Significance of various pulmonary and extrapulmonary abnormalities on HRCT of the chest in scleroderma lung

Abstract

Patients with systemic sclerosis (SSc) are routinely investigated with high-resolution computed tomography (HRCT) chest for early detection and accurate characterization of complicating interstitial lung diseases. Though the primary aim of HRCT is to delineate the burden of pulmonary involvement and to characterize the nature of fibrosis to potentially help guide management, it provides an opportunity to evaluate extrapulmonary manifestations, particularly the dilated pulmonary artery, esophageal dilatation, and pericardial abnormalities which have their own clinical significance. The aim of this article is to discuss the significance of various pulmonary and extrapulmonary abnormalities that may be identified on HRCT chest of SSc patients.

Correlation of HRCT Scoring System with PFT Parameters in Interstitial Lung Disease Associated with Systemic Sclerosis Patients

“…Honeycombing represents irreversible end-stage lung fibrosis) leading to lung parenchymal architectural distortion and moderate to severely decreased PFT with higher HRCT score. 2 Of three patients who could not perform PFT, two had a UIP pattern with honey-combing suggesting severe disease associated with honey-combing and coarse reticular fibrosis. This was in agreement with the observations of Ooi et al 7 Traction bronchiectasis was seen in 14 (38.8%) cases in our study, in contrast with Ibrahim et al 23 .…”

“…This paradox may be explained by the early pulmonary involvement and inflammatory alveolar infiltrates which affects lung function less severely than fibrosis. 2,7 Majority of the patients with GGO and fine reticulations had moderately deranged PFT with consistently higher total HRCT score. The GGO in these cases may be due to fine alveolar fibrosis as HRCT does not have sufficient spatial (anatomical) resolution to separately identify these two features in patients with extensive GGO.…”

“…This was categorised under organising pneumonia.GGO was noted in 28 (77.7%) patients in our study; however only GGO as an abnormality was found in 11% of the cases, in contrast with other workers. 2,20 Shah et al 21 had observed GGO in 66% of cases and GGO alone in 7% of cases. Few patients with only GGO or associated with fine reticulation (17%) had better lung function results (predicted FVC >70%) but higher total HRCT score.…”

“…More than 90% of the patients with SSc have evidence of interstitial lung disease (ILD) at autopsy and 40% of patients show abnormal pulmonary function tests (PFTs). 2 Pulmonary complications, such as ILD and pulmonary hypertension are the most common cardio-pulmonary findings in patients with SSc. These account for approximately 60% of SSc related deaths.…”

Multimodality imaging in connective tissue disease-related interstitial lung disease

“…S1). 28 Conversely to other CTDs, patients with rheumatoid arthritis (RA)-related ILD most frequently present with UIP pattern (Fig 3). 29 Once the UIP pattern is established, the prognosis is said to be similar to that of idiopathic UIP.…”