PT - JOURNAL ARTICLE AU - Keishi Sugino AU - Takayuki Kabuki AU - Kazutoshi Shibuya AU - Sakae Homma TI - Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension AID - 10.1136/bcr-2017-221755 DP - 2018 May 26 TA - BMJ Case Reports PG - bcr-2017-221755 VI - 2018 4099 - http://casereports.bmj.com/content/2018/bcr-2017-221755.short 4100 - http://casereports.bmj.com/content/2018/bcr-2017-221755.full AB - 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.