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Abstract Number: 0826

Plasma Exchange for Severe Alveolar Hemorrhage in ANCA-Associated Vasculitis: Emulation of a Target Trial

Anne-Claire Sanna1, Ségolène Gendreau2, Cyrielle Desnos3, Dimitri Titeca-Beauport4, Jolan Malherbe5, Florian Reizine6, Kladoum Nassarmadji7, Louis Guillaume8, Alban Deroux9, Eddine Bendiab10, Jennifer Catano11, Julien Campagne12, Marc Pineton de Chambrun13, Valentin Coirier14, Clara Vigneron11, Maxime SAMSON15, Antoine Neel16, Benoît Brilland17, Jean-Philippe Martellosio18, Maxim Desgrouas19, Olivier Simon20, Perrine SMETS21, Romain Paule22, Stanislas Faguer23, Stéphane Giorgiutti24, Thylbert Deltombe20, François Arrive18, Ingrid Masson25, Thomas Quemeneur26, Cécile Audrey Durel27, Etienne de Montmollin11, François Perrin28, Herve Lobbes29, Quentin Lajoye30, Rafik Mesbah31, Rodéreau Outh32, Adeline Lacraz33, Damien Contou34, Fabien Parazols35, Florian Garo36, Grégoire Ducoux37, Halil Yildiz38, Isabelle Rey39, Viviane Queyrel40, Rita Eid11, Robin Arcani41, Xavier Puéchal42, Raphael Porcher43, Nicolas de Prost11 and Benjamin Terrier44, and French Vasculitis Study Group (FVSG); Groupe de Recherche en Réanimation Respiratoire du patient d'Onco-Hématologie (GrrrOH), 1Cochin Hospital, Paris, France, 2Mondor Hospital, Paris, 3Tenon Hospital, Paris, France, 4CHU Amiens, Amiens, France, 5CHU Caen, Caen, France, 6CHU Vannes, Vannes, 7APHP, Paris, 8Hopital de Metz, Metz, France, 9Clinique Universitaire de Médecine Interne, Department of Internal Medicine, Grenoble University Hospital, Grenoble, France, 10CHU Montpellier, Montpellier, France, 11APHP, Paris, France, 12Hôpital Robert Schuman - Competence center for autoimmune diseases, Internal Medicine, Metz, France, 13Sorbonne Université, Paris, France, 14CHU Rennes, Rennes, France, 15Dijon University Hospital, Dijon, France, 16Department of Internal Medicine, CHU Nantes, Nantes, France, 17CHU Angers, Angers, France, 18CHU Poitiers, Poitiers, France, 19CHU Orléans, Orleans, France, 20CHU Réunion, Réunion, France, 21Clermont Ferrand University Hospital - National reference center for autoimmune disease, Internal Medicine, Clermont-Ferrand, France, 22Foch Hospital, Suresnes, France, 23CHU Toulouse, Toulouse, France, 24CHRU Strasbourg, Strasbourg, France, 25CHU Saint Etienne, Saint Etienne, France, 26CH Valenciennes, Valenciennes, France, 27HCL, Lyon, France, 28CH Saint Nazaire, Saint Nazaire, France, 29CHU Clermont-Ferrand, Clermont-Ferrand, France, 30CHU Bordeaux, Bordeaux, France, 31CH Boulogne, Boulogne, France, 32CH, Perpignan, France,, Perpignan, France, 33CH Bayonne, Bayonne, France, 34CH Argenteuil, Argenteuil, France, 35CH Perpignan, Perpignan, France, 36CHU Nimes, Nimes, France, 37CHU Lyon, Lyon, France, 38Université de Louvain, Louvain, Belgium, 39CH Annecy, Annecy, France, 40CHU Nice, Nice, France, 41APHM, Marseille, France, 42National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, 43Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, 44Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

Meeting: ACR Convergence 2024

Keywords: ANCA associated vasculitis

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Session Information

Date: Saturday, November 16, 2024

Title: Abstracts: Vasculitis – ANCA-Associated I

Session Type: Abstract Session

Session Time: 1:00PM-2:30PM

Background/Purpose: Mortality is high in patients with diffuse alveolar hemorrhage (DAH) associated with ANCA-associated vasculitis (AAV). In the PEXIVAS study, plasma exchange (PLEX) did not have a significant impact on the occurrence of death and/or end-stage kidney disease. However, less than 9% had severe DAH. In these patients, death was numerically more common in the absence of PLEX, but did not show a statistically significant difference, possibly due to the small number of events. The objective of this study was to evaluate whether PLEX reduces mortality in patients with severe DAH by emulating a target trial.

