Abstract

Currently, lung impairment is the leading factor responsible for the morbidity and mortality associated with systemic sclerosis. Therefore, the recognition of the various tomography patterns becomes decisive in the clinical management of these patients. In high-resolution computed tomography studies, the most common pattern is that of nonspecific interstitial pneumonia. However, there are other forms of lung involvement that must also be recognized. The aim of this study was to review the literature on the main changes resulting from pulmonary involvement in systemic sclerosis and the corresponding radiological findings, considering the current classification of interstitial diseases. We searched the Medline (PubMed), Lilacs, and SciELO databases in order to select articles related to pulmonary changes in systemic sclerosis and published in English between 2000 and 2015. The pulmonary changes seen on computed tomography in systemic sclerosis are varied and are divided into three main categories: interstitial, alveolar, and vascular. Interstitial changes constitute the most common type of pulmonary involvement in systemic sclerosis. However, alveolar and vascular manifestations must also be recognized and considered in the presence of atypical clinical presentations and inadequate treatment responses.

Highlights

  • Systemic sclerosis (SSc) is an autoimmune connective tissue disease, of unknown cause, characterized by inflammatory changes, fibrosis, obstructive small-vessel vasculopathy, 1

  • Of all cases of pulmonary fibrosis in SSc, 50.0–77.5% are due to nonspecific interstitial pneumonia, which could explain that difference in prognosis[10,11]

  • Nonspecific interstitial pneumonia is a particular form of idiopathic interstitial pneumonia characterized by varying degrees of inflammation and fibrosis, from forms predominated by inflammatory processes to those predominated by fibrosis, which are the most common, without the fibroblastic foci and honeycombing seen in usual interstitial pneumonia[8,12]

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Summary

Introduction

Systemic sclerosis (SSc) is an autoimmune connective tissue disease, of unknown cause, characterized by inflammatory changes, fibrosis, obstructive small-vessel vasculopathy, 1.

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