Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome
Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disorder, characterized by chronic urticarial rash and immunoglobulin M (IgM) monoclonal gammopathy. Anti-interleukin-1 (IL-1) therapies have been shown to be more effective in managing the clinical symptoms of SchS compared to...
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Frontiers Media S.A.
2025-01-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1520470/full |
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| author | Hua Bai Dongming Zhou Jinwen Liu Jie He Zhou Min Wenyong Fan Bing Chen Yong Xu |
| author_facet | Hua Bai Dongming Zhou Jinwen Liu Jie He Zhou Min Wenyong Fan Bing Chen Yong Xu |
| author_sort | Hua Bai |
| collection | DOAJ |
| description | Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disorder, characterized by chronic urticarial rash and immunoglobulin M (IgM) monoclonal gammopathy. Anti-interleukin-1 (IL-1) therapies have been shown to be more effective in managing the clinical symptoms of SchS compared to anti-IL-6 therapies. In this case report, we present a male patient with urticarial rash, fever, and arthralgia. Laboratory tests identified the presence of IgMκ monoclonal protein, and the absence of IL-1β in serum. Whole exome sequencing (WES) did not reveal any pathological variants associated with monogenic autoinflammatory diseases or the MYD88 L265P mutation. He met the diagnostic criteria for SchS and was treated with bortezomib, leading to a significant improvement in clinical symptoms and a decline in IgMκ monoclonal protein levels. The patient tolerated the treatment well. This case suggests that bortezomib may be considered as a potential treatment option for SchS, in addition to anti-IL-1 therapies and bruton tyrosine kinase (BTK) inhibitors. |
| format | Article |
| id | doaj-art-445f9a2a25d2430dbe6f1ecad659a41d |
| institution | Kabale University |
| issn | 1664-3224 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj-art-445f9a2a25d2430dbe6f1ecad659a41d2025-01-28T06:40:59ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-01-011610.3389/fimmu.2025.15204701520470Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndromeHua Bai0Dongming Zhou1Jinwen Liu2Jie He3Zhou Min4Wenyong Fan5Bing Chen6Yong Xu7Department of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Orthopedics, Tongji Hospital affiliated to Tongji University School of Medicine, Tongji University, Shanghai, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaDepartment of Hematology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, ChinaSchnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disorder, characterized by chronic urticarial rash and immunoglobulin M (IgM) monoclonal gammopathy. Anti-interleukin-1 (IL-1) therapies have been shown to be more effective in managing the clinical symptoms of SchS compared to anti-IL-6 therapies. In this case report, we present a male patient with urticarial rash, fever, and arthralgia. Laboratory tests identified the presence of IgMκ monoclonal protein, and the absence of IL-1β in serum. Whole exome sequencing (WES) did not reveal any pathological variants associated with monogenic autoinflammatory diseases or the MYD88 L265P mutation. He met the diagnostic criteria for SchS and was treated with bortezomib, leading to a significant improvement in clinical symptoms and a decline in IgMκ monoclonal protein levels. The patient tolerated the treatment well. This case suggests that bortezomib may be considered as a potential treatment option for SchS, in addition to anti-IL-1 therapies and bruton tyrosine kinase (BTK) inhibitors.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1520470/fullSchnitzler syndromeIgM monoclonal gammopathyplasma cellurticarial rashinflammatory responsebortezomib |
| spellingShingle | Hua Bai Dongming Zhou Jinwen Liu Jie He Zhou Min Wenyong Fan Bing Chen Yong Xu Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome Frontiers in Immunology Schnitzler syndrome IgM monoclonal gammopathy plasma cell urticarial rash inflammatory response bortezomib |
| title | Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome |
| title_full | Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome |
| title_fullStr | Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome |
| title_full_unstemmed | Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome |
| title_short | Case report: Therapeutic use of bortezomib in a patient with Schnitzler syndrome |
| title_sort | case report therapeutic use of bortezomib in a patient with schnitzler syndrome |
| topic | Schnitzler syndrome IgM monoclonal gammopathy plasma cell urticarial rash inflammatory response bortezomib |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2025.1520470/full |
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