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A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis
Joint Authors
Oka, Hideharu
Nakau, Kouichi
Kajihama, Aya
Sugimoto, Masaya
Azuma, Hiroshi
Nagamori, Tsunehisa
Yoshida, Yoichiro
Takahashi, Hironori
Minami-Hori, Masako
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-02-21
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Abstract EN
Juvenile-onset systemic sclerosis (jSSc) is a rare condition, having unique characteristic features compared to adult-onset SSc.
Although cardiac involvement (CI) is known as a leading cause of mortality overall in SSc, the importance of CI in jSSc has not been emphasized.
Here we present a 13-year-old female with jSSc overlapped with dermatomyositis (DM) complicated CI.
She developed skin thickness and induration, Raynaud’s phenomenon, digital pitting scars in fingertips, and skeletal myositis.
Oral prednisolone and pulse methotrexate treatment led to the improvement of skin findings; however two weeks after the initiation she suddenly presented with muscle pain and dyspnea within a few days.
Cardiac investigations then showed pericardiac effusion and diastolic dysfunction due to significant biventricular hypertrophy causing heart failure.
As pericardiac effusion and exacerbation of skeletal myositis were evident, steroid pulse therapy was initiated.
Unexpectedly, not only the myositis but also the CI including diastolic dysfunction was improved.
She thereafter followed a favorable clinical course without reactivation of the CI or cardiac fibrosis.
As a conclusion, close attention to CI must be paid in jSSc patients, especially when skeletal muscle involvement is evident and immunosuppressive therapy may be effective for CI in jSSc in cases where it occurs abruptly.
American Psychological Association (APA)
Nagamori, Tsunehisa& Yoshida, Yoichiro& Takahashi, Hironori& Oka, Hideharu& Kajihama, Aya& Nakau, Kouichi…[et al.]. 2017. A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis. Case Reports in Pediatrics،Vol. 2017, no. 2017, pp.1-7.
https://search.emarefa.net/detail/BIM-1149201
Modern Language Association (MLA)
Nagamori, Tsunehisa…[et al.]. A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis. Case Reports in Pediatrics No. 2017 (2017), pp.1-7.
https://search.emarefa.net/detail/BIM-1149201
American Medical Association (AMA)
Nagamori, Tsunehisa& Yoshida, Yoichiro& Takahashi, Hironori& Oka, Hideharu& Kajihama, Aya& Nakau, Kouichi…[et al.]. A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis. Case Reports in Pediatrics. 2017. Vol. 2017, no. 2017, pp.1-7.
https://search.emarefa.net/detail/BIM-1149201
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1149201