| Title: | Evidence of B cell clonality and investigation into properties of the IgM in patients with Schnitzler syndrome |
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| Authors: | ID Pathak, Shelly (Author)
ID Rowczenio, Dorota (Author)
ID Lara-Reyna, Samuel (Author)
ID Kačar, Mark, Klinika Golnik (Author)
ID Owen, Roger (Author)
ID Doody, Gina (Author)
ID Krause, Karoline (Author)
ID Lachmann, Helen J (Author)
ID Doffinger, Rainer (Author)
ID Newton, Darren (Author)
ID Savic, Sinisa (Author) |
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| Files: |  PDF - Presentation file, download (1003,75 KB)
MD5: 4C056291BB507B45C3EEF1A2227A4075
 URL - Source URL, visit https://www.frontiersin.org/articles/10.3389/fimmu.2020.569006/pdf
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| Language: | English |
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| Typology: | 1.01 - Original Scientific Article |
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| Organization: |  UKPBAG - University Clinic of Respiratory and Allergic Diseases Golnik
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| Abstract: | The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. The two main defining features of this late-onset condition are neutrophilic urticarial dermatoses (NUD) and the presence of an IgM monoclonal component. While the former aspect has been extensively studied in this disease setting, the enigmatic paraproteinaemia and its potential consequential effects within SchS, has not previously been thoroughly addressed. Previous studies analyzing clonal B cell repertoires have largely focused on autoimmune disorders such as Systemic Lupus Erythematous (SLE) and hematological malignancies such as Chronic Lymphocytic Leukaemia (CLL), where B-cell clonality is central to disease pathology. The present study uses next-generation sequencing to provide detailed insight into aspects of B cell VDJ recombination and properties of the resulting immunoglobulin chains. An overview of IgH regional dynamics in 10 SchS patients, with a particular focus on CDR3 sequences and VDJ gene usage is reported, highlighting the presence of specific B cell expansions. Protein microarray detected a substantial proportion of autoreactive IgM to nuclear target proteins, though a single universal target was not identified. Together, these genetic and functional findings impart new understanding into this rare disorder. |
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| Keywords: | Schnitzler syndrome, B-lymhocytes, paraproteinemias, pararoteins, immunoglobulin M, autoinflammatory diseases, IgM |
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| Publication status: | Published |
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| Publication version: | Version of Record |
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| Place of publishing: | Švica |
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| Publisher: | Frontiers Media SA |
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| Year of publishing: | 2020 |
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| Number of pages: | str. 1-10 |
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| Numbering: | [Vol.] 11 |
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| PID: | 20.500.12556/DiRROS-13871  |
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| UDC: | 616-097 |
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| ISSN on article: | 1664-3224 |
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| DOI: | 10.3389/fimmu.2020.569006 |
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| COBISS.SI-ID: | 58382339 |
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| Copyright: | © 2020 Pathak, Rowczenio, Lara-Reyna, Kacar, Owen, Doody, Krause, Lachmann, Doffinger, Newton and Savic. |
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| Note: | Nasl. z nasl. zaslona;
Soavtor iz Slovenije: Mark Kačar;
Opis vira z dne 6. 4. 2021;
Št. članka: 569006;
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| Publication date in DiRROS: | 08.04.2021 |
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| Views: | 1567 |
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| Downloads: | 1054 |
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