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Sjögren's disease
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==Signs and symptoms== In a 2021 article on Sjogren's patients, a majority of individuals stated that eight Sjogren's symptoms had a major or moderate impact on their life: [[fatigue]] (79%); dry eyes (75%); dry mouth (73%); joint pain (65%); trouble sleeping (64%); eye discomfort (60%); muscle pain (56%); and brain fog (54%).<ref>{{cite journal | doi=10.1016/j.ajo.2020.05.043 | title=Ocular Manifestations and Burden Related to Sjögren Syndrome: Results of a Patient Survey | date=2020 | last1=Saldanha | first1=Ian J. | last2=Bunya | first2=Vatinee Y. | last3=McCoy | first3=Sara S. | last4=Makara | first4=Matthew | last5=Baer | first5=Alan N. | last6=Akpek | first6=Esen K. | journal=American Journal of Ophthalmology | volume=219 | pages=40–48 | pmid=32569739 | pmc=7606749 }}</ref><ref>{{cite journal | pmc=9427679 | date=2022 | last1=McCoy | first1=S. S. | last2=Woodham | first2=M. | last3=Bartels | first3=C. M. | last4=Saldanha | first4=I. J. | last5=Bunya | first5=V. Y. | last6=Maerz | first6=N. | last7=Akpek | first7=E. K. | last8=Makara | first8=M. A. | last9=Baer | first9=A. N. | title=Symptom-Based Cluster Analysis Categorizes Sjögren's Disease Subtypes: An International Cohort Study Highlighting Disease Severity and Treatment Discordance | journal=Arthritis & Rheumatology | volume=74 | issue=9 | pages=1569–1579 | doi=10.1002/art.42238 | pmid=35594474 }}</ref><ref>{{cite journal | pmc=9610846 | date=2022 | last1=McCoy | first1=S. S. | last2=Woodham | first2=M. | last3=Bunya | first3=V. Y. | last4=Saldanha | first4=I. J. | last5=Akpek | first5=E. K. | last6=Makara | first6=M. A. | last7=Baer | first7=A. N. | title=A comprehensive overview of living with Sjögren's: Results of a National Sjögren's Foundation survey | journal=Clinical Rheumatology | volume=41 | issue=7 | pages=2071–2078 | doi=10.1007/s10067-022-06119-w | pmid=35257256 }}</ref> Primary symptoms are dryness ([[xerostomia|dry mouth]] and [[keratoconjunctivitis sicca|dry eyes]]<ref name="Bri2016" />), pain and [[fatigue]].<ref>{{cite journal |last1=Cornec |first1=Divi |last2=Devauchelle-Pensec |first2=Valérie |last3=Mariette |first3=Xavier |last4=Jousse-Joulin |first4=Sandrine |last5=Berthelot |first5=Jean-Marie |last6=Perdriger |first6=Aleth |last7=Puéchal |first7=Xavier |last8=Le Guern |first8=Véronique |last9=Sibilia |first9=Jean |last10=Gottenberg |first10=Jacques-Eric |last11=Chiche |first11=Laurent |last12=Hachulla |first12=Eric |last13=Yves Hatron |first13=Pierre |last14=Goeb |first14=Vincent |last15=Hayem |first15=Gilles |last16=Morel |first16=Jacques |last17=Zarnitsky |first17=Charles |last18=Dubost |first18=Jean Jacques |last19=Saliou |first19=Philippe |last20=Pers |first20=Jacques Olivier |last21=Seror |first21=Raphaèle |last22=Saraux |first22=Alain |title=Severe Health-Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient-Reported Outcomes: Data From a Large Therapeutic Trial |journal=Arthritis Care & Research |date=April 2017 |volume=69 |issue=4 |pages=528–535 |doi=10.1002/acr.22974 |pmid=27390310 |s2cid=22904103 |doi-access=free }}</ref> Other symptoms can include [[dry skin]], vaginal dryness, a chronic cough, numbness in the arms and legs, [[fatigue (medical)|feeling tired]], muscle and joint pains, and [[thyroid problems]].<ref name= NIH2014Fact/> Those affected are also at an increased risk (15%) of [[lymphoma]].<ref name=Bri2016>{{cite journal |last1=Brito-Zerón |first1=Pilar |last2=Baldini |first2=Chiara |last3=Bootsma |first3=Hendrika |last4=Bowman |first4=Simon J. |last5=Jonsson |first5=Roland |last6=Mariette |first6=Xavier |last7=Sivils |first7=Kathy |last8=Theander |first8=Elke |last9=Tzioufas |first9=Athanasios |last10=Ramos-Casals |first10=Manuel |title=Sjögren syndrome |journal=Nature Reviews Disease Primers |date=7 July 2016 |volume=2 |issue=1 |pages=16047 |doi=10.1038/nrdp.2016.47 |pmid=27383445 |s2cid=4049076 |hdl=11568/809074 |hdl-access=free }}</ref><ref name="John2008">{{cite book| url= https://books.google.com/books?id=3zM9yCy8uqUC&pg=PA389 |title= Primer on the rheumatic diseases|last1=John H.| first1= Klippel| date =2008 |publisher= Springer |isbn= 978-0-387-68566-3 |edition= 13th |location= New York, New York |page=389|access-date=15 July 2016| archive-url= https://web.archive.org/web/20160815233354/https://books.google.ca/books?id=3zM9yCy8uqUC&pg=PA389 |archive-date=15 August 2016| url-status= live}}</ref> [[File:Sjogrens Syndrome.jpg|thumb|upright=1.3|Characteristic dryness appears at a number of locations, such as the tongue, face, and eyes. Marked at left are the salivary glands (which may be swollen), not a facial rash.]] The hallmark symptom of Sjögren's disease is [[xerostomia|dry mouth]] and [[keratoconjunctivitis sicca]] (dry eyes).<ref name=NIH2018/> [[Vaginal dryness]], [[xeroderma|dry skin]], and dry [[human nose|nose]] may also occur.<ref name=NIH2018/> Other organs of the body may also be affected,<ref>{{cite journal|last1=Sandhya|first1=Pulukool|last2=Jeyaseelan|first2=Lakshmanan|last3=Scofield|first3=Robert Hal|last4=Danda|first4=Debashish|date=26 June 2015|title=Clinical Characteristics and Outcome of Primary Sjogren's Syndrome: A Large Asian Indian Cohort|journal=The Open Rheumatology Journal|language=en|volume=9|issue=1|pages=36–45|doi=10.2174/1874312901409010036|pmid=26161156|pmc=4493630|doi-access=free}}</ref> including the kidneys, blood vessels, lungs, liver, pancreas, and brain.<ref name=NIH2018>{{cite web|title=Sjögren's Syndrome Information Page|url=https://www.ninds.nih.gov/Disorders/All-Disorders/Sj%C3%B6grens-Syndrome-Information-Page#disorders-r1|website=nih.gov|access-date=18 May 2018|archive-date=19 May 2018|archive-url=https://web.archive.org/web/20180519032953/https://www.ninds.nih.gov/Disorders/All-Disorders/Sj%C3%B6grens-Syndrome-Information-Page#disorders-r1|url-status=dead}}</ref><ref>{{cite web|title=Indian Rheumatology Association {{!}} IRA E-NEWSLETTER|url=https://www.indianrheumatology.org/ira-e-newsletter/issue/2020/july/patients-portal/sjogren-syndrome-expert-speaks|archive-url=https://web.archive.org/web/20200706070816/https://www.indianrheumatology.org/ira-e-newsletter/issue/2020/july/patients-portal/sjogren-syndrome-expert-speaks|url-status=dead|archive-date=July 6, 2020|access-date=6 July 2020|website=www.indianrheumatology.org}}</ref> In some people with Sjögren's disease, skin dryness may be the result of [[lymphocytic]] [[Infiltration (medical)|infiltration]] into [[skin glands]]. The symptoms may develop insidiously, with the diagnosis often not considered for several years because sicca (dryness) may be attributed to medications, a dry environment, or aging, or may be regarded as not of a severity warranting the level of investigation necessary to establish the presence of the underlying autoimmune disorder.<ref name=foxetal/> Sjögren's disease can damage [[vital organs]], with symptoms that may plateau or worsen, or go into [[Remission (medicine)|remission]], as with other autoimmune diseases. Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease. Many patients can treat problems [[symptomatically]]. Others experience [[blurred vision]], constant eye discomfort, recurrent [[mouth infections]], swollen [[parotid glands]], [[dysphonia]] ([[Vocal cord dysfunction|vocal disorders]] including hoarseness), and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life. Some patients can develop kidney involvement (autoimmune [[tubulointerstitial nephritis]]) leading to [[proteinuria]] (excess protein in urine), [[urinary concentrating defect]], and [[distal renal tubular acidosis]].<ref>{{cite web|title=Indian Rheumatology Association {{!}} IRA E-NEWSLETTER|url=https://www.indianrheumatology.org/ira-e-newsletter/issue/2020/july/rheumatologists-and-health-professionals/electrolyte-abnormalities-in-sjgren-syndrome|access-date=6 July 2020|website=www.indianrheumatology.org|archive-date=6 July 2020|archive-url=https://web.archive.org/web/20200706070815/https://www.indianrheumatology.org/ira-e-newsletter/issue/2020/july/rheumatologists-and-health-professionals/electrolyte-abnormalities-in-sjgren-syndrome|url-status=dead}}</ref> === Complications === Among the complications discussed above, women with anti-Ro/SS-A and anti-La/SS-B antibodies who become pregnant have an increased rate of [[neonatal lupus erythematosus]] with [[congenital]] [[heart block]] requiring a [[pacemaker]].