Imaging lung disease in systemic sclerosis

Abstract

Interstitial lung disease and pulmonary hypertension (PH) are the most common cardiopulmonary findings in patients with systemic sclerosis (SSc). About two thirds of patients suffering from SSc develop scleroderma interstitial lung disease. PH is present in about 20% of SSc patients and is typically associated with severe lung disease, although it may be an isolated manifestation of SSc. High-resolution CT scanning is a key method for evaluating chest involvement. There are four roles of imaging in scleroderma interstitial lung disease: 1) detection of lung involvement, 2) identification of patients likely to respond to treatment, 3) assessment of treatment efficacy, and 4) exclusion of other significant diseases to include PH and cardiac and esophageal abnormalities.

Fig. 1

Coronal and sagittal chest CT reconstructions (lung window) demonstrate the classic appearance of early lung involvement in scleroderma interstitial lung disease. Initially subtle alterations of increased ground glass opacity and accentuated reticular markings (a and b) that affect the peripheral, posterior, and basilar portions of the lungs

Fig. 2

Coronal and sagittal chest CT reconstructions (lung window) demonstrate the classic appearance of scleroderma interstitial lung disease in patients with progressive disease, showing extensive architectural distortion due to progressive pulmonary fibrosis (a and b)

Fig. 3

Esophageal dilatation with retained debris is an early and frequent manifestation of scleroderma and may help differentiate interstitial lung disease due to connective tissue disease from that of other etiologies