The DOI matches its Crossref record; 65 of the 65 references it cites were found in a registry or an index.
Sjögren Syndrome‐associated lymphomas: an update on pathogenesis and management
Open accessAbstract
Summary: Primary Sjögren Syndrome ( pSS ) is an autoimmune disease associated with an increased risk of lymphoma. Lymphomas complicating pSS are mostly low‐grade B cell non‐Hodgkin lymphomas, predominantly of marginal zone histological type. Mucosal localization is predominant, notably mucosa‐associated lymphoid tissue lymphomas. Lymphomas often develop in organs where pSS is active, such as salivary glands. Germinal centre (GC)‐like structures, high TNFSF13B (BAFF) and Flt3‐ligand (FLT3LG) levels and genetic impairment of TNFAIP3 are new predictors of lymphoma development. These new findings allow a better understanding of the pathogenic mechanisms leading to lymphoma. We propose the following scenario: auto‐immune B cells with rheumatoid factor (RF) activity are continuously stimulated by immune complexes containing antibodies against more specific auto‐antigens, such as SSA/Ro, SSB/La or others. Germline abnormality of TNFAIP3 leads to a decreased control of the NF‐ kB pathway and thus promotes survival of B cells and oncogenic mutations especially in GC structure. Moreover, B cells are stimulated by a positive loop of activation induced by BAFF secretion. Thus, lymphomagenesis associated with pSS exemplifies the development of antigen‐driven B‐cell lymphoma. The control of disease activity by a well‐targeted immunosuppressor is the primary objective of the management of the patient in order to repress chronic B cell stimulation.
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1 retracted
RETRACTED ARTICLE: DCs induce CD40-independent immunoglobulin class switching through BLyS and APRIL
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RetractedWHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
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WHO classification of haematolymphoid tumours
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Chromatin–IgG complexes activate B cells by dual engagement of IgM and Toll-like receptors
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Mice Transgenic for Baff Develop Lymphocytic Disorders along with Autoimmune Manifestations
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Increased Risk of Lymphoma in Sicca Syndrome
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Cited by
EULAR recommendations for the management of Sjogren's syndrome with topical and systemic therapies
Ramos-Casals, M., Brito-Zerón, P., Bombardieri, S., Bootsma, H., De Vita, S., Dörner, T., Fisher, B. A., Gottenberg, J.-E., Hernandez-Molina, G., Kocher, A., et al. (2020) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2019-216114 · Cited by 732
Self-citationSjögren’s syndrome: a systemic autoimmune disease
Negrini, S., Emmi, G., Greco, M., Borro, M., Sardanelli, F., Murdaca, G., Indiveri, F., & Puppo, F. (2022) · Clinical and Experimental Medicine · doi:10.1007/s10238-021-00728-6 · Cited by 459
Metadata
- Authors
- Nocturne, G., & Mariette, X.
- Venue
- British Journal of Haematology
- Publisher
- Wiley
- Published
- 2014-10-15
- Volume
- 168
- Issue
- 3
- Pages
- 317-327
- ISSN
- 0007-1048 (ISSN-L)
- 1365-2141
- OpenAlex
- W2023989799
- Cited by
- 295
- References
- 65
- FWCI
- 11.99
- Type
- journal article
- Language
- en
- Open access
- bronze
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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