PROGNOSTICATION IN SCLERODERMA-RELATED PULMONARY DISEASE: A COMPARISON OF CURRENT MODELS

Abstract

SESSION TITLE: Systemic Disease and the Lung 2 SESSION TYPE: Original Investigations PRESENTED ON: 10/10/2018 02:30 PM - 03:30 PM PURPOSE: Scleroderma (SSc) is frequently associated with lung diseases, including Pulmonary Hypertension (PH) and Interstitial Lung Disease (ILD). The presence of pulmonary comorbidities is associated with a worse prognosis, emphasizing the necessity of evidence based prognostic tools. DIBOSA score is a new scoring system based on a 6-Minute Walk Test (6MWT) and is shown to predict mortality in Scleroderma. The objective of this study was to compare three scoring systems for prognostication in Scleroderma population: DIBOSA score (tested for all SSc patients), SADL model (developed for SSc-ILD) and REVEAL risk score (developed for PH). METHODS: A retrospective review of a Scleroderma registry at a single center was conducted. 286 consecutive Scleroderma patients presenting between 2003 and 2013 were initially reviewed. Aforementioned scores were calculated from available data. C-statistic was used to compare Area Under Receiver Operator Curve (AUROC) to predict a primary end point of 1-year mortality. DIBOSA score vs SADL model, and DIBOSA vs REVEAL scores were compared in SSc-ILD and SSc-PH populations respectively. RESULTS: In our data of 286 Scleroderma patients, average age was 54 years, 79% females, 142 (51.26%) had SSc-ILD, 129 (45.1%) had SSc-PH (all PH groups) and 41 (14.34%) had Scleroderma associated Pulmonary Arterial Hypertension (SSc-PAH). DIBOSA score and SADL model were compared in SSc-ILD population (n=118) resulting in an AUROC of 0.7977 and 0.7538 respectively (P=0.6699). DIBOSA and REVEAL scores were compared in SSc-PH populations, resulting in an AUROC of 0.6680 and 0.4060 respectively (P=0.0523, n=85). When compared in the SSc-PAH subgroup DIBOSA and REVEAL scores resulted in an AUROC of 0.7292 and 0.5975 respectively (P=0.5138, n=29). CONCLUSIONS: DIBOSA score was non-inferior to SADL model in prognostication of SSc-ILD. DIBOSA score was non-inferior to REVEAL risk score is SSc-PH and SSc-PAH populations. CLINICAL IMPLICATIONS: DIBOSA score is a more generalizable prognostication tool, and can be used in prognostication of all Scleroderma patients irrespective of pulmonary comorbidities; and is non-inferior to other models developed specifically for SSc-ILD (SADL model) and PAH (REVEAL score). This emphasizes the clinical utility of 6MWT in Scleroderma, which allows for the calculation of the DIBOSA score for prognostication. DISCLOSURES: No relevant relationships by Abhishek Gadre, source=Web Response Speaker/Speaker's Bureau relationship with Actelion Pharmaceuticals Please note: $20001 - $100000 Added 11/30/2017 by Kristin Highland, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Bayer Healthcare Please note: $5001 - $20000 Added 11/30/2017 by Kristin Highland, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Gilead Sciences Please note: $5001 - $20000 Added 11/30/2017 by Kristin Highland, source=Web Response, value=Honoraria Consultant relationship with Boehringer Ingelheim Please note: $20001 - $100000 Added 11/30/2017 by Kristin Highland, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with United Therapeutics Please note: $5001 - $20000 Added 11/30/2017 by Kristin Highland, source=Web Response, value=Honoraria No relevant relationships by Michael Smith, source=Web Response No relevant relationships by Ramya Vajapey, source=Web Response