Respiratory failure & ventilation

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

Ann Intensive CarePublished ancillary analysis of the national multicenter retrospective PLEXHIA cohort

Authors' conclusion

This large ICU cohort highlights three clinically relevant phenotypes of AAV-related DAH, which could inform the development of phenotype-driven immunosuppressive strategies.

Study context

Population
Patients in the ICU with severe ANCA-associated vasculitis-related diffuse alveolar hemorrhage (respiratory symptoms with new infiltrates consistent with alveolar hemorrhage, along with oxygen saturation ≤85%, high-flow oxygen or mechanical ventilation requirement), 2012–2024patients with severe ANCA-associated vasculitides (AAV)-related diffuse alveolar hemorrhage (DAH) included (66 years old, 52% female)
Participants
197 patients
Exposure
Clusters compared regarding organ involvement, severity, management, and mortality at 30 and 90 days
Outcome
Renal replacement therapy
Methodological detailsKohonen self-organizing maps, data-driven
  • Kohonen self-organizing maps
  • Data-driven

Main finding

As reported in the abstract

MPO pulmonary-renal (MPO-PR)

73%

Observational data: associations, not effects.

Further findings

Mortality · 30 days

OutcomeMPO-PRMild-RPR3-MO
Invasive mechanical ventilation71%— not stated in the abstract68%

— not stated in the abstract

Invasive mechanical ventilation

Most common in the MPO-PR and PR3 multi-organ (PR3-MO) groups

Mortality · 30 days

Thirty-day mortality differed significantly

Clinical phenotypes

Marked clinical heterogeneity; three phenotypes emerged

Original publication

Phenotypic characterization of patients with ANCA-associated vasculitis and diffuse alveolar hemorrhage: a data-driven analysis of a multicentre, retrospective, nationwide cohort. Ann Intensive Care. Published 5 October 2026. doi:10.1016/j.aicoj.2026.100180

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