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Review
. 2021 Apr 23;14(5):403.
doi: 10.3390/ph14050403.

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

Barbara Ruaro  1 , Marco Confalonieri  1 , Francesco Salton  1 , Barbara Wade  2 , Elisa Baratella  3 , Pietro Geri  1 , Paola Confalonieri  1 , Metka Kodric  1 , Marco Biolo  1 , Cosimo Bruni  4
Affiliations
Review

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

Barbara Ruaro et al. Pharmaceuticals (Basel). .

Abstract

Systemic sclerosis (SSc) is an autoimmune disease, characterized by the presence of generalized vasculopathy and tissue fibrosis. Collagen vascular disorder in SSc is due to fibroblast and endothelial cell dysfunctions. This leads to collagen overproduction, vascular impairment and immune system abnormalities and, in the last stage, multi-organ damage. Thus, to avoid organ damage, which has a poor prognosis, all patients should be carefully evaluated and followed. This is particularly important in the initial disease phase, so as to facilitate early identification of any organ involvement and to allow for appropriate therapy. Pulmonary disease in SSc mainly involves interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). High-resolution computed tomography (HRCT) and pulmonary function tests (PFT) have been proposed to monitor parenchymal damage. Although transthoracic echocardiography is the most commonly used screening tool for PAH in SSc patients, definitive diagnosis necessitates confirmation by right heart catheterization (RHC). Moreover, some studies have demonstrated that nailfold videocapillaroscopy (NVC) provides an accurate evaluation of the microvascular damage in SSc and is able to predict internal organ involvement, such as lung impairment. This review provides an overview of the correlation between lung damage and microvascular involvement in SSc patients.

Keywords: interstitial lung disease; microvascular involvement; nailfold capillaroscopy; pulmonary arterial hypertension; pulmonary involvement; systemic sclerosis.

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Conflict of interest statement

The authors declare that there are no conflict of interest.

Figures

Figure 1
Figure 1
A 43-year-old female with a diagnosis of systemic sclerosis. Axial high-resolution CT scan obtained in the supine position shows subtle ground-glass opacities in the subpleural regions of the lung bases, suspicious for NSIP (non-specific interstitial pneumonia) (a). When these findings are not prominent, an additional scan can be acquired in the prone position to differentiate ground-glass opacities due to gravitational phenomena from interstitial lung disease. In this patient the ground-glass opacities persist in the prone position, confirming the interstitial lung involvement (b). Additional systemic sclerosis-related findings should be searched for, such as a dilated esophagus on images reconstructed using an appropriate mediastinal window setting (c).
Figure 2
Figure 2
From below: pulmonary arterial pressure, respiratory and EKG waveforms during arterial catheterization. The first part of the pressure trace reflects the pressure in a pulmonary artery (large swings, dicrotic notch), then the balloon is inflated and the tip of the Swan Ganz catheter floats until it wedges in a small artery (small swings synchronous with respiratory rate), allowing a pulmonary arterial wedge pressure (PAWP) to be obtained, which is an indirect measure of left ventricle pressure.
Figure 3
Figure 3
Vasculopathy in systemic sclerosis patients and the link between pulmonary damage and peripheral vascular manifestations.
See this image and copyright information in PMC

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