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RheumatologyMain1-10Q1A54-year-oldmancomestothephysiciancomplainingofmorningfacialpuffinessandbiiaterallegswelling.Hisothermedicalproblemsincluderecurrentpulmonaryinfectionsduetobronchiectasisandpsoriasis.Hisbloodpressureis143/92mmHgandpulseis92/min,Hisbodymassindexis24kg/㎡.Examinationshowsafourthheartsound.Hepatomegaly,palpablekidneys,and2+pittingedemaofthelowerextremitiestothekneesbilaterallyarepresentonexamination.Urinalysisshows4+proteinuriaandnormalurinarysediment.WhichofthefollowingisthemostlikelydiagnosisInthispatient?A.AmyloidosisB.HepatorenalsyndromeC.HypertensivenephrosclerosisD.IgAnephropathyE.PolycysticrenaldiseaseA1Correctanswer:AThispatient'sciinicalpresentationoffacialswelling,bilaterallower-extremityedema;andmassiveproteinuriaareconsistentwithnephroticsyndrome.Thepresenceofnephroticsyