Nephrotic Syndrome in Adults

National Kidney and Urologic Diseases Information Clearinghouse

What is nephrotic syndrome?

U.S. Department of Health and Human Services NATIONAL INSTITUTES OF HEALTH

Nephroticsyndromeisacollectionof symptomsthatindicatekidneydamage. Nephroticsyndromeincludesthefollowing: proteinurialargeamountsofprotein intheurine hyperlipidemiahigherthannormalfat andcholesterollevelsintheblood edema,orswelling,usuallyinthelegs, feet,oranklesandlessofteninthe handsorface hypoalbuminialowlevelsofalbumin intheblood Albuminisaproteinthatactslikeasponge, drawingextrafluidfromthebodyintothe bloodstreamwhereitremainsuntilremoved

bythekidneys.Whenalbuminleaksintothe urine,thebloodlosesitscapacitytoabsorb extrafluidfromthebody,causingedema. Nephroticsyndromeresultsfromaproblem withthekidneysfilters,calledglomeruli. Glomeruliaretinybloodvesselsinthe kidneysthatremovewastesandexcessfluids fromthebloodandsendthemtothebladder asurine. Asbloodpassesthroughhealthykidneys,the glomerulifilteroutthewasteproductsand allowthebloodtoretaincellsandproteins thebodyneeds.However,proteinsfrom theblood,suchasalbumin,canleakinto theurinewhentheglomeruliaredamaged. Innephroticsyndrome,damagedglomeruli allow3gramsormoreofproteintoleakinto theurinewhenmeasuredovera24-hour period,whichismorethan20timesthe amountthathealthyglomeruliallow.

Vein
(clean blood leaves the kidney)

Glomeruli
(tiny filters in the kidney)

Kidneys

Artery Ureters
(blood and waste enter the kidney)

Bladder

Ureter
(waste and fluids go out in urine)

Asbloodpassesthroughhealthykidneys,theglomerulifilteroutthewasteproductsandallowthebloodto retaincellsandproteinsthebodyneeds.

What causes nephrotic syndrome?

Nephroticsyndromecanbecausedbydiseasesthataffectonlythekidneys,suchas focalsegmentalglomerulosclerosis(FSGS) ormembranousnephropathy.Diseasesthat affectonlythekidneysarecalledprimary causesofnephroticsyndrome.Theglomeruliareusuallythetargetsofthesediseases forreasonsthatarenotfullyunderstood.In FSGSthemostcommonprimarycause ofnephroticsyndromescartissueforms inpartsoftheglomeruli.Inmembranous nephropathy,immunemoleculesformharmfuldepositsontheglomeruli. Nephroticsyndromecanalsobecausedby systemicdiseases,whicharediseasesthat affectmanypartsofthebody,suchasdiabetesorlupus.Systemicdiseasesthataffect thekidneysarecalledsecondarycausesof nephroticsyndrome.Morethan50percent ofnephroticsyndromecasesinadultshave secondarycauses,withdiabetesbeingthe mostcommon.1

What are the complications of nephrotic syndrome?

Thelossofdifferentproteinsfromthebody canleadtoavarietyofcomplicationsin peoplewithnephroticsyndrome.Blood clotscanformwhenproteinsthatnormally preventthemarelostthroughtheurine. Bloodclotscanblocktheflowofbloodand oxygenthroughabloodvessel.Lossof immunoglobulinsimmunesystemproteins thathelpfightdiseaseandinfectionleads toanincreasedriskofinfections.These infectionsincludepneumonia,alunginfection;cellulitis,askininfection;peritonitis,an abdominalinfection;andmeningitis,abrain andspineinfection.Medicationsgivento treatnephroticsyndromecanalsoincrease theriskoftheseinfections.Othercomplicationsofnephroticsyndromeinclude hypothyroidismaconditioninwhich thethyroidglanddoesnotproduce enoughthyroidhormonetomeetthe bodysneeds anemiaaconditioninwhichredblood cellsarefewerorsmallerthannormal, whichmeanslessoxygeniscarriedto thebodyscells coronaryarterydisease,alsocalled coronaryheartdiseaseheartdisease causedbynarrowingofthearteriesthat supplybloodtotheheart highbloodpressure,alsocalled hypertensionaconditioninwhich bloodflowsthroughthebloodvessels withaforcegreaterthannormal acutekidneyinjurysuddenandtemporarylossofkidneyfunction

What are the signs and symptoms of nephrotic syndrome?

Inadditiontoproteinuria,hyperlipidemia, edema,andhypoalbumina,peoplewith nephroticsyndromemayexperience weightgain fatigue foamyurine lossofappetite

OnlineMedicalLibrary.www.merckmanuals.com/ professional/genitourinary_disorders/glomerular_ disorders/nephrotic_syndrome.html.UpdatedJanuary 2010.AccessedFebruary15,2012.

1NephroticSyndrome.TheMerckManuals

2 NephroticSyndromeinAdults

How is nephrotic syndrome diagnosed?

Urinesamplesaretakentodiagnosepeople suspectedofhavingnephroticsyndrome. Nephroticsyndromeisdiagnosedwhenlarge amountsofproteinarefoundintheurine. Thebloodproteinalbuminmakesupmuch oftheproteinthatislost,thoughmanyother importantproteinsarealsolostinnephrotic syndrome. Thepresenceofalbuminintheurinecanbe detectedwithadipsticktestperformedona urinesample.Theurinesampleiscollected inaspecialcontainerinahealthcareprovidersofficeorcommercialfacilityandcan betestedinthesamelocationorsenttoalab foranalysis.Forthetest,anurseortechnicianplacesastripofchemicallytreated paper,calledadipstick,intotheurine. Patchesonthedipstickchangecolorwhen proteinispresentinurine. Amoreprecisemeasurementisusually neededtoconfirmthediagnosis.Eithera singleurinesampleora24-hourcollection ofurinecanbesenttoalabforanalysis. Withthesingleurinesample,thelabmeasuresbothalbuminandcreatinine,awaste productofnormalmusclebreakdown.The comparisonofthemeasurementsiscalleda urinealbumin-to-creatinineratio.Aurine samplecontainingmorethan30milligrams ofalbuminforeachgramofcreatininemay signalaproblem.Witha24-hourcollection ofurine,thelabmeasuresonlytheamount ofalbuminpresent.Thesingleurinesample iseasiertocollectthanthe24-hoursample andisusuallysufficienttoconfirmdiagnosis, thoughthe24-hourcollectionmaybeusedin somecases.

Oncenephroticsyndromeisdiagnosed, bloodtestsareusuallyneededtocheckfor systemicdiseasesthatmaybecausingthe nephroticsyndromeandtofindouthowwell thekidneysareworkingoverall.Ablood testinvolvesdrawingbloodatahealthcare providersofficeorcommercialfacilityand sendingthesampletoalabforanalysis. Thoughbloodtestscanpointtowardsystemicdiseases,akidneybiopsyisusually neededtodiagnosethespecificunderlying diseasecausingthenephroticsyndromeand todeterminethebesttreatment.Akidney biopsyisaprocedurethatinvolvestakinga pieceofkidneytissueforexaminationwitha microscope.Kidneybiopsiesareperformed byahealthcareproviderinahospitalwith lightsedationandlocalanesthetic.Abiopsy isoftennotneededforapersonwithdiabetesbecausethepersonsmedicalhistory andlabtestsmaybeenoughtodiagnosethe problemasbeingaresultofdiabetes.

How is nephrotic syndrome treated?

Treatingnephroticsyndromeincludes addressingtheunderlyingcauseaswellas takingstepstoreducehighbloodpressure, edema,highcholesterol,andtherisksof infection.Treatmentusuallyincludesmedicationsandchangesindiet. Medicationsthatlowerbloodpressurecan alsosignificantlyslowtheprogressionof kidneydiseasecausingnephroticsyndrome. Twotypesofbloodpressureloweringmedications,angiotensin-convertingenzyme (ACE)inhibitorsandangiotensinreceptor blockers(ARBs),haveproveneffectivein slowingtheprogressionofkidneydiseaseby reducingthepressureinsidetheglomeruli

3 NephroticSyndromeinAdults

andtherebyreducingproteinuria.Many peoplerequiretwoormoremedicationsto controltheirbloodpressure.Inadditionto anACEinhibitororanARB,adiuretica medicationthataidsthekidneysinremovingfluidfromthebloodcanalsobeuseful inhelpingtoreducebloodpressureaswell asedema.Betablockers,calciumchannel blockers,andotherbloodpressuremedicationsmayalsobeneeded. Statinmedicationsmaybegiventolower cholesterol. Peoplewithnephroticsyndromeshould receivethepneumococcalvaccine,which helpsprotectagainstabacteriumthatcommonlycausesinfection,andyearlyflushots. Bloodthinningmedicationsareusuallyonly giventopeoplewithnephroticsyndrome whodevelopabloodclot;thesemedications arenotusedasapreventivemeasure. Nephroticsyndromemaygoawayoncethe underlyingcausehasbeentreated.More informationabouttreatingtheunderlyingcausesofnephroticsyndromecan befoundintheNationalKidneyand UrologicDiseasesInformationClearinghousepublicationGlomerular Diseasesat www.kidney.niddk.nih.gov.

Points to Remember
Nephroticsyndromeincludesthe following: proteinurialargeamountsof proteinintheurine hyperlipidemiahigherthan normalfatandcholesterollevels intheblood edema,orswelling,usuallyinthe legs,feet,oranklesandlessoften inthehandsorface hypoalbuminialowlevelsalbu minintheblood Primarycausesofnephroticsyndromearediseasesthataffectonly thekidneys,suchasfocalsegmental glomerulosclerosis(FSGS).Secondarycausesofnephroticsyndromearediseasesthataffectmany partsofthebody,suchasdiabetes. Inadditiontoproteinuria,hyperlipidemia,edema,andhypoalbumina, peoplewithnephroticsyndrome mayexperience weightgain fatigue foamyurine lossofappetite Thelossofdifferentproteinsfrom thebodycanleadtoavariety ofcomplicationsinpeoplewith nephroticsyndrome. Treatingnephroticsyndrome includesaddressingtheunderlying causeandtakingstepstoreduce highbloodpressure,edema,high cholesterol,andtherisksofinfection.Treatmentusuallyincludes medicationsandchangesindiet.

Eating, Diet, and Nutrition

Eating,diet,andnutritionhavenotbeen showntoplayaroleincausingorpreventing nephroticsyndromeinadults.Forpeople whohavedevelopednephroticsyndrome, limitingintakeofdietarysodium,oftenfrom salt,andfluidmayberecommendedtohelp reduceedema.Adietlowinsaturatedfat andcholesterolmayalsoberecommendedto helpcontrolhyperlipidemia.

4 NephroticSyndromeinAdults

Hope through Research

Inrecentyears,researchershavelearned muchaboutkidneydisease.TheNational InstituteofDiabetesandDigestiveand KidneyDiseases(NIDDK)sponsorsseveral programsaimedatunderstandingglomerulardiseasessuchasFSGSandmembranous nephropathy,whichareprimarycausesof nephroticsyndrome.TheNIDDKalsostudiesdiseasessuchaslupusanddiabetes,which aresecondarycausesofnephroticsyndrome. Findingtreatmentsfortheseunderlying causeswillhelppreventnephroticsyndrome orstopitsprogression. Participantsinclinicaltrialscanplayamore activeroleintheirownhealthcare,gain accesstonewresearchtreatmentsbefore theyarewidelyavailable,andhelpothers bycontributingtomedicalresearch.For informationaboutcurrentstudies,visit www.ClinicalTrials.gov.

National Kidney Foundation 30East33rdStreet NewYork,NY10016 Phone:18006229010or2128892210 Fax:2126899261 Internet:www.kidney.org The NephCure Foundation 15WaterlooAvenue Berwyn,PA19312 Phone:18666374287or6105400186 Email:info@nephcure.org Internet:www.nephcure.org The Nephrotic Syndrome Study Network (NEPTUNE) UniversityofMichigan 206SimpsonMemorialInstitute 102Observatory AnnArbor,MI48109 Phone:18779NEPTUNE (18779637886)or7346155021 Fax:7346156005 Email:NEPTUNE-Study@umich.edu Internet:www.neptune-study.org

For More Information

American Kidney Fund 6110ExecutiveBoulevard,Suite1010 Rockville,MD20852 Phone:18006388299 Internet:www.kidneyfund.org National Heart, Lung, and Blood Institute Health Information Center P.O.Box30105 Bethesda,MD208240105 Phone:3015928573 TTY:2406293255 Fax:2406293246 Email:nhlbiinfo@nhlbi.nih.gov Internet:www.nhlbi.nih.gov

Acknowledgments
PublicationsproducedbytheClearinghouse arecarefullyreviewedbybothNIDDKscientistsandoutsideexperts.Thispublication wasreviewedbyPatrickGipson,M.D.,and MatthiasKretzler,M.D.,bothoftheUniversityofMichiganMedicalCenter.

Youmayalsofindadditionalinformationaboutthis topicbyvisitingMedlinePlusatwww.medlineplus.gov. Thispublicationmaycontaininformationabout medications.Whenprepared,thispublication includedthemostcurrentinformationavailable. Forupdatesorforquestionsaboutanymedications, contacttheU.S.FoodandDrugAdministrationtollfreeat1888INFOFDA(18884636332)orvisit www.fda.gov.Consultyourhealthcareproviderfor moreinformation.

5 NephroticSyndromeinAdults

National Kidney and Urologic Diseases Information Clearinghouse

3InformationWay Bethesda,MD208923580 Phone:18008915390 TTY:18665691162 Fax:7037384929 Email:nkudic@info.niddk.nih.gov Internet:www.kidney.niddk.nih.gov TheNationalKidneyandUrologicDiseases InformationClearinghouse(NKUDIC) isaserviceoftheNationalInstituteof DiabetesandDigestiveandKidneyDiseases (NIDDK).TheNIDDKispartofthe NationalInstitutesofHealthoftheU.S. DepartmentofHealthandHumanServices. Establishedin1987,theClearinghouse providesinformationaboutdiseasesofthe kidneysandurologicsystemtopeoplewith kidneyandurologicdisordersandtotheir families,healthcareprofessionals,andthe public.TheNKUDICanswersinquiries, developsanddistributespublications,and workscloselywithprofessionalandpatient organizationsandGovernmentagencies tocoordinateresourcesaboutkidneyand urologicdiseases.

Thispublicationisnotcopyrighted.TheClearinghouse encouragesusersofthispublicationtoduplicateand distributeasmanycopiesasdesired. Thispublicationisavailableat www.kidney.niddk.nih.gov.

U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES National Institutes of Health

NIHPublicationNo.124624 March2012
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