title: Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide.
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.
authors.affiliation: Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Sugawara
authors.forename: Masaru
authors.initials: M
authors.lastname: Kato
authors.forename: Ryo
authors.initials: R
authors.lastname: Hisada
authors.forename: Kenji
authors.initials: K
authors.lastname: Oku
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Bohgaki
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Horita
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Yasuda
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Atsumi
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008947:Mixed Connective Tissue Disease / Q000150:complications*; D013902:Radiography, Thoracic; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D003520:Cyclophosphamide