TY - JOUR T1 - Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension JF - BMJ Case Reports DO - 10.1136/bcr-2017-221755 VL - 2018 SP - bcr-2017-221755 AU - Keishi Sugino AU - Takayuki Kabuki AU - Kazutoshi Shibuya AU - Sakae Homma Y1 - 2018/05/26 UR - http://casereports.bmj.com/content/2018/bcr-2017-221755.abstract N2 - 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. ER -