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Novel treatment (new drug/intervention; established drug/procedure in new situation)
CASE REPORT
Systemic scleroderma-related interstitial pneumonia associated with borderline pulmonary arterial hypertension
  1. Keishi Sugino1,
  2. Takayuki Kabuki2,
  3. Kazutoshi Shibuya3,
  4. Sakae Homma1
  1. 1Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
  2. 2Department of Cardiovascular Medicine, Toho University Omori Medical Center, Tokyo, Japan
  3. 3Department of Diagnostic Pathology, Toho University Omori Medical Center, Tokyo, Japan
  1. Correspondence to Dr Keishi Sugino, ks142129_ikusou{at}ybb.ne.jp

Summary

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.

  • interstitial lung disease
  • pulmonary hypertension
  • connective tissue disease
  • drugs and medicines

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bcr-2017-221755

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    Footnotes

    • Contributors KSu and SH: study design, data analysis, manuscript preparation and guarantor of the paper. KSu, TK and KSh: data collection and data analysis. KSu, KSh and SH: manuscript preparation and review. All authors had full access to all of the data in the study and can take responsibility for the integrity of the data and the accuracy of the data analysis.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.