Methods: We emulated a target trial using observational data from a national multicenter retrospective database including patients with severe AAV-associated DAH. The primary outcome was 30-day mortality after ICU admission. The primary endpoint was analyzed using a Cox model to account for censored data with adjustment for pre-specified confounders. To emulate a clinical trial, we performed inverse probability weighting using a Cox regression model with prespecified variables (age, ANCA, BVAS, creatinine level >300µM, hemoptysis, SAPSII score, invasive mechanical ventilation during the first 7 days after ICU admission, and methylprednisolone pulses).

Results: We included 184 patients from 42 centers. One hundred forty-four (78.3%) patients received PLEX (PLEX group) and 40 (21.7%) patients did not receive PLEX (no-PLEX group). 

Baseline characteristics included median age 66 years [53, 75], female sex 51%, granulomatosis with polyangiitis in 49.5% and MPO-ANCA in 53.3%. Severe DAH was characterized by pulmonary infiltrates in 87.7%, hemorrhagic bronchoalveolar lavage in 71.2%, hemoptysis in 69.6%, and a hemoglobin level of 7.5 [6.6, 8.6] g/dL. Baseline characteristics were similar between the two groups except for more severe renal impairment with a higher creatinine level in the PLEX group (357 µmol/L [IQR 217-511] vs. 171 µmol/L [IQR 84-514], p=0.01). Severity of patients at ICU admission and supportive care was similar between the two groups, with a median SAPS II score of 42 [IQR 31-55] and the need for invasive mechanical ventilation (IMV) in 54% of patients. Intravenous cyclophosphamide was used more frequently in the PLEX group (77% vs. 55%, p=0.01). 

Eighty-five percent of patients were alive at 30 days and 73% were alive at 6 months. There was no difference in 30-day survival according to the use of PLEX or not in the emulated cohort, with 30-day survival of 85% (95%CI 81-90) in the PLEX group and 88% (95%CI 77-96) in the no-PLEX group (HR 1.23, 95%CI 0.57-3.89).

Conclusion: This large study of severe AAV-associated DAH shows that PLEX are still widely used and that 30-day mortality is 15%. The target trial emulation study showed that the use of PLEX did not affect early mortality in patients with severe AAV-associated DAH.


Disclosures: A. Sanna: None; S. Gendreau: None; C. Desnos: None; D. Titeca-Beauport: None; J. Malherbe: None; F. Reizine: None; K. Nassarmadji: None; L. Guillaume: None; A. Deroux: None; E. Bendiab: None; J. Catano: None; J. Campagne: None; M. Pineton de Chambrun: None; V. Coirier: None; C. Vigneron: None; M. SAMSON: AstraZeneca; Novartis; CSL Vifor; GlasgowSmithKline; Chugai; BOEHRINGER; FRESENIUS KABI, 2; A. Neel: Amgen, 2, AstraZeneca, 2, CSL Vifor, 2, GSK, 2, Sanofi, 2; B. Brilland: None; J. Martellosio: None; M. Desgrouas: None; O. Simon: None; P. SMETS: None; R. Paule: None; S. Faguer: None; S. Giorgiutti: None; T. Deltombe: None; F. Arrive: None; I. Masson: None; T. Quemeneur: None; C. Durel: None; E. de Montmollin: None; F. Perrin: None; H. Lobbes: None; Q. Lajoye: None; R. Mesbah: None; R. Outh: None; A. Lacraz: None; D. Contou: None; F. Parazols: None; F. Garo: None; G. Ducoux: None; H. Yildiz: None; I. Rey: None; V. Queyrel: None; R. Eid: None; R. Arcani: None; X. Puéchal: None; R. Porcher: None; N. de Prost: None; B. Terrier: AstraZeneca, 2, GlaxoSmithKline, 2, Novartis, 2, Vifor Pharma, 2.

To cite this abstract in AMA style:

Sanna A, Gendreau S, Desnos C, Titeca-Beauport D, Malherbe J, Reizine F, Nassarmadji K, Guillaume L, Deroux A, Bendiab E, Catano J, Campagne J, Pineton de Chambrun M, Coirier V, Vigneron C, SAMSON M, Neel A, Brilland B, Martellosio J, Desgrouas M, Simon O, SMETS P, Paule R, Faguer S, Giorgiutti S, Deltombe T, Arrive F, Masson I, Quemeneur T, Durel C, de Montmollin E, Perrin F, Lobbes H, Lajoye Q, Mesbah R, Outh R, Lacraz A, Contou D, Parazols F, Garo F, Ducoux G, Yildiz H, Rey I, Queyrel V, Eid R, Arcani R, Puéchal X, Porcher R, de Prost N, Terrier B. Plasma Exchange for Severe Alveolar Hemorrhage in ANCA-Associated Vasculitis: Emulation of a Target Trial [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/plasma-exchange-for-severe-alveolar-hemorrhage-in-anca-associated-vasculitis-emulation-of-a-target-trial/. Accessed .
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