MORPHOLOGICAL INDICES ASSOCIATED WITH PULMONARY HYPERTENSION AND MORTALITY PREDICTION IN INTERSTITIAL LUNG DISEASE

TOPIC: Diffuse Lung Disease TYPE: Original Investigations PURPOSE: Pulmonary hypertension (PH) is common in interstitial lung disease (ILD) and associated with increased morbidity mortality. PH detected by echocardiography (ECHO) impacts survival patients ILD. The mortality association of other surrogate indices PH, such as clinical, serologic, histopathologic, radiographic biomarkers, remains unclear. METHODS: Indiana University (IRB#11488) Chicago ILD (IRB#17-1617) databases were screened to identify histopathologic specimens obtained from biopsies or at the time transplantation 2006-2019. Demographic, data electronic medical record (EMR). artery: aorta (PA/A) ratio was evaluated on computed tomography (CT) scans. Morphological deemed present upon identification any following parameters: (a) PA/A >0.9 CT; (b) right ventricular systolic pressure >40 mmHg ECHO; (c) NT-proBNP >300pg/uL; (d) pulmonary vasculopathy histology. Patients these compared those without. Six-minute walk distance (6MWD) also assessed. Transplant-free analyzed using Cox regression models. RESULTS: 568 (idiopathic fibrosis, n=260, connective tissue disease-ILD, n=75, hypersensitivity pneumonitis, n=102, pneumonia autoimmune features, n=80, ILDs, n=51) identified. 71% (n=401). These had lower forced vital capacity (FVC, 59% vs 65%; P<0.001), diffusing (DLCO, 44% 50%; shorter 6MWD (320m 356m; P=0.002), more frequently required supplemental oxygen (65% 46%; greater prevalence CT honeycombing (52% 34%; P<0.001) when without.In unadjusted analyses, all morphological assessed predicted In contrast, only histologic remained predictive fully adjusted models that accounted for age, sex, FVC, DLCO, subtype, BMI, immunosuppression, smoking, hypothyroidism, coronary artery disease. >450m, index absence improved (HR=0.55; 95%CI=0.34-0.89; P=0.016; HR=0.74; 95%CI=0.56-0.98; P=0.039, respectively) analyses. Presence combined a 6MWD<450m highest risk (HR=2.31; 95%CI=1.83-2.92; P<0.001). CONCLUSIONS: Radiologic are comparatively stronger predictors than ECHO CLINICAL IMPLICATIONS: PH-associated could be valuable surrogates identifying earlier intervention DISCLOSURES: Advisory Committee Member relationship Genentech Please note: 2019 Added 04/26/2021 Ayodeji Adegunsoye, source=Web Response, value=Consulting fee Boehringer Ingelheim 2018-2021 04/29/2021 Speaker/Speaker's Bureau value=Honoraria Consultant 2018-2020 No relevant relationships Remzi Bag, Response Linda Bocanegra-English, 12 months 04/20/2021 Ryan Boente, Veracyte $1001 - $5000 Jonathan Chung, Riverain current 06/02/2021 john donatelli, Aliya Husain, Cathryn Lee, Steven Montner, Kavitha Selvan, Danielle Stahlbaum, PI Boehringer-Ingelheim >$100000 Mary Strek, value=Grant/Research Support Galapagos $70,000-100,00 05/03/2021 Novartis $5001 $20000 Removed Endpoint Adjudication Fibrogen $1-$1000 Rekha Vij,