Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature
Gastrointestinal perforation in patients with juvenile dermatomyositis has been reported as a life-threatening complication in the literature. However, effective treatment of juvenile dermatomyositis with gastrointestinal perforation remains challenging. We report the case of a patient who developed...
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Frontiers Media S.A.
2025-01-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2024.1522559/full |
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| author | Xue-mei Xu Shuang Ye Ying-ying Jin Chen-xi Liu Sheng-fang Bao Hua Huang Fei Ding Zhen Yang Yan-liang Jin |
| author_facet | Xue-mei Xu Shuang Ye Ying-ying Jin Chen-xi Liu Sheng-fang Bao Hua Huang Fei Ding Zhen Yang Yan-liang Jin |
| author_sort | Xue-mei Xu |
| collection | DOAJ |
| description | Gastrointestinal perforation in patients with juvenile dermatomyositis has been reported as a life-threatening complication in the literature. However, effective treatment of juvenile dermatomyositis with gastrointestinal perforation remains challenging. We report the case of a patient who developed intestinal perforation 5 months after being diagnosed with anti-nuclear matrix protein 2 antibody-positive juvenile dermatomyositis. We systematically reviewed the literature on the medical and/or surgical treatment of gastrointestinal perforation in juvenile dermatomyositis. In addition to our case, as of October 2023, we identified 29 cases of gastrointestinal perforation in patients with juvenile dermatomyositis. Current treatment options for gastrointestinal perforation in juvenile dermatomyositis mainly include corticosteroids, methylprednisolone pulses, rituximab, intravenous immunoglobulin, cyclophosphamide, methotrexate, cyclosporine A, mycophenolate mofetil, and other traditional disease-modifying anti-rheumatic drugs. Notably, juvenile dermatomyositis complicated by gastrointestinal perforation is always associated with disease severity and activity. However, these are extremely severe patients who may not respond to treatment with methylprednisolone pulses and rituximab. Given the limited efficacy of conventional high-intensity systemic immunosuppressive therapy for juvenile dermatomyositis with gastrointestinal perforation, there is an urgent need to explore novel therapeutic approaches. We report on the successful use of vedolizumab in combination with corticosteroids, cyclophosphamide, and intravenous immunoglobulin as a novel therapeutic strategy for treating juvenile dermatomyositis complicated by gastrointestinal perforation. Importantly, up to now, there has been no report of juvenile dermatomyositis with gastrointestinal perforation treated with vedolizumab combined with traditional disease-modifying anti-rheumatic drugs in children. |
| format | Article |
| id | doaj-art-646d056d79cb490db2baa4cc2637a792 |
| institution | Kabale University |
| issn | 2296-2360 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj-art-646d056d79cb490db2baa4cc2637a7922025-01-20T07:19:51ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602025-01-011210.3389/fped.2024.15225591522559Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literatureXue-mei Xu0Shuang Ye1Ying-ying Jin2Chen-xi Liu3Sheng-fang Bao4Hua Huang5Fei Ding6Zhen Yang7Yan-liang Jin8Department of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology, Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Rheumatology and Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaGastrointestinal perforation in patients with juvenile dermatomyositis has been reported as a life-threatening complication in the literature. However, effective treatment of juvenile dermatomyositis with gastrointestinal perforation remains challenging. We report the case of a patient who developed intestinal perforation 5 months after being diagnosed with anti-nuclear matrix protein 2 antibody-positive juvenile dermatomyositis. We systematically reviewed the literature on the medical and/or surgical treatment of gastrointestinal perforation in juvenile dermatomyositis. In addition to our case, as of October 2023, we identified 29 cases of gastrointestinal perforation in patients with juvenile dermatomyositis. Current treatment options for gastrointestinal perforation in juvenile dermatomyositis mainly include corticosteroids, methylprednisolone pulses, rituximab, intravenous immunoglobulin, cyclophosphamide, methotrexate, cyclosporine A, mycophenolate mofetil, and other traditional disease-modifying anti-rheumatic drugs. Notably, juvenile dermatomyositis complicated by gastrointestinal perforation is always associated with disease severity and activity. However, these are extremely severe patients who may not respond to treatment with methylprednisolone pulses and rituximab. Given the limited efficacy of conventional high-intensity systemic immunosuppressive therapy for juvenile dermatomyositis with gastrointestinal perforation, there is an urgent need to explore novel therapeutic approaches. We report on the successful use of vedolizumab in combination with corticosteroids, cyclophosphamide, and intravenous immunoglobulin as a novel therapeutic strategy for treating juvenile dermatomyositis complicated by gastrointestinal perforation. Importantly, up to now, there has been no report of juvenile dermatomyositis with gastrointestinal perforation treated with vedolizumab combined with traditional disease-modifying anti-rheumatic drugs in children.https://www.frontiersin.org/articles/10.3389/fped.2024.1522559/fulljuvenile dermatomyositisgastrointestinal perforationgastrointestinal involvementabdominal paingastrointestinal bleedingvedolizumab |
| spellingShingle | Xue-mei Xu Shuang Ye Ying-ying Jin Chen-xi Liu Sheng-fang Bao Hua Huang Fei Ding Zhen Yang Yan-liang Jin Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature Frontiers in Pediatrics juvenile dermatomyositis gastrointestinal perforation gastrointestinal involvement abdominal pain gastrointestinal bleeding vedolizumab |
| title | Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature |
| title_full | Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature |
| title_fullStr | Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature |
| title_full_unstemmed | Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature |
| title_short | Anti-nuclear matrix protein 2 antibody-associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab: a case report after a long-term follow-up and a review of the literature |
| title_sort | anti nuclear matrix protein 2 antibody associated juvenile dermatomyositis with gastrointestinal perforations was successfully treated with traditional therapeutic drugs combined with vedolizumab a case report after a long term follow up and a review of the literature |
| topic | juvenile dermatomyositis gastrointestinal perforation gastrointestinal involvement abdominal pain gastrointestinal bleeding vedolizumab |
| url | https://www.frontiersin.org/articles/10.3389/fped.2024.1522559/full |
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