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Sjögren's disease
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===Organ-related impacts=== ====Non-Hodgkin lymphoma==== Results from a number of studies indicate that, compared to other autoimmune diseases, Sjögren's disease is associated with a notably high incidence of [[non-Hodgkin lymphoma]], a cancer of white blood cells.<ref name=Voulgarelis2010/> About 5% of patients with SS develop some form of [[lymphoid malignancy]].<ref name="pmid18068857">{{cite journal|title=Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias |journal=Best Pract Res Clin Rheumatol |volume=21 |issue=6 |pages=989–1010 |year=2007 |pmid=18068857 |doi=10.1016/j.berh.2007.09.001|last1=Tzioufas|first1=Athanasios G.|last2=Voulgarelis|first2=Michael}}</ref> Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases.<ref name="pmid17119030"/> The most common lymphomas are salivary extranodal marginal zone B cell lymphomas ([[MALT lymphoma]]s in the salivary glands)<ref name="pmid17992593">{{cite journal |title=Clinical, immunologic, and molecular factors predicting lymphoma development in Sjogren's syndrome patients |journal=Clin Rev Allergy Immunol |volume=32 |issue=3 |pages=265–74 |year=2007 |pmid=17992593 |doi=10.1007/s12016-007-8001-x |last1=Voulgarelis |first1=Michael |last2=Skopouli |first2=Fotini N.|s2cid=19070113 }}</ref> and [[diffuse large B-cell lymphoma]].<ref name="pmid17119030">{{cite journal|title=Malignant lymphomas in autoimmunity and inflammation: a review of risks, risk factors, and lymphoma characteristics |journal=Cancer Epidemiol. Biomarkers Prev. |volume=15 |issue=11 |pages=2069–77 |year=2006 |pmid=17119030 |doi=10.1158/1055-9965.EPI-06-0300|last1=Smedby|first1=K. E.|last2=Baecklund|first2=E.|last3=Askling|first3=J.|doi-access=free}}</ref> Lymphomagenesis in primary Sjögren's disease patients is considered a multistep process, with the first step being chronic stimulation of autoimmune B cells, especially B cells that produce [[rheumatoid factor]] <!-- (RF) --> at sites targeted by the disease.<ref name="pmid10765938">{{cite journal | vauthors = Martin T, Weber JC, Levallois H, Labouret N, Soley A, Koenig S, Korganow AS, Pasquali JL | title = Salivary gland lymphomas in patients with Sjögren's syndrome may frequently develop from rheumatoid factor B cells | journal = Arthritis Rheum. | volume = 43 | issue = 4 | pages = 908–16 | date = April 2000 | pmid = 10765938 | doi = 10.1002/1529-0131(200004)43:4<908::AID-ANR24>3.0.CO;2-K | doi-access = free }}</ref><ref name="pmid15837810">{{cite journal | vauthors = Bende RJ, Aarts WM, Riedl RG, de Jong D, Pals ST, van Noesel CJ | title = Among B cell non-Hodgkin's lymphomas, MALT lymphomas express a unique antibody repertoire with frequent rheumatoid factor reactivity | journal = J. Exp. Med. | volume = 201 | issue = 8 | pages = 1229–41 | date = April 2005 | pmid = 15837810 | pmc = 2213160 | doi = 10.1084/jem.20050068 }}</ref> This increases the frequency of [[oncogenic]] [[mutation]], leading to any dysfunction at checkpoints of autoimmune B-cell activation to transform into malignancy. A study's finding has concluded the continuous stimulation of autoimmune B cells, leads to subtle germinal abnormalities in genes having specific consequences in B cells, which underlies the susceptibility to lymphoma.<ref name="NocturneBoudaoud2014">{{cite journal |last1=Nocturne |first1=G. |last2=Boudaoud |first2=S. |last3=Miceli Richard |first3=C. |last4=Viengchareun |first4=S. |last5=Lazure |first5=T. |last6=Nititham |first6=J. |last7=Taylor |first7=K. E. |last8=Criswell |first8=L. A. |last9=Ma |first9=A. |last10=Busato |first10=F. |last11=Melki |first11=J. |last12=Dubost |first12=J. J. |last13=Hachulla |first13=E. |last14=Gottenberg |first14=J. E. |last15=Lombes |first15=M. |last16=Tost |first16=J. |last17=Mariette |first17=X. |title=OP0023 Germinal and Somatic Genetic Variants of TNFAIP3 Promote Lymphomagenesis Process Complicating Primary Sjögren's Syndrome |journal=Annals of the Rheumatic Diseases |date=June 2013 |volume=72 |issue=Suppl 3 |pages=A55.3–A56 |doi=10.1136/annrheumdis-2013-eular.228 |s2cid=75620379 }}</ref> ====Other organs==== Apart from the notably higher incidence of malignant NHL, Sjögren's patients show only modest or clinically insignificant deterioration in specific organ-related function.{{cn|date=December 2024}}
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