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, Haruka Moriya Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Search for other works by this author on: Oxford Academic Masaru Kato Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Correspondence to: Masaru Kato, Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, N15W7, Kita-Ku, 060-8638 Sapporo, Japan. E-mail: ktmasaru@med.hokudai.ac.jp Search for other works by this author on: Oxford Academic Ryo Hisada Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Keita Ninagawa Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Maria Tada Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Kodai Sakiyama Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Mitsutaka Yasuda Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Michihito Kono Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Yuichiro Fujieda Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Olga Amengual Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic
https://orcid.org/0000-0003-2582-253X , Yasuka Kikuchi Department of Diagnostic Imaging, Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Ichizo Tsujino Department of Respiratory Medicine, Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Takahiro Sato Department of Respiratory Medicine, Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic Tatsuya Atsumi Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan Search for other works by this author on: Oxford Academic
Rheumatology, Volume 63, Issue 7, July 2024, Pages 1868–1873, https://doi.org/10.1093/rheumatology/kead485
Published:
15 September 2023
Article history
Received:
31 May 2023
Accepted:
23 August 2023
Published:
15 September 2023
Corrected and typeset:
29 September 2023
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Haruka Moriya, Masaru Kato, Ryo Hisada, Keita Ninagawa, Maria Tada, Kodai Sakiyama, Mitsutaka Yasuda, Michihito Kono, Yuichiro Fujieda, Olga Amengual, Yasuka Kikuchi, Ichizo Tsujino, Takahiro Sato, Tatsuya Atsumi, The chest CT signs for pulmonary veno-occlusive disease correlate with pulmonary haemodynamics in systemic sclerosis, Rheumatology, Volume 63, Issue 7, July 2024, Pages 1868–1873, https://doi.org/10.1093/rheumatology/kead485
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Abstract
Objectives
Pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc) sometimes accompanies pulmonary veno-occlusive disease (PVOD). We aimed to reveal the relationship between clinical signs of PVOD and severity of pulmonary vasculopathy in SSc.
Methods
This study included 52 consecutive SSc patients who had pulmonary haemodynamic abnormalities [mean pulmonary arterial pressure (mPAP) >20 mmHg, pulmonary vascular resistance >2 WU or pulmonary artery wedge pressure (PAWP) >15 mmHg]. A chest CT scan was evaluated in all patients. Patients were divided into two groups, the 0–1 group and the 2–3 group, according to the number of chest CT signs for PVOD, including mediastinal lymph node enlargement, thickened interlobular septal wall and ground glass opacity. Pulmonary haemodynamics, echocardiography and MRI-based cardiac function, pulmonary function and serum biomarkers were compared between the two groups.
Results
Mediastinal lymph node enlargement, thickened interlobular septal wall and ground glass opacity were observed in 11 (21%), 32 (62%) and 11 (21%) patients, respectively. The 2–3 group (n = 15) had higher mPAP (P = 0.02) but lower diffusing capacity of carbon monoxide (DLCO)/alveolar volume (P = 0.02) compared with the 0–1 group (n = 37). Other parameters, including PAWP, cardiac output, left ventricular ejection fraction, left atrial diameter, forced vital capacity, brain natriuretic peptide and Krebs von den Lunge-6 were not different between the two groups.
Conclusions
The CT signs for PVOD had a positive correlation with mPAP but a negative correlation with DLCO in SSc patients, indicating that PAH-SSc may reflect a spectrum of pulmonary vascular disease that ranges from the pulmonary artery to the vein.
pulmonary arterial hypertension, SSc, pulmonary veno-occlusive disease, chest CT
© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com
This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/pages/standard-publication-reuse-rights)
Issue Section:
Clinical Science
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