D23-13 Characteristics of Patients With Acute Exacerbation of Autoimmune Related Interstitial Lung Disease Requiring Mechanical Ventilation or Extracorporeal Membrane Oxygenation

Abstract Introduction The incidence of acute exacerbations of systemic autoimmune related disease-interstitial lung disease (AE-SARD-ILD) is approximately 7.2% per year. In-hospital mortality remains between 50%-100%. The highest mortality rate has been reported amongst patients requiring invasive mechanical ventilation (IMV) or extracorporeal membrane oxygenation (EMCO), both around 75%. While corticosteroids remain the backbone of treatment, their use has been extrapolated from the guidelines for treatment of acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). Newer data suggests that patients with non-IPF exacerbations have improved survival and lower recurrence rates with higher steroid doses. We hypothesize that higher dose steroids will decrease the need for IMV and ECMO in patients with AE-SARD-ILD. Methods We identified a retrospective cohort of patients from our institution admitted for AE-SARD-ILD from 2016-2024. Baseline demographic data was collected. Treatment data was collected including cumulative steroid dose, additional immunosuppression, need for IMV, and ECMO use. Outcome data collected included in-hospital mortality and transplant free survival. Results A total of 51 patients met criteria for inclusion. 19/51 (37%) required IMV and 9/51 (17.6%) required ECMO support. 8/9 (88%) were placed on ECMO as bridge to transplant. Overall, ECMO in-hospital mortality was 66.7%. 2/9 (22%) of the ECMO patients were transplanted and 1 recovered all of whom were discharged alive. Transplant free survival was 5/19 (26%) in the IMV group. 6/20 (30%) patients on either IMV or ECMO were on background antifibrotic therapy and 13/20 (65%) were on non-steroid background immunosuppression. Of the entire cohort, 35/51 (68%) of patients admitted with AE-SARD-ILD survived to discharge. Cumulative steroid dosing during admission ranged from 80 mg to 5510 mg for the entire cohort. Average daily steroid dose ranged from 18 mg/day to 240 mg/day. Discussion Our cohort showed reduced overall in-hospital mortality when compared with other published populations. Despite this, mortality amongst patients requiring IMV or ECMO remained high, representing most in-hospital mortality within this group and consistent with other published data. This highlights the need to develop effective therapeutic strategies both to prevent initiation of IMV and ECMO as well as effective strategies for management once patients require IMV and ECMO. There was marked heterogeneity in steroid dosing, which underscores the need for evidence-based guidance on optimal dosing. Future directions include analysis of steroid dosing prior to intensive care admission, IMV, or ECMO initiation compared with patients who did not require these interventions to better understand the effect of corticosteroids on these outcomes. This abstract is funded by: None