Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis.

Domenico Sambataro,Nicoletta Del Papa,Gianluca Sambataro,Francesca Pignataro,Lorenzo Malatino,Carlo Vancheri,Michele Colaci,Wanda Maglione

doi:10.3390/diagnostics10040225

Abstract

Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients.

Highlights

IntroductionPulmonary involvement is the primary cause of death in Systemic sclerosis (SSc) patients [1]
Systemic sclerosis (SSc) is a connective tissue disease characterized by vasculopathy, Raynaud’s phenomenon (RP) and fibrosis that involves skin and internal organs.Pulmonary involvement is the primary cause of death in SSc patients [1]
Considering only the dcSSc patients, TE-Interstitial lung disease (ILD) and ground-glass opacities (GGOs) correlated with the European Scleroderma Study Group (ESSG) index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure

Summary

Introduction

Pulmonary involvement is the primary cause of death in SSc patients [1]. Interstitial lung disease (ILD) occurs in over 90% of patients and 40% of them show restrictive changes in pulmonary function tests [2]. Diagnostics 2020, 10, 225 radiography has significantly improved sensitivity in the evaluation of pulmonary involvement [3], providing additional and more definite information about lung damage. The most common HRCT pattern observed in SSc is nonspecific interstitial pneumonia (NSIP), with a lower degree of coarse reticulations [4] and a greater proportion of ground-glass opacities (GGOs), which can reflect a potentially reversible inflammatory infiltration [5]. It is not that uncommon in SSc patients to observe GGOs together with honeycombing (HC), the main feature of usual interstitial pneumonia (UIP). HC may be prevalent in about 10–20% of SSc patients, depicting a UIP pattern

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