• Modern rheumatology · Jan 2018

    Case Reports

    Combined disease with pulmonary arterial hypertension and pulmonary venous hypertension revealed after treatment of heart failure with preserved ejection fraction in a case with primary Sjögren syndrome.

    • Yoshioki Yamasaki, Kengo Suzuki, Ryo Kamijima, Yusa Asari, Kosei Tsuchida, Machiko Mizushima, Takahiro Okazaki, Yoshihiro J Akashi, Shoichi Ozaki, and Hidehiro Yamada.
    • a Division of Rheumatology and Allergology, St. Marianna University School of Medicine , Sugao, Miyamae, Kawasaki City, Kanagawa Prefecture , Japan.
    • Mod Rheumatol. 2018 Jan 1; 28 (1): 193-196.

    AbstractA 49-year-old woman with primary Sjögren syndrome initially developed pulmonary venous hypertension (PVH) due to heart failure with preserved ejection fraction. Endomyocardial biopsy specimens showed mild myocardial fibrosis. Pulmonary arterial hypertension (PAH) was revealed after the treatment with diuretics. During the treatment for PAH using upfront combination with pulmonary vasodilators and immunosuppressants, the patient developed combined disease with PAH and PVH. A careful hemodynamic assessment is necessary in such cases.

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