262. Clinical characteristics of participants with and without alveolar hemorrhage in the PEXIVAS trial
Creators: name: Fussner1, Lynn
name: Luis Felipe Flores-Suarez2,
name: Cartin-Ceba3, Rodrigo
name: Specks4, Ulrich
name: P. Gerard Cox5
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Alveolar hemorrhage (AH), a potentially life-threatening manifestation of ANCA-associated vasculitis (AAV), is characterized by bleeding into the distal air spaces of the lungs. Studies describing patients with AH in AAV are typically small and/or retrospective. Presented here are the baseline and outcome data for participants in the PEXIVAS trial with and without AH. <strong>Methods:</strong> Clinical data were collected prospectively from 704 participants with AAV at 95 centers in 16 countries in PEXIVAS, a randomized controlled trial comparing 7 sessions of plasma exchange versus none, along with two glucocorticoid regimens. For the present analysis, treatment groups were combined and participants grouped based on presence or absence of AH at enrollment. Baseline characteristics and 1 year outcomes are summarized and compared. Participants with AH were, <em>a priori</em>, subcategorized as severe AH if they had an oxygen saturation ≤85% on room air. <strong>Results:</strong> Among the 704 participants in PEXIVAS, 191 (27%) had AH and 513 (73%) did not have AH. Participants with AH were younger (mean age 61.1 years with AH vs. 63.9 without AH, p=0.018), more frequently proteinase 3-ANCA positive (51.5% vs. 36.5%, p&lt;0.001), and less frequently had newly-diagnosed (as opposed to relapsing) AAV (85.8% vs.93.0%, p=0.004). Participants with AH had higher baseline disease severity based on BVAS/WG (median 11 vs. 7, p&lt;0.001) and lower serum creatinine (median 184 vs. 304 µmol/L, p&lt;0.001), but were more frequently dialyzed at enrollment (25.3% vs. 18.0%, p=0.033). Additional baseline characteristics are presented in Table 1. Among the 191 participants with AH, 61 (31.9%) had severe AH, of which 29 (47.5%) required mechanical ventilation. Twenty-three (12.0%) participants with AH at enrollment died in the first year as compared to 34 (6.6%) without AH (p=0.019). Of the 23 deaths in the AH group, 16 (69.6%) occurred in those with severe AH. Among participants who died within 1 year of enrollment, 14 of 23 (60.9%) deaths among participants with AH occurred within 30 days of enrollment versus 11 of the 34 (32.4%) in those without AH (p=0.033). The risk of end-stage kidney disease (ESKD) and serious infections in the first year were similar between participants with and without AH (ESKD 16.0% vs. 13.1%, p=0.341; serious infections 41.4% vs. 39.2%, p=0.599). <strong>Conclusions:</strong> Based on the PEXIVAS trial cohort, patients with AAV and AH have a high overall degree of disease severity and differ from those without AH in multiple other ways, including age, ANCA specificity, and use of dialysis, and are more likely to die within 30 days and 1 year. These prospectively-collected data among participants with a wide spectrum of severity of AH provide important insights into the characteristics of this disease manifestation of AAV as will analysis of the impact on AH of the different treatment regimens in the PEXIVAS trial. <strong>Disclosures</strong>: Dr. Merkel reports the receiving funds for the following activities: Consulting and Research Support: AbbVie, AstraZeneca, Boeringher-Ingelheim, Bristol-Myers Squibb, ChemoCentryx, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Takeda. Consulting only: CSL Behring, Dynacure, EMDSerono, Jannsen, Kiniksa, Kyverna, Magenta, MiroBio, Neutrolis, Novartis, Pfizer, Sparrow, Talaris. Royalties: UpToDate. Remaining authors: None <strong>Table 1. </strong>Baseline characteristics of participants with and without alveolar hemorrhage enrolled in the PEXIVAS trial. P-values are based on crude associations. <strong>Characteristic</strong> <strong>Alveolar Hemorrhage n=191</strong> <strong>No Alveolar Hemorrhage n=513</strong> <strong>P-value</strong> Age, mean years (SD) 61.1 (15.4) 63.9 (13.4) 0.018 Female, n (%) 78 (40.8) 229 (44.6) 0.366 ANCA type, n (%) PR3 99 (51.8) 187 (36.5) &lt;0.001 MPO 92 (48.2) 326 (63.6) New diagnosis of AAV, n (%) 164 (85.8) 477 (93.0) 0.004 BVAS-WG, median (25<sup>th</sup>-75<sup>th</sup> percentile) 11 (9-13) 7 (6-9) &lt;0.001 Creatinine, median µmol/L<br> (25<sup>th</sup>-75<sup>th</sup> percentile) 184 (115-320) 304 (212-442) &lt;0.001 Dialysis at baseline, n (%) 48 (25.3) 92 (18.0) 0.033 Randomized to plasma exchange, n (%) 95 (49.7) 257 (50.1) 0.932 Randomized to reduced glucocorticoid, n (%) 96 (50.3) 257 (50.1) 0.969 Immunosuppression, n (%) Oral cyclophosphamide 44 (23.0) 197 (38.4) &lt;0.001 Intravenous cyclophosphamide 107 (56.0) 247 (48.2) Rituximab 40 (20.9) 69 (13.4) 1 Year Outcomes, n (%) Death 23 (12.0) 34 (6.6) 0.019 End-stage kidney disease 25 (13.1) 82 (16.0) 0.341 Serious infection 79 (41.4) 201 (39.2) 0.599