Sjogren's Syndrome - American College of Rheumatology

Sjögren's (SHOW'-grenz) is a systemic autoimmune, rheumatic disease that affects the entire body. The most common problems are dry mouth, dry eyes, fatigue and ... ContributeNow RheumPAC(ForUSmembers) RheumatologyResearchFoundation Doyouneedhelp? 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TentimesasmanywomenasmenarediagnosedwithSjögren's.Whilemostoftendiagnosedinwomenduringmiddleageoraftermenopause,Sjögren'scanaffectpeopleofanyage,includingchildren.YoungerSjögren'spatientsoftenpresentdifferentlycomparedtoadults. Sjögren'spatientsshouldbemonitoredcloselyforriskfactorsanddevelopmentofbloodcancers,especiallynon-HodgkinBcelllymphoma(seebelow). PregnantSjögren'spatientswhoarepositivefortheautoantibodySSA/Roshouldseekcarefromaperinatologist(obstetricianwhomanageshighriskpregnancies)andbemonitoredforsignsoffetalheartblock. AbouthalfofthosewithSjögren'shaveanotherautoimmunedisease,suchasrheumatoidarthritis,lupus,orscleroderma,sopatientswithotherautoimmunediseasesshouldalsobeassessedforsignsofSjögren's. NosystemicimmunomodulatingtherapyhasbeenFDA-approvedforSjögren's.However,manypotentialtreatmentsareusedandothersbeinginvestigated.Patientsmaybetreatedwithnonsteroidalanti-inflammatorydrugs(NSAIDs),corticosteroids,hydroxychloroquine,andotherdrugsthatcalmtheimmunesystem.Treatmentsfordrynessaimtorelievesymptomsandpreventcomplicationssuchasthrush,otherinfections,anddentaldamage. Accordingtothe2008NationalArthritisDataWorkgroupreport(whichincludedtheAmericanCollegeofRheumatology)ontheprevalenceofrheumaticdiseases,Sjögren'spatientswithoutanaccompanying,majorautoimmuneconnectivetissuediseasenumberbetween400,000and3.1millionadultsand,whenincludingpatientswhoalsohaveanotherrelateddisease,theprevalenceistwicethatnumber.IntreatingSjögren'spatients,embracingamultidisciplinaryteamapproachandcommunicationwithothermedicaldisciplinesiscriticalforoptimalmanagement. Inthe1930s,SwedishphysicianHenrikSjögrendescribedagroupofwomenwhohadprofounddryeyesanddrymouthmanyofwhomalsohadchronicarthritis.Today,rheumatologistsknowmoreaboutthediseasenamedforSjögrenandcanofferpatientsadviceabouthowtoliveandmanageit. WhatIsSjögren'sDisease? Sjögren'sisasystemic,autoimmune,rheumaticdiseasethatcanaffectmanydifferentbodyparts,especiallythemoisture-producingglands,andcausewidespreaddrynessandotherseriousproblems.Althoughdrymouthanddryeyesarethemostcommonsymptoms,drynesscanalsooccurinthenose,sinuses,ears,throat,skin,and,inwomen,thevagina.Thereisspeculationthat,inmenwithSjögren's,theprostatemightbeaffectedsimilarlytootherorgans.Theseproblemsmayinterferewithwork,socialactivities,andqualityoflife.Patientsmaynoticeirritation,agrittyfeeling,orpainfulburningintheeyes;dryeyesareatincreasedriskforinfectionandsusceptibletocornealdamageifnottreated.Drymouthcanleadtodifficultyeatingandswallowingdryfoods.Itcanleadtodentalcavities,chipping,breaking,andlossofteeth.Drymouthmayincreasegingivitis(guminflammation)andoralyeastinfections(candida)thatmaycausepainandburning.Sjögren'sfrequentlycausesswellingoftheparotidglands(theglandsbelowtheearsandrunalongthejawline).Drynesscanincreaseinfectionsintheeyes,mouth,sinuses,lungs,andvagina. Sjögren'scanalsoaffectthejoints,muscles,nervoussystem(centralnervoussystemandperipheralnervoussystem,includingtheautonomicnervoussystem),gastrointestinaltract(includingthepancreasandliver),skin,bloodvessels,lungs,andkidneys.Jointpainandstiffnesswithmildswellingarecommon,eveninthosewithoutrheumatoidarthritis.Rashesmayoccur,includinginflammationofsmallbloodvessels(vasculitis),mostcommonlyonthelowerlegs.Sun-sensitiverashismorecommonontheback,chest,face,andarms.Peripheralneuropathycancausenumbnessandtingling,especiallyinthefeet,andcanfrequentlypre-datesymptomsofdryness.Fatigue,cognitivedysfunction,andsleepabnormalitiesarefrequentlyreportedsymptoms.Patientsshouldalsobemonitoredfordepressionandanxiety.. Sjögren'scanbeaccompaniedbyotherautoimmuneconnectivetissuedisorders,suchassystemiclupuserythematosus,rheumatoidarthritis,scleroderma,autoimmunethyroiddisease,antiphospholipidsyndrome,sarcoidosis,andceliacdisease.Itcanoccasionallybeconfusedwithfibromyalgia,multiplesclerosis,andanewlydescribeddiseasecalledIgG4-relateddisease. WhatCausesSjögren'sDisease? ThecauseofSjögren'sisunknown,butweknowitisanautoimmunedisorder.Thissuggeststhattheimmunesystem,whichnormallyprotectsthebodyagainstcancersandinfections,isreactingagainstitsowntissues.Theformationofabnormalantibodies(calledautoantibodies)inbloodthatreacttoself-proteinsisamarkerand/orcontributortothisprocess.Thedecreaseintearsandsalivaoccurswhentheglandsthatproducethesefluidsbecomedysfunctionalanddamagedbyinflammationandinfiltrationbybloodcellscalledlymphocytes.Researchsuggeststhatgeneticfactorsandsexhormoneswhencombinedwithanenvironmentalagent,suchasaviralinfection,maytriggerdevelopmentofthedisease. HowIsSjögren'sDiseaseDiagnosed? Diagnosisdependsonacombinationofsymptoms,physicalexamination,bloodtests,andspecialstudiestolookforobjectiveevidenceofdryeyesanddrymouth.ThediagnosisofSjögren’scannotbebasedonsymptomsalonebecausedryeyesandmoutharehighlyprevalentsymptomsinthegeneralpopulationandcanbecausedbymanyotherconditionsormedications.Specialtestsmayassessfordecreasesintearorsalivaproduction.Aneyeexaminationwithstainingofthecorneaandconjunctivawithvitaldyeshelpsdetectandassessfordamage(i.e.dryspots)totheoutersurfaceoftheeyescausedbydryness.Bloodtestscanlookforautoantibodiesthatoftenoccurinthisdisease.Typicalantibodiesincludeanti-nuclearantibodies(ANA),anti-SSA/Ro,SSB/Laantibodiesorrheumatoidfactor.Biopsyoftheminorsalivaryglands(usuallytakenfromtheinnerlowerlip)mayalsobeusedtomakeadiagnosiswhenautoantibodiesareabsent. ChildrenandyoungadultsoftenpresentwithdifferentsignsandsymptomsofSjögren'scomparedtoolderadults.Initialsymptomsmorecommonlyincludeswollenparotidglandsandarthralgias(jointpains).Thepediatricpopulationalsomayhaveneurologicandkidneymanifestations.Drynessfeaturesmightormightnotbepresentatdiagnosis. HowIsSjögren'sDiseaseTreated? Currently,nosystemicimmunomodulatingtherapiesareFDA-approvedforSjögren's.Hydroxychloroquine(Plaquenil™),anantimalarialdrug,maybehelpfulinsomepatientswithSjögren'sandreducejointpain,fatigue,orrash.Patientswithsystemicproblems,suchasfever,severerash,,lungdisease,neurologicproblemsorkidneyinvolvementmayrequiretreatmentwithcorticosteroids(suchasprednisoneandmethylprednisolone)and/orimmunosuppressivedrugslikemethotrexate,azathioprine(Imuran™),mycophenolatemofetil(CellCept™),leflunomide(Arava™),orcyclophosphamide(Cytoxan™).Inaddition,biologicaltherapies,suchasrituximab(Rituxan™),maybeused,especiallyinseveredisease. Dryeyesusuallyrespondtoartificialtearsappliedregularlyduringthedayortoointmentappliedatnight.Othermeasures,suchaspluggingorblockingtearductsbytheopthalmologist,canbeusedinmoreseverecases.Eyedropsthatreduceinflammationintheglandsaroundtheeyes,suchascyclosporine(Restasis™andCequa™)andlifitegrast(Xiidra™),maysignificantlyimprovesymptomsanddecreasetheneedforartificialtears.Drinkingwater,usingsugarfreegumsandcandiessweetenedwithxylitol,orusingsalivasubstitutesmayrelievedrymouth.Manypatientsbenefitfromusingprescriptionmedicationsthatstimulatesalivaflow,suchaspilocarpine(Salagen™)orcevimeline(Evoxac™).Ifpatientsdevelopyeastinfections,anti-fungaltherapiesmaybeneeded.Humidifiersandnasalsalineirrigationmayimprovenasaldryness.Medicationsthatreducegastricacid(suchasproton-pumpinhibitorsandH2blockers)maylessensymptomsofacidreflux Allpatientsshouldreceiveregulardentalcaretopreventcavitiesandtoothloss.Fluorideintheformofprescription-strengthtoothpasteorvarnishesadministeredbythedentistisknowntopreventthesecomplications.Patientswithdryeyesshouldregularlyseeaneyedoctor(ophthalmologistoroptometrist)tomonitorforsignsofdamagetothecornea.Patientswithexcessiverednessandpainintheeyesshouldbeevaluatedforinfections. BroaderHealthImpactofSjögren’sDisease Symptomsvaryintypeandintensity,buttheburdenofillnessformanypeoplewithSjögren'sishigh.Regularmedicalcare,follow-up,andreferraltootherspecialistsasneededareimportantforoptimalcare.InadditiontohavingarheumatologistorknowledgeableprimarycareproviderwhounderstandsSjögren'stocoordinateapatient'scare,allSjögren'spatientsshouldregularlyseeaneyedoctoranddentistororalmedicinespecialist.Doctorswhospecializeintheblood(hematologist),nerves(neurologist),lungs(pulmonologist),intestinaltract(gastroenterologist),kidneys(nephrologistandurologist),gynecologist,andotherspecialistsmaybecalleduponasneeded. OneofthemostseverepotentialcomplicationsofSjögren'sislymphoma,acancerofthelymphglands.Lymphomaoccursinupto10%ofSjögren'spatients.ThehighestriskisforB-cellnon-Hodgkinlymphoma,withtheparotidglandbeingacommonsite.OthercancersoccurringatahigherrateinSjögren'sincludemultiplemyeloma,thyroid,andstomachcancer. RecognitionofpotentiallunginvolvementinSjögren'shasincreasedinrecentyears.TenpercentofpatientsarediagnosedwithinterstitiallungdiseaseinthefirstyearfollowingdiagnosiswithSjögren'sand20%withinfiveyears.CysticlungdiseaseisidentifiedmorefrequentlyinSjögren'scomparedtootherconnectivetissuediseases.Asmanyas65%ofSjögren'spatientswithnolungdiseasesymptomswillhaveabnormalimagingstudies. Neurologicalmanifestationsmayincludenumbnessandtingling,especiallyinthefeetandlegs.However,thehands,face,andotherareasmayalsobeinvolved.Weaknessandabnormalgaitmayoccurinseverecases.Involvementoftheautonomicnervoussystemhascometotheforewithlong-termCOVID.Moreattentionisbeingpaidtosymptomsofautonomicnervedysfunction(dysautonomia)inSjögren's.Symptomsofdysautonomiacanincludeheartrateabnormalities,sweating,bloodpressurefluctuations,andtemperatureregulation,andaffectdigestion,bladdercontrol,andbalance.OnetypeofdysautonomiaisPOTS(posturalorthostatictachycardiasyndrome),whichcancausedizzinessandfaintness,especiallyuponstanding. Fetalheartblockduetoanti-SSA/Roautoantibodiesmayoccurinbabiesofpregnantmothers.Monitoringforthispotentialcomplicationinthefetusisparamount,sotreatmentcanbeinitiatedinuterooruponbirth.TheneedformonitoringSjögren'spatientsforcardiovasculardisease,includingpulmonaryarterialhypertension,atherosclerosis,andothercardiovascularevents,alsoshouldberecognized. LivingwithSjögren'sDisease Sjögren'scannotbecured,butinmanycases,propertreatmenthelpstoalleviatesymptomsandpreventcomplications.Rheumatologistsaremusculoskeletalandautoimmunediseasespecialistsand,therefore,morelikelytomakeacorrectdiagnosisofSjogren’sthanothercareproviders.Theyalsocanadvisepatientsaboutthebestavailabletreatmentoptionsandcoordinatecarefromotherspecialistswhenneeded. PeoplewithSjögren'susuallycanlivefullliveswithproperself-careandthecareofknowledgeablehealthcareprofessionals.Patientsshouldbemonitoredregularlyforthedevelopmentofseverecomplications,suchaslymphoma,vasculitis,andpulmonary,kidney,andliverdisease,anypainorrednessintheeyesshouldbeevaluatedpromptly,asthismaysignalaninfectionorcornealdamage.Toreducetheriskforcavitiesandotherdentalproblems,patientsmustfollowprotocolsfordrymouth,suchasregularuseoffluoride,followastrictoralhygieneregimen,andseekregulardentalcare. Patientsshouldseetheirphysicianregularlyforgeneralhealthscreeningandpreventativemedicine.Theyshouldalsopaycloseattentiontoanyabnormalpersistentswellingintheglandsaroundthefaceorneck,underthearms,orinthegroinareas,asthismaysignifylymphoma. UpdatedDecember2021bytheSjögren’sFoundationandtheAmericanCollegeofRheumatologyCommitteeonCommunicationsandMarketing. Thisinformationisprovidedforgeneraleducationonly.Individualsshouldconsultaqualifiedhealthcareproviderforprofessionalmedicaladvice,diagnosisandtreatmentofamedicalorhealthcondition. Resources Sjögren'sSyndromeinSpanish(Español) DownloadPrint-FriendlyPDF QuickLinks GlossaryofRheumatologyTerms ShareYourStory