Epidemiological Study of Pulmonary Hypertension in Patients With Interstitial Lung Diseases (PH-ILD) in Greece: A Retrospective Multicenter Study
Author(s)
Athanasios Zacharias, MD, MSc1, Afroditi Boutou, MD, MSc, PhD1, Sofia Zafeiri, MSc2, Panagiotis Papapetrou, MSc2, Antonis Georgiou, MSc2, Eirini Kefalidou, MSc2, Anastasia Anthi, MD, PhD3, Effrosyni Dima, MD, PhD3, Frantzeska Frantzeskaki, PhD4, Thomas Chrysochoidis, MD1, Panagiotis Gourgiotis, MD1, Panagiotis Kariofillis, MD, PhD5, Eftychia Demerouti, MD, MMedSc, PhD5, Ioanna Mitrouska, MD, PhD6, Vaia Stamatopoulou, MD6, Eleni Stagaki, PhD7, George Giannakoulas, MD1, Iraklis Tsagkaris, PhD4, Georgia Pitsiou, MD, MSc, PhD1.
1Aristotle University of Thessaloniki, Thessaloniki, Greece, 2Galenica S.A., Kifisia, Greece, 3Evangelismos Hospital, Athens, Greece, 4National and Kapodistrian University of Athens, Athens, Greece, 5Onassis Hospital, Athens, Greece, 6University Hospital of Heraklion, Heraklion, Greece, 7Sotiria Chest Hospital, Athens, Greece.
1Aristotle University of Thessaloniki, Thessaloniki, Greece, 2Galenica S.A., Kifisia, Greece, 3Evangelismos Hospital, Athens, Greece, 4National and Kapodistrian University of Athens, Athens, Greece, 5Onassis Hospital, Athens, Greece, 6University Hospital of Heraklion, Heraklion, Greece, 7Sotiria Chest Hospital, Athens, Greece.
OBJECTIVES: To describe the epidemiological indicators, as well as the demographic characteristics, comorbidities, and clinical outcomes of patients diagnosed with PH-ILD in Greece.
METHODS: A retrospective cohort study was conducted using anonymized data collected via structured questionnaires from all seven nationally recognized reference centers for PH-ILD in Greece. Adult patients (≥18 years) with a confirmed diagnosis of both pulmonary hypertension (ICD-10: I27.0/I27.2) and interstitial lung disease (ICD-10: J84 series) between January 2022 and December 2024 were included. Patients diagnosed solely based on transthoracic echocardiography were excluded. Demographic characteristics, comorbidities, and clinical outcomes -including pulmonary vascular resistance (PVR) and 6-minute walk distance (6MWD)- were analyzed.
RESULTS: A total of 69 adult patients with PH-ILD were identified. The mean age at diagnosis was 65.9 years (SD: 10.9), and 39% were female. The most common ILD subtypes were connective tissue disease-associated ILD (CTD-ILD, 43%) and idiopathic pulmonary fibrosis (IPF, 33%). Frequent comorbidities included hypertension / coronary artery disease (42%), diabetes (19%), and gastroesophageal reflux disease (19%). The mean PVR was 6.47 Wood units (WU), with 54% of patients presenting with PVR ≥5 WU. The average 6MWD at diagnosis was 275.3 meters (SD: 100.6), with 53% walking < 300 meters. Twelve patients (17%) had died by data collection, with a higher mean PVR among deceased (8.66 WU) versus survivors (6.02 WU).
CONCLUSIONS: This multicenter study provides real-world data on patients diagnosed with PH-ILD in Greece across all national reference centers. Most patients were diagnosed with poor functional and hemodynamic status, with over half presenting with PVR ≥5 WU and a 6MWD under 300 meters. Furthermore, a 17% mortality rate within a short follow-up period underscores the rapid progression and poor prognosis of the disease. These findings emphasize the need for increased disease awareness and earlier detection.
METHODS: A retrospective cohort study was conducted using anonymized data collected via structured questionnaires from all seven nationally recognized reference centers for PH-ILD in Greece. Adult patients (≥18 years) with a confirmed diagnosis of both pulmonary hypertension (ICD-10: I27.0/I27.2) and interstitial lung disease (ICD-10: J84 series) between January 2022 and December 2024 were included. Patients diagnosed solely based on transthoracic echocardiography were excluded. Demographic characteristics, comorbidities, and clinical outcomes -including pulmonary vascular resistance (PVR) and 6-minute walk distance (6MWD)- were analyzed.
RESULTS: A total of 69 adult patients with PH-ILD were identified. The mean age at diagnosis was 65.9 years (SD: 10.9), and 39% were female. The most common ILD subtypes were connective tissue disease-associated ILD (CTD-ILD, 43%) and idiopathic pulmonary fibrosis (IPF, 33%). Frequent comorbidities included hypertension / coronary artery disease (42%), diabetes (19%), and gastroesophageal reflux disease (19%). The mean PVR was 6.47 Wood units (WU), with 54% of patients presenting with PVR ≥5 WU. The average 6MWD at diagnosis was 275.3 meters (SD: 100.6), with 53% walking < 300 meters. Twelve patients (17%) had died by data collection, with a higher mean PVR among deceased (8.66 WU) versus survivors (6.02 WU).
CONCLUSIONS: This multicenter study provides real-world data on patients diagnosed with PH-ILD in Greece across all national reference centers. Most patients were diagnosed with poor functional and hemodynamic status, with over half presenting with PVR ≥5 WU and a 6MWD under 300 meters. Furthermore, a 17% mortality rate within a short follow-up period underscores the rapid progression and poor prognosis of the disease. These findings emphasize the need for increased disease awareness and earlier detection.
Conference/Value in Health Info
2025-11, ISPOR Europe 2025, Glasgow, Scotland
Value in Health, Volume 28, Issue S2
Code
CO98
Topic
Clinical Outcomes, Epidemiology & Public Health, Real World Data & Information Systems
Topic Subcategory
Performance-based Outcomes
Disease
Rare & Orphan Diseases