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Updated: Feb 10, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension
Keishi Sugino1, Takayuki Kabuki2, Kazutoshi Shibuya3
1Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan.
Abstract:
A 65-year-old woman with a 35-year history of limited cutaneous systemic scleroderma was admitted to our hospital complaining of a 3-month history of progressive dyspnoea on exertion. High-resolution CT images of the chest revealed diffuse reticular opacities and traction bronchiectasis predominantly in the bilateral lower lobes of the lung. Specimens obtained during video-assisted thoracic surgery were consistent with fibrocellular non-specific interstitial pneumonia and accompanied by accumulation of lymph follicles within areas of fibrosis. Although the patient received combination therapy with prednisolone and intravenous cyclophosphamide at a dosage of 500 mg/m2 monthly for 5 months, her clinical condition deteriorated gradually. In addition, right heart catheterisation revealed borderline pulmonary arterial hypertension with mean pulmonary artery pressure of 24 mm Hg. Therefore, we initiated a combination therapy of an antifibrotic agent, pirfenidone for 12 months, and the dual endothelin receptor antagonist, macitentan, with prednisolone. As a result, her clinical condition improved dramatically.
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