%0 Journal Article %A Keishi Sugino %A Takayuki Kabuki %A Kazutoshi Shibuya %A Sakae Homma %T Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension %D 2018 %R 10.1136/bcr-2017-221755 %J BMJ Case Reports %P bcr-2017-221755 %V 2018 %X 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. %U https://casereports.bmj.com/content/casereports/2018/bcr-2017-221755.full.pdf