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

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.

High-Resolution Computed Tomography: Lights and Shadows in Improving Care for SSc-ILD Patients

“…Before the advent of Computed Tomography scanning (CT), the identification of ILD was often a difficult task, even if early autopsy studies reported that up to 100% of patients had parenchymal involvement. Indeed, as many as 90% of SSc-ILD patients have interstitial abnormalities on high-resolution computed tomography (HRCT) and 40-75% have changes in pulmonary function tests (PFT) [19][20][21][22]. The clinical history of these patients usually reveals the insidious onset of exertional dyspnoea and a nonproductive cough.…”

“…Considering the heterogeneity of SSc-ILD and the potential absence of symptoms, patients may be diagnosed earlier and more efficiently through a multidisciplinary evaluation and team collaboration [22][23][24][25][26][27][28][29]. Furthermore, it is prudent to consider as many parameters as possible to enhance clinical outcomes when assessing and monitoring newly diagnosed patients.…”

New Insights into Profibrotic Myofibroblast Formation in Systemic Sclerosis: When the Vascular Wall Becomes the Enemy

“…Lung vascular damage is mainly represented by PAH, a condition that plays an important role in SSc prognosis, as it is related to increased mortality [ 4 , 21 , 22 , 23 ]. Histologically, early pathologic lesions of PAH manifest with medial and intimal thickening, subsequent subintimal fibrosis in an onion-skin pattern and eventual reduplication of the internal elastic lamina [ 24 ], while later lesions comprise plexiform and “glomeruloid” nodules in small arteries with fibrin thrombi formation [ 24 ].…”

High-Resolution Computed Tomography and Lung Ultrasound in Patients with Systemic Sclerosis: Which One to Choose?

“…Systemic sclerosis (SSc) is a connective tissue disease characterised by immune activation, vasculopathy and fibrosis [ 1 , 2 , 3 , 4 , 5 , 30 , 31 , 32 ]. The fibrosis and vasculopathy processes are strictly involved in the development of SSc-ILD [ 1 , 2 , 3 , 4 , 5 , 30 , 31 , 32 ].…”