Abstract
ObjectiveTo prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function.MethodsSeventy-four patients with SSc undergoing chest CT...
Highlights
Systemic sclerosis (SSc) is a multisystem connective tissue disease that may affect the lung with interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH).[1]
►► Our study demonstrates that patients with systemic sclerosis (SSc) have different patterns of pulmonary vascular changes at spirometry-gated CT scans, with pulmonary vascular volume significantly increased in patients with diffusion lung capacity for carbon monoxide (DLco)% reduction and even more in those with a restrictive pattern
Among the clinical- laboratoristic parameters, pulmonary vascular volume (PVV)/LV correlated with those related to cardiovascular involvement and to scores related to disease activity, disability and quality of life (VAS disease activity, Heart Assessment Questionnaire (HAQ), HAQ for walking, EQ-5D-5L)
Summary
Systemic sclerosis (SSc) is a multisystem connective tissue disease that may affect the lung with interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH).[1]. ►► Our study demonstrates that patients with SSc have different patterns of pulmonary vascular changes at spirometry-gated CT scans, with pulmonary vascular volume significantly increased in patients with DLco% reduction and even more in those with a restrictive pattern. Recent technological advances in CT image post processing permitted non-invasive and fully automated quantitative analysis of pulmonary vasculature, without the use of contrast media and exposure to repeated ionising radiation during injection.[4,5,6,7] quantitative imaging requires standardised acquisition protocols and quality certified and validated image analysis methodology to ensure accuracy and precision of the quantitative information provided.[8] Among the sources of variation in quantifying chest radiological features, lung volume at acquisition time is the most relevant. Few studies used spirometry-gated CT to account for this possible source of variation.[9,10,11,12]
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