Pulmonary artery hypertension prior to the relapse of adult-onset Still's disease

Yuki Hara; Takayoshi Morita; Katsunao Tanaka; Fusako Sera; Yasushi Sakata; Masayuki Nishide; Yuichi Maeda; Masashi Narazaki; Atsushi Kumanogoh

doi:10.1002/rcr2.746

Pulmonary artery hypertension prior to the relapse of adult-onset Still's disease

Respirol Case Rep. 2021 Apr 28;9(5):e00746. doi: 10.1002/rcr2.746. eCollection 2021 May.

Authors

Yuki Hara¹, Takayoshi Morita¹, Katsunao Tanaka², Fusako Sera², Yasushi Sakata², Masayuki Nishide¹, Yuichi Maeda¹, Masashi Narazaki^{1

3}, Atsushi Kumanogoh¹

Affiliations

¹ Department of Respiratory Medicine and Clinical Immunology, Graduate School of Medicine Osaka University Osaka Japan.
² Department of Cardiovascular Medicine, Graduate School of Medicine Osaka University Osaka Japan.
³ Department of Advanced Clinical and Translational Immunology, Graduate School of Medicine Osaka University Osaka Japan.

Abstract

Adult-onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33-year-old woman was admitted to our hospital with dyspnoea on exertion. Although she had not had symptoms of AOSD for 18 months before her admission, she presented with gradually progressing PAH. Because she had no typical symptoms of AOSD, she was treated with pulmonary vasodilators. However, her PAH did not improve. At one month after vasodilator treatment, she developed a high fever with elevation of ferritin. We determined that her AOSD had relapsed. Immunosuppressants were started and both her AOSD and PAH quickly improved. PAH may develop in the absence of typical symptoms of AOSD and immunosuppressants may be effective in such a case.

Keywords: AOSD; PAH; autoimmune disease.

Publication types

Case Reports