Characteristics of pulmonary arterial hypertension in patients with systemic sclerosis and anticentriole autoantibodies

Hisataka Maki, Kana Kubota, Masaru Hatano, Shun Minatsuki, Eisuke Amiya, Ayumi Yoshizaki, Yoshihide Asano, Hiroyuki Morita, Shinichi Sato, Issei Komuro

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)

Abstract

Summary Anticentriole autoantibodies-positive systemic sclerosis (SSc) has been reported to develop pulmonary arterial hypertension (PAH) at a high rate. In this report, we describe two patients with anticentriole antibodies-positive SSc-PAH who were treated with pulmonary vasodilators. Both cases were elderly women with poor physical conditions and clinical findings of SSc. Case 1 was resistant to combination therapy with pulmonary vasodilators; in Case 2, hemodynamic improvement was obtained by upfront combination therapy at an early stage. Because anticentriole antibodies-positive SSc-PAH rapidly deteriorates, careful hemodynamic observation and timely aggressive use of pulmonary vasodilators should be considered.

Original languageEnglish
Pages (from-to)413-418
Number of pages6
JournalInternational Heart Journal
Volume61
Issue number2
DOIs
Publication statusPublished - 2020

Keywords

  • Elderly

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