Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab
Thrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Macrophage activation syndrome (MAS) is also a rare, life-threatening hyperinflammatory condition that is comorbid with SLE. However, the association between TMA and MAS in patients wi...
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| Format: | Article |
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Frontiers Media S.A.
2023-01-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2022.1097528/full |
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| author | Makoto Yamaguchi Masashi Mizuno Fumiya Kitamura Shiho Iwagaitsu Hironobu Nobata Hiroshi Kinashi Shogo Banno Akimasa Asai Takuji Ishimoto Takayuki Katsuno Takayuki Katsuno Yasuhiko Ito |
| author_facet | Makoto Yamaguchi Masashi Mizuno Fumiya Kitamura Shiho Iwagaitsu Hironobu Nobata Hiroshi Kinashi Shogo Banno Akimasa Asai Takuji Ishimoto Takayuki Katsuno Takayuki Katsuno Yasuhiko Ito |
| author_sort | Makoto Yamaguchi |
| collection | DOAJ |
| description | Thrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Macrophage activation syndrome (MAS) is also a rare, life-threatening hyperinflammatory condition that is comorbid with SLE. However, the association between TMA and MAS in patients with SLE has rarely been assessed, and the difficulty of diagnosing these conditions remains prevalent. The efficacy of eculizumab has been reported for SLE patients whose conditions are complicated with TMA. However, no study has investigated the therapeutic efficacy of eculizumab for TMA concomitant with SLE-associated MAS. Herein, we report the first case of TMA concomitant with SLE-associated MAS that was initially refractory to conventional immunosuppressive therapy but showed remarkable recovery after eculizumab treatment. Furthermore, we evaluated serum syndecan-1 and hyaluronan levels, which are biomarkers of endothelial damage. We found that these levels decreased after the administration of eculizumab, suggesting that TMA was the main pathology of the patient. This case illustrates that it is important to appropriately assess the possibility of TMA during the course of SLE-associated MAS and consider the use of eculizumab as necessary. |
| first_indexed | 2024-04-10T23:48:23Z |
| format | Article |
| id | doaj.art-b0dd3448520d4223ad9b6ea606798718 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-04-10T23:48:23Z |
| publishDate | 2023-01-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj.art-b0dd3448520d4223ad9b6ea6067987182023-01-10T21:53:47ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2023-01-01910.3389/fmed.2022.10975281097528Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumabMakoto Yamaguchi0Masashi Mizuno1Fumiya Kitamura2Shiho Iwagaitsu3Hironobu Nobata4Hiroshi Kinashi5Shogo Banno6Akimasa Asai7Takuji Ishimoto8Takayuki Katsuno9Takayuki Katsuno10Yasuhiko Ito11Department of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanRenal Replacement Therapy, Nagoya University Graduate School of Medicine, Nagoya, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University Medical Center, Okazaki, JapanDepartment of Nephrology and Rheumatology, Aichi Medical University, Nagakute, JapanThrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Macrophage activation syndrome (MAS) is also a rare, life-threatening hyperinflammatory condition that is comorbid with SLE. However, the association between TMA and MAS in patients with SLE has rarely been assessed, and the difficulty of diagnosing these conditions remains prevalent. The efficacy of eculizumab has been reported for SLE patients whose conditions are complicated with TMA. However, no study has investigated the therapeutic efficacy of eculizumab for TMA concomitant with SLE-associated MAS. Herein, we report the first case of TMA concomitant with SLE-associated MAS that was initially refractory to conventional immunosuppressive therapy but showed remarkable recovery after eculizumab treatment. Furthermore, we evaluated serum syndecan-1 and hyaluronan levels, which are biomarkers of endothelial damage. We found that these levels decreased after the administration of eculizumab, suggesting that TMA was the main pathology of the patient. This case illustrates that it is important to appropriately assess the possibility of TMA during the course of SLE-associated MAS and consider the use of eculizumab as necessary.https://www.frontiersin.org/articles/10.3389/fmed.2022.1097528/fullthrombotic microangiopathymacrophage activation syndromesystemic lupus erythematosusneuropsychiatric systemic lupus erythematosuseculizumab |
| spellingShingle | Makoto Yamaguchi Masashi Mizuno Fumiya Kitamura Shiho Iwagaitsu Hironobu Nobata Hiroshi Kinashi Shogo Banno Akimasa Asai Takuji Ishimoto Takayuki Katsuno Takayuki Katsuno Yasuhiko Ito Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab Frontiers in Medicine thrombotic microangiopathy macrophage activation syndrome systemic lupus erythematosus neuropsychiatric systemic lupus erythematosus eculizumab |
| title | Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| title_full | Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| title_fullStr | Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| title_full_unstemmed | Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| title_short | Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| title_sort | case report thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab |
| topic | thrombotic microangiopathy macrophage activation syndrome systemic lupus erythematosus neuropsychiatric systemic lupus erythematosus eculizumab |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2022.1097528/full |
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