Hepatic veno-occlusive disease accompanied by thrombotic microangiopathy developed during treatment of juvenile dermatomyositis and macrophage activation syndrome : A case report
© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissionsoup.com..
Hepatic veno-occlusive disease (VOD) is a complication of haematopoietic stem cell transplantation. VOD is associated with the occurrence of thrombotic microangiopathy (TMA). In haematopoietic stem cell transplantation, VOD and TMA are endothelial syndromes resulting from endothelial cell activation and dysfunction. In rheumatic disease, while TMA is not rare, there are few reports of VOD. In idiopathic myositis, only one case with VOD and TMA complications has been reported, and there are no published cases in juvenile dermatomyositis (JDM). We report a case of JDM manifesting VOD and TMA complications during the treatment for myositis and macrophage activation syndrome (MAS). A 5-year-old boy diagnosed as anti-nuclear matrix protein 2 antibody-positive JDM was complicated by MAS. He received pulsed methylprednisolone, prednisolone, and tacrolimus, but JDM and MAS progressed. He was then treated with intravenous cyclophosphamide and cyclosporine A, with improvement in myositis symptoms and MAS. After initiation of cyclophosphamide and cyclosporine A, he developed haemolysis, painful hepatomegaly, liver damage, and ascites. He was diagnosed with VOD and TMA. Cyclophosphamide and cyclosporine A were discontinued, with recovery from VOD and TMA. The patient remained well on treatment with methotrexate, without any relapse of JDM and MAS to date. The presence of vasculopathy and hypercytokinaemia because of JDM and MAS exacerbated endothelial cell damage. In the present case, we suggest that the main cause of VOD was medication with CY and CsA, which had been used to treat acute exacerbation of MAS and JDM.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:7 |
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| Enthalten in: |
Modern rheumatology case reports - 7(2023), 2 vom: 19. Juni, Seite 404-409 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Mouri, Mariko [VerfasserIn] |
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| Links: |
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| Themen: |
83HN0GTJ6D |
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| Anmerkungen: |
Date Completed 21.06.2023 Date Revised 22.06.2023 published: Print Citation Status MEDLINE |
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| doi: |
10.1093/mrcr/rxac086 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM349296529 |
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| 245 | 1 | 0 | |a Hepatic veno-occlusive disease accompanied by thrombotic microangiopathy developed during treatment of juvenile dermatomyositis and macrophage activation syndrome |b A case report |
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| 520 | |a © Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissionsoup.com. | ||
| 520 | |a Hepatic veno-occlusive disease (VOD) is a complication of haematopoietic stem cell transplantation. VOD is associated with the occurrence of thrombotic microangiopathy (TMA). In haematopoietic stem cell transplantation, VOD and TMA are endothelial syndromes resulting from endothelial cell activation and dysfunction. In rheumatic disease, while TMA is not rare, there are few reports of VOD. In idiopathic myositis, only one case with VOD and TMA complications has been reported, and there are no published cases in juvenile dermatomyositis (JDM). We report a case of JDM manifesting VOD and TMA complications during the treatment for myositis and macrophage activation syndrome (MAS). A 5-year-old boy diagnosed as anti-nuclear matrix protein 2 antibody-positive JDM was complicated by MAS. He received pulsed methylprednisolone, prednisolone, and tacrolimus, but JDM and MAS progressed. He was then treated with intravenous cyclophosphamide and cyclosporine A, with improvement in myositis symptoms and MAS. After initiation of cyclophosphamide and cyclosporine A, he developed haemolysis, painful hepatomegaly, liver damage, and ascites. He was diagnosed with VOD and TMA. Cyclophosphamide and cyclosporine A were discontinued, with recovery from VOD and TMA. The patient remained well on treatment with methotrexate, without any relapse of JDM and MAS to date. The presence of vasculopathy and hypercytokinaemia because of JDM and MAS exacerbated endothelial cell damage. In the present case, we suggest that the main cause of VOD was medication with CY and CsA, which had been used to treat acute exacerbation of MAS and JDM | ||
| 650 | 4 | |a Case Reports | |
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| 650 | 4 | |a Cyclophosphamide | |
| 650 | 4 | |a hepatic veno-occlusive disease | |
| 650 | 4 | |a juvenile dermatomyositis | |
| 650 | 4 | |a macrophage activation syndrome | |
| 650 | 4 | |a thrombotic microangiopathy | |
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| 700 | 1 | |a Tanaka, Eriko |e verfasserin |4 aut | |
| 700 | 1 | |a Hiratoko, Kanako |e verfasserin |4 aut | |
| 700 | 1 | |a Okubo, Mariko |e verfasserin |4 aut | |
| 700 | 1 | |a Inoue, Michio |e verfasserin |4 aut | |
| 700 | 1 | |a Morio, Tomohiro |e verfasserin |4 aut | |
| 700 | 1 | |a Shimizu, Masaki |e verfasserin |4 aut | |
| 700 | 1 | |a Nishino, Ichizo |e verfasserin |4 aut | |
| 700 | 1 | |a Okiyama, Naoko |e verfasserin |4 aut | |
| 700 | 1 | |a Mori, Masaaki |e verfasserin |4 aut | |
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