Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome
Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-04-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.894464/full |
| _version_ | 1818482430018846720 |
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| author | Yuehua Huang Yanying Wang Fan Yu Xuehan Mao Bianhong Wang Jingxian Li Lihong Li |
| author_facet | Yuehua Huang Yanying Wang Fan Yu Xuehan Mao Bianhong Wang Jingxian Li Lihong Li |
| author_sort | Yuehua Huang |
| collection | DOAJ |
| description | Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and MYD88 L265P mutation, but no lymphoplasmacytic lymphoma on bone marrow examination. She fulfilled the diagnosis of Schnitzler syndrome and was treated with the Bruton tyrosine kinase inhibitor ibrutinib in combination with prednisone. Her symptoms improved dramatically, and the level of IgMκ monoclonal protein also declined. She tolerated the treatment well. This case highlights the potential therapeutic role of Bruton tyrosine kinase inhibitors in Schnitzler syndrome. |
| first_indexed | 2024-12-10T11:47:46Z |
| format | Article |
| id | doaj.art-886f06da3c07408386d231b9d19cd36b |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-10T11:47:46Z |
| publishDate | 2022-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-886f06da3c07408386d231b9d19cd36b2022-12-22T01:50:01ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-04-011310.3389/fimmu.2022.894464894464Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler SyndromeYuehua HuangYanying WangFan YuXuehan MaoBianhong WangJingxian LiLihong LiSchnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and MYD88 L265P mutation, but no lymphoplasmacytic lymphoma on bone marrow examination. She fulfilled the diagnosis of Schnitzler syndrome and was treated with the Bruton tyrosine kinase inhibitor ibrutinib in combination with prednisone. Her symptoms improved dramatically, and the level of IgMκ monoclonal protein also declined. She tolerated the treatment well. This case highlights the potential therapeutic role of Bruton tyrosine kinase inhibitors in Schnitzler syndrome.https://www.frontiersin.org/articles/10.3389/fimmu.2022.894464/fullSchnitzler syndromeinterleukin-1Bruton tyrosine kinase inhibitoribrutinibcase report |
| spellingShingle | Yuehua Huang Yanying Wang Fan Yu Xuehan Mao Bianhong Wang Jingxian Li Lihong Li Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome Frontiers in Immunology Schnitzler syndrome interleukin-1 Bruton tyrosine kinase inhibitor ibrutinib case report |
| title | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
| title_full | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
| title_fullStr | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
| title_full_unstemmed | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
| title_short | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
| title_sort | case report therapeutic use of ibrutinib in a patient with schnitzler syndrome |
| topic | Schnitzler syndrome interleukin-1 Bruton tyrosine kinase inhibitor ibrutinib case report |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.894464/full |
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