<ref>{{cite journal |title=Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjögren's syndrome |journal= Ann. Rheum. Dis. |volume=63 |issue=11 |pages=1496–7 |date=November 2004 |pmid=15479901 |pmc=1754813 |doi=10.1136/ard.2003.014944 |last1=Manthorpe |first1=R |last2=Svensson |first2=A |last3=Wirestrand |first3=LE}}</ref> Type I [[cryoglobulinemia]] is a known complication of Sjögren's disease.<ref>{{cite journal|title=Cryoglobulinemia in primary Sjögren's syndrome: prevalence and clinical characteristics in a series of 115 patients |journal=Semin Arthritis Rheum. |volume=28 |issue=3 |pages= 200–5 |date= December 1998 |pmid=9872481 |doi=10.1016/S0049-0172(98)80037-1|last1=Ramos-Casals| first1= Manel| last2= Cervera |first2= Ricard|last3=Yagüe|first3=Jordi|last4=García-Carrasco |first4= Mario| last5= Trejo| first5= Olga| last6= Jiménez| first6= Sonia|last7=Morlà|first7=Rosa M|last8=Font|first8=Josep|last9=Ingelmo|first9=Miguel}}</ref> Sjögren's disease can affect such organs as the liver, pancreas, kidneys, lungs, and central nervous system.<ref>{{cite web |title=Symptoms |url=https://www.sjogrens.org/understanding-sjogrens/symptoms |website=Sjögren's Foundation |access-date=14 April 2020 |archive-date=19 April 2020 |archive-url=https://web.archive.org/web/20200419222815/https://www.sjogrens.org/understanding-sjogrens/symptoms |url-status=live }}</ref> ===Associated conditions=== Sjögren's disease is associated with a number of other medical conditions, many of which are [[autoimmune disease|autoimmune]] or [[rheumatic disorder|rheumatic]] disorders, such as [[coeliac disease|celiac disease]],<ref name=LundinWihmenga2015>{{cite journal| vauthors= Lundin KE, Wijmenga C|title=Coeliac disease and autoimmune disease–genetic overlap and screening|journal=Nat Rev Gastroenterol Hepatol|volume=12|issue=9|pages=507–15|date=Sep 2015|pmid=26303674|doi=10.1038/nrgastro.2015.136|s2cid=24533103|type=Review}}</ref><ref name=DenhamHill2013>{{cite journal|vauthors=Denham JM, Hill ID|title=Celiac disease and autoimmunity: review and controversies|journal=Curr Allergy Asthma Rep|volume=13|issue=4|pages=347–53|date=August 2013 |pmid= 23681421| pmc= 3725235 |doi=10.1007/s11882-013-0352-1|type=Review}}</ref> [[fibromyalgia]], [[Systemic lupus erythematosus|systemic lupus erythematosus (lupus)]], [[autoimmune thyroiditis]], [[multiple sclerosis]] and [[spondyloarthropathy]],<ref name=GabrielMichaud2009>{{cite journal|vauthors=Gabriel SE, Michaud K|title=Epidemiological studies in incidence, prevalence, mortality, and comorbidity of the rheumatic diseases| journal=Arthritis Res Ther|volume=11|issue=3|page=229|date=2009|pmid=19519924|pmc=2714099|doi=10.1186/ar2669|type=Review |doi-access=free }}</ref> and several [[malignancy|malignancies]], principally [[non-Hodgkin lymphoma]].<ref name=GabrielMichaud2009 /><ref name= PapageorgiouVoulgarelis2015>{{cite journal|vauthors=Papageorgiou A, Voulgarelis M, Tzioufas AG|title=Clinical picture, outcome and predictive factors of lymphoma in Sjӧgren syndrome|journal=Autoimmun Rev|volume=14|issue=7|pages=641–9|date=Jul 2015|pmid=25808075|doi=10.1016/j.autrev.2015.03.004|type=Review}}</ref> Sjogren's is the second most common cause of [[dysautonomia]].<ref>{{cite journal | doi=10.3389/fimmu.2021.702505 | doi-access=free | title=Autonomic Nervous System Dysfunction in Primary Sjögren's Syndrome | date=2021 | last1=Davies | first1=Kristen | last2=Ng | first2=Wan-Fai | journal=Frontiers in Immunology | volume=12 | pmid=34381453 | pmc=8350514 }}</ref><ref>{{cite journal | pmid=25622919 | date=2015 | last1=Imrich | first1=R. | last2=Alevizos | first2=I. | last3=Bebris | first3=L. | last4=Goldstein | first4=D. S. | last5=Holmes | first5=C. S. | last6=Illei | first6=G. G. | last7=Nikolov | first7=N. P. | title=Predominant Glandular Cholinergic Dysautonomia in Patients with Primary Sjögren's Syndrome | journal=Arthritis & Rheumatology | volume=67 | issue=5 | pages=1345–1352 | doi=10.1002/art.39044 | pmc=4414824 }}</ref><ref>{{cite web | url=https://my.clevelandclinic.org/health/diseases/6004-dysautonomia | title=Dysautonomia: Malfunctions in Your Body's Automatic Functions }}</ref><ref>{{cite web | url=https://sjogrens.org/blog/2023/dysautonomia-in-sjogrens | title=Dysautonomia in Sjögren's | date=26 October 2023 }}</ref>
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