The Extrapulmonary Manifestations of Systemic Sclerosis on Chest High Resolution Computed Tomography

Abstract

Background: Systemic sclerosis (SS) is a collagen vascular disease of unknown etiology that is characterized by connective tissue abnormalities. This study aimed to evaluate the extra-pulmonary manifestations of SS on chest high resolution computed tomography (HRCT).

Materials and methods: The medical records of patients with SS who presented to our hospital in a 10-year period were retrospectively reviewed. Forty patients with SS were included in this study. The extra pulmonary manifestations of SS were evaluated in these patients, including esophageal involvement, pulmonary arterial dilatation, pleural abnormalities, pericardial disease and mediastinal lymph node involvement.

Results: The most common extra-pulmonary manifestation was esophageal dilatation, which was detected in 70% of the cases followed by pleural involvement. Pulmonary arterial dilatation was seen in 20%, pleural involvement in 40%, pericardial involvement in 40% and mediastinal lymphadenopathy in 30%. The most common pleural abnormality was diffuse pleural thickening and the most common pericardial abnormality was pericardial effusion. There was an association between the severity of lung fibrosis with the incidence of esophageal dilatation and pulmonary arterial hypertension (PAH) in our series. Patients with SS and interstitial lung disease (ILD) who had PAH, had more severe lung fibrosis than those without PAH.

Conclusion: Patients with SS may have a variety of extra-pulmonary manifestations, which can be detected using HRCT. Our study evidenced that HRCT was useful for detecting extra-pulmonary findings of SS such as esophageal dysmotility and dilatation, enlargement of main pulmonary artery and PAH, pleuropericardial involvement and mediastinal lymphadenopathy.

Keywords: Chest; High resolution computed tomography; Lung fibrosis; Systemic sclerosis.

Figure 1.

Chest HRCT of the lung (A) and mediastinal (B) window setting in a 42-year old woman with SS showing dilatation of the esophagus

Figure 2.

HRCT shows aspiration pneumonitis in the right lung (white arrow) associated with esophageal dysmotility (black arrow).

Figure 3.

HRCT scan demonstrating pericardial effusion (white lines).

Figure 4.

Axial HRCT scan in mediastinal window setting in a 55 year old woman with SS showing mediastinal lymphadenopathy in anterior (arrow in A) and middle (asterisks in B) mediastinum.