Abstract
Background Patients with pulmonary hypertension (PH) and lung disease may pose a diagnostic dilemma between idiopathic pulmonary arterial hypertension (IPAH) and PH associated with lung disease (PH-CLD). The prognostic impact of common CT parenchymal features is unknown.
Methods 660 IPAH and PH-CLD patients assessed between 2001–19 were included. Reports for all CT scans one year prior to diagnosis were analysed for common lung parenchymal patterns. Cox regression and Kaplan-Meier analysis was performed.
Results At univariate analysis of the whole cohort, centrilobular ground glass (CGG) changes (Hazard Ratio, HR 0.29) and ground glass opacification (GGO, HR 0.53) predicted improved survival while honeycombing (HR 2.79), emphysema (HR 2.09) and fibrosis (HR 2.38) predicted worse survival (p all <0.001). Fibrosis was an independent predictor after adjusting for baseline demographics, PH severity and DLco (HR 1.37, p<0.05). Patients with a clinical diagnosis of IPAH who had an absence of reported parenchymal lung disease (IPAH-noLD) demonstrated superior survival to patients diagnosed with either IPAH who had coexistent CT lung disease or PH-CLD (2-year survival of 85%, 60% and 46% respectively, p<0.05). CGG changes were present in 23.3% of IPAH-noLD and 5.8% of PH-CLD patients. There was no significant difference in survival between IPAH-noLD patients with or without CGG changes. PH-CLD patients with fibrosis had worse survival than those with emphysema.
Interpretation Routine clinical reports of CT lung parenchymal disease identify groups of patients IPAH and CLD-PH with significantly different prognoses. Isolated CGG changes are not uncommon in IPAH but are not associated with worse survival.
Footnotes
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Conflicts of Interest: Conflict of interest: D.G. Kiely reports grants, personal fees and funds for meeting attendance from Janssen and GSK, personal fees and funds for meeting attendance from MSD, personal fees from Acceleron, outside the submitted work. R. Condliffe reports personal fees and funds for meeting attendance from Janssen, GSK and MSD, outside the submitted work. A. Swift reports honoraria and consultancy fees from Janssen Pharmaceuticals, and received a research grant from GlaxoSmithKline. R.A. Lewis reports non-financial support from Actelion Pharmaceuticals, outside the submitted work. KD, MS, SA, SR, CH, LS, PM, FA, DA, JMW and HU report no conflict of interest.
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- Received September 9, 2021.
- Accepted December 7, 2021.
- Copyright ©The authors 2021
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