Editing Sjögren's disease (section)

== Prognosis ==
===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}}

===Burden of illness===
Sjögren's disease is associated with a high burden of illness,<ref>{{cite journal |last1=Vivino |first1=Frederick B. |title=Sjogren's syndrome: Clinical aspects |journal=Clinical Immunology |date=September 2017 |volume=182 |pages=48–54 |doi=10.1016/j.clim.2017.04.005 |pmid=28428095 }}</ref> and has been shown to markedly reduce [[HRQoL|quality of life]] (QoL),<ref>{{cite web | url=https://www.sjogrens.org/understanding-sjogrens/resources/patient-survey-results | title=Patient Survey Results }}</ref> with a significant impact on ability to work resulting from increased rates of disability.<ref>{{cite journal |last1=Meijer |first1=Jiska M. |last2=Meiners |first2=Petra M. |last3=Huddleston Slater |first3=James J. R. |last4=Spijkervet |first4=Fred K. L. |last5=Kallenberg |first5=Cees G. M. |last6=Vissink |first6=Arjan |last7=Bootsma |first7=Hendrika |title=Health-related quality of life, employment and disability in patients with Sjögren's disease |journal=Rheumatology |date=September 2009 |volume=48 |issue=9 |pages=1077–1082 |doi=10.1093/rheumatology/kep141 |pmid=19553376 }}</ref><ref name=":0">{{cite journal |last1=Miyamoto |first1=Samira T |last2=Valim |first2=Valéria |last3=Fisher |first3=Benjamin A |title=Health-related quality of life and costs in Sjögren's syndrome |journal=Rheumatology |date=18 June 2021 |volume=60 |issue=6 |pages=2588–2601 |doi=10.1093/rheumatology/key370 |pmid=30770918 |url=http://pure-oai.bham.ac.uk/ws/files/54982264/HRQoL_and_cost_revised_v2_final_clean_with_figures.pdf }}</ref><ref>{{cite journal |last1=Zhang |first1=Qiuxiang |last2=Wang |first2=Xulin |last3=Chen |first3=Haoyang |last4=Shen |first4=Biyu |title=Sjögren's syndrome is associated with negatively variable impacts on domains of health-related quality of life: evidence from Short Form 36 questionnaire and a meta-analysis |journal=Patient Preference and Adherence |date=10 May 2017 |volume=11 |pages=905–911 |doi=10.2147/PPA.S132751 |pmid=28546741 |pmc=5436777 |doi-access=free }}</ref> The reduction in QoL is similar to that seen in other chronic conditions such as [[rheumatoid arthritis]], [[lupus]] and [[fibromyalgia]].<ref name=":0" />

===Mortality===
Published studies on the survival of Sjögren's disease patients have been limited in varied respects, perhaps owing to the relatively small sample sizes and the fact that secondary Sjögren's disease is associated with other autoimmune diseases.
A 2010 study found a slight increase in [[mortality rate]]s of Sjögren's patients in comparison with the remainder of the population.<ref name=Voulgarelis2010/> A 2016 study found that primary Sjögren's was not associated with an increase in all-cause mortality as compared with the general population, but that a subset of patients with extraglandular involvement, vasculitis, hypocomplementaemia, and cryoglobulinaemia may be at increased risk of mortality.<ref>{{cite journal |last1=Singh |first1=Abha G. |last2=Singh |first2=Siddharth |last3=Matteson |first3=Eric L. |title=Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies |journal=Rheumatology |date=27 September 2015 |volume=55 |issue=3 |pages=450–460 |doi=10.1093/rheumatology/kev354 |pmid=26412810 |pmc=5009445 |doi-access=free }}</ref> A 2021 metaanalysis showed a 46% increase in mortality, with significantly greater mortality risk in patients with older age, male gender, vasculitis, interstitial lung disease, low complements, positive anti-La/SSB and cryoglobulinaemia.<ref>{{cite journal |last1=Huang |first1=Hong |last2=Xie |first2=Wenhui |last3=Geng |first3=Yan |last4=Fan |first4=Yong |last5=Zhang |first5=Zhuoli |title=Mortality in patients with primary Sjögren's syndrome: a systematic review and meta-analysis |journal=Rheumatology |date=1 September 2021 |volume=60 |issue=9 |pages=4029–4038 |doi=10.1093/rheumatology/keab364 |pmid=33878179 }}</ref>

Among those without other autoimmune disorders, [[life expectancy]] is unchanged.<ref name="Sin2016">{{cite journal| last1= Singh| first1=AG|last2=Singh|first2=S|last3=Matteson|first3=EL|date=March 2016|title=Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies |journal= Rheumatology| volume= 55| issue= 3| pages=450–60|doi=10.1093/rheumatology/kev354|pmid=26412810|pmc=5009445}}</ref>