A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis
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-...
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2017-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2017/1479012 |
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| author | Tsunehisa Nagamori Yoichiro Yoshida Hironori Takahashi Hideharu Oka Aya Kajihama Koichi Nakau Masaya Sugimoto Masako Minami-Hori Hiroshi Azuma |
| author_facet | Tsunehisa Nagamori Yoichiro Yoshida Hironori Takahashi Hideharu Oka Aya Kajihama Koichi Nakau Masaya Sugimoto Masako Minami-Hori Hiroshi Azuma |
| author_sort | Tsunehisa Nagamori |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-5cce4764caef4f329d0db9ab1794bf74 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-5cce4764caef4f329d0db9ab1794bf742025-08-20T03:34:14ZengWileyCase Reports in Pediatrics2090-68032090-68112017-01-01201710.1155/2017/14790121479012A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with DermatomyositisTsunehisa Nagamori0Yoichiro Yoshida1Hironori Takahashi2Hideharu Oka3Aya Kajihama4Koichi Nakau5Masaya Sugimoto6Masako Minami-Hori7Hiroshi Azuma8Department of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Dermatology, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanDepartment of Pediatrics, Asahikawa Medical University, 2-1-1 Midorigaoka Higashi, Asahikawa, Hokkaido, JapanJuvenile-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.http://dx.doi.org/10.1155/2017/1479012 |
| spellingShingle | Tsunehisa Nagamori Yoichiro Yoshida Hironori Takahashi Hideharu Oka Aya Kajihama Koichi Nakau Masaya Sugimoto Masako Minami-Hori Hiroshi Azuma 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 |
| title | A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis |
| title_full | A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis |
| title_fullStr | A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis |
| title_full_unstemmed | A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis |
| title_short | A Marked Response to Immunosuppressive Intervention for Abruptly Occurring Cardiac Complications in a Case of Juvenile Systemic Sclerosis Overlapped with Dermatomyositis |
| title_sort | marked response to immunosuppressive intervention for abruptly occurring cardiac complications in a case of juvenile systemic sclerosis overlapped with dermatomyositis |
| url | http://dx.doi.org/10.1155/2017/1479012 |
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