j.aicoj.2026.100180

Segolene Gendreau ^a,^b,^c,^{^{2
Contributed equally as first authors.},}⁎ , Anne-Claire Sanna ^d,^{^{2
Contributed equally as first authors.}}, Cyrielle Desnos ^e, Romain Lombardi ^f, Jolan Malherbe ^g, Dimitri Titeca-Beauport ^h, Florian Reizine ⁱ, Raphael Porcher ^j, Benjamin Terrier ^d,^k,^{^{1
Contributed equally as last authors.}}, Nicolas de Prost ^a,^b,^c,^l,^{^{1
Contributed equally as last authors.}}

on behalf of the PLEXHIA investigators

^a Université Paris-Est-Créteil (UPEC), Faculté de Santé, Créteil, France

^b Service de Médecine intensive et réanimation, Hôpitaux Universitaires Henri Mondor, DHU ATVB, 94000 Créteil, Assistance Publique - Hôpitaux de Paris

^c Université Paris Est Créteil, Institut Mondor de Recherche Biomédicale, Groupe de recherche clinique CARMAS, 94000 Créteil, France

^d Service de Médecine Interne, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris

^e Service de Médecine Intensive Réanimation, Hôpital Tenon, APHP Sorbonne Université

^f Service de Médecine Intensive Réanimation, CHU de Nice

^g Service de Médecine Intensive Réanimation, CHU de Caen Normandie

^h Service de Néphrologie et Service de Médecine Intensive et Réanimation, CHU Amiens Picardie

ⁱ Service de Réanimation, Centre Hospitalier de Vannes

^j Centre d’Épidémiologie Clinique, Hôtel-Dieu, AP-HP, Paris, France

^k Université Paris Cité, F-75006 Paris, France

^l INSERM U955, Team « Viruses, Hepatology, Cancer », Créteil, France

^⁎ Corresponding author.

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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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Keywords : ANCA-associated vasculitis, alveolar hemorrhage, intensive care