Treatment of vasodilator-resistant mixed connective tissue disease-associated pulmonary arterial hypertension with glucocorticoid and cyclophosphamide

Eri Sugawara, Masaru Kato*, Ryo Hisada, Kenji Oku, Toshiyuki Bohgaki, Tetsuya Horita, Shinsuke Yasuda, Tatsuya Atsumi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

7 Scopus citations

Abstract

Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide. This case suggested the potential benefit of immunosuppressants in patients with severe MCTD-associated PAH.

Original languageEnglish
Pages (from-to)445-448
Number of pages4
JournalInternal Medicine
Volume56
Issue number4
DOIs
StatePublished - 2017

Keywords

  • Glucocorticoid
  • Intravenous cyclophosphamide
  • Mixed connective tissue disease
  • Pulmonary arterial hypertension

ASJC Scopus subject areas

  • Internal Medicine

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