Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody
Joint Authors
Ishii, Tomonori
Ishii, Yusho
Shirai, Tsuyoshi
Hoshi, Yousuke
Fujita, Yoko
Shirota, Yuko
Sugimura, Koichiro
Shimokawa, Hiroaki
Harigae, Hideo
Fujii, Hiroshi
Source
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-02-26
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Abstract EN
Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications.
Among them, anticentriole antibody is recently reported to be highly associated with SSc-associated pulmonary arterial hypertension (SSc-PAH).
In general, several vasodilators are used as therapeutic drugs for SSc-PAH, whereas immunosuppressive therapies are not.
Here, we report the case of a 62-year-old female with anticentriole antibody-positive SSc-PAH treated with immunosuppressants and vasodilators.
She presented with two-year exertional dyspnea and was diagnosed with PAH and SSc owing to the centriole staining pattern and other symptoms without digital sclerosis.
Oral vasodilators were initially administered but were not sufficiently effective on dyspnea.
Immunosuppressants such as prednisolone and cyclophosphamide were started.
Both of them improved mean pulmonary arterial pressure and 6-minute walk distance, and the anticentriole antibody also disappeared.
In this case, SSc-PAH with anticentriole antibody was properly diagnosed and immunosuppressants and vasodilators improved the hemodynamics of PAH with anticentriole antibody and stably maintained it and, in addition, reduced the titer of anticentriole antibody.
This indicates that anticentriole antibody might represent a good responsive group to therapies among subgroups of patients with SSc-PAH.
American Psychological Association (APA)
Ishii, Yusho& Fujii, Hiroshi& Sugimura, Koichiro& Shirai, Tsuyoshi& Hoshi, Yousuke& Fujita, Yoko…[et al.]. 2020. Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody. Case Reports in Rheumatology،Vol. 2020, no. 2020, pp.1-7.
https://search.emarefa.net/detail/BIM-1151128
Modern Language Association (MLA)
Ishii, Yusho…[et al.]. Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody. Case Reports in Rheumatology No. 2020 (2020), pp.1-7.
https://search.emarefa.net/detail/BIM-1151128
American Medical Association (AMA)
Ishii, Yusho& Fujii, Hiroshi& Sugimura, Koichiro& Shirai, Tsuyoshi& Hoshi, Yousuke& Fujita, Yoko…[et al.]. Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody. Case Reports in Rheumatology. 2020. Vol. 2020, no. 2020, pp.1-7.
https://search.emarefa.net/detail/BIM-1151128
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1151128