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

doi:10.2169/internalmedicine.56.7668

CASE REPORTS

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

Author information

Eri Sugawara
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Masaru Kato
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Ryo Hisada
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Kenji Oku
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Toshiyuki Bohgaki
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Tetsuya Horita
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Shinsuke Yasuda
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan
Tatsuya Atsumi
Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan

Keywords: pulmonary arterial hypertension, mixed connective tissue disease, glucocorticoid, intravenous cyclophosphamide

JOURNAL OPEN ACCESS

2017 Volume 56 Issue 4 Pages 445-448

DOI https://doi.org/10.2169/internalmedicine.56.7668

Details

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.

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