A RETROSPECTIVE DESCRIPTIVE ANALYSIS OF SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE AND PULMONARY HYPERTENSION

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Patients with systemic sclerosis (SSc) can present with pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), or a combination of both ILD and pulmonary hypertension (PH). Little is known about the frequency of those who present with each disorder individually and subsequently develop the concurrent condition. We sought to examine the presentation, progression and prognosis between these phenotypes in a cohort of SSc patients. METHODS: We examined a cohort of SSc patients from 2006 to 2016. We further classified this group into those who presented with SSc-ILD alone, PAH alone, and SSc-ILD concomitant with PH on initial consult. Using the most recent right heart catheterization data (RHC), high-resolution CT (HRCT) findings and expert census from the electronic medical record (EMR), we tracked the progression of those who developed ILD and PH. Kaplan-Meier (KM) curves were generated to assess 3-year transplant-free survival with the log-rank test used to compare differences between the groups. RESULTS: There were 53 patients in our cohort who were categorized into 3 groups as follows: SSc-ILD - PH (n = 14), SSc - PAH (n = 13), and SSc-ILD (n = 26). In the SSc – PAH group, 54% (n = 7) went on to develop ILD, while in the SSc-ILD group 38% (n = 10) subsequently developed PH. The 3-year survival for the groups with SSc-ILD - PH (n = 14), SSc-PAH who went onto to develop ILD (n = 7), SSc-PAH who did not go on to develop ILD (n = 6), SSc-ILD who went on to develop PH (n = 10), and SSc-ILD who did not go on to develop PH (n = 16) was 85%, 85%, 83%, 68%, 93%, respectively (p-value = 0.16). CONCLUSIONS: In our cohort of SSc, over half the patients who presented with SSc and PAH developed ILD subsequently. There was a nonsignificant trend towards decreased transplant-free survival in the group who presented with SSc-ILD and went on to develop PH. CLINICAL IMPLICATIONS: The recent approval of the antifibrotic agent Nintedanib for those with SSc-related ILD has opened the door for a new era of disease-modifying therapy. The large percentage of patients with PAH who subsequently developed ILD highlights the importance of monitoring for this and highlights the potential importance of a combined PH and ILD clinic as SSc patients’ disease trajectories are often intertwined. DISCLOSURES: No relevant relationships by Scott Barnett, source=Web Response Advisory Committee Member relationship with Boehringer Ingelheim Please note: $5001 - $20000 Added 04/16/2020 by Christopher King, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Actelion Please note: $5001 - $20000 Added 04/16/2020 by Christopher King, source=Web Response, value=Honoraria Advisory Committee Member relationship with Actelion Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Boehringer Ingelheim pharmaceuticals Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Actelion Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boehringer Ingelheim Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Genentech Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Honoraria Advisory Committee Member relationship with United therapeutics Please note: $1001 - $5000 Added 04/27/2020 by Christopher King, source=Web Response, value=Consulting fee Consultant relationship with Veracyte Please note: $1001 - $5000 Added 04/03/2020 by Steven Nathan, source=Web Response, value=Honoraria Consultant relationship with United Therapeutics Please note: $5001 - $20000 Added 05/27/2020 by Steven Nathan, source=Web Response, value=Consulting fee Consultant relationship with Bellerophon Please note: $5001 - $20000 Added 05/27/2020 by Steven Nathan, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Roche-Genentech Please note: $5001 - $20000 Added 05/27/2020 by Steven Nathan, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boerhinger-Ingelheim Please note: $20001 - $100000 Added 05/27/2020 by Steven Nathan, source=Web Response, value=Honoraria No relevant relationships by Ankush Ratwani, source=Web Response No relevant relationships by Anthony Rowe, source=Web Response Scientific Medical Advisor relationship with Johnson & Johnson Please note: $1001 - $5000 Added 04/22/2020 by Oksana Shlobin, source=Web Response, value=Consulting fee Scientific Medical Advisor relationship with Bayer Please note: $1001 - $5000 Added 04/22/2020 by Oksana Shlobin, source=Web Response, value=Grant/Research Support Scientific Medical Advisor relationship with United Therapeutics Please note: $1001 - $5000 Added 04/22/2020 by Oksana Shlobin, source=Web Response, value=Consulting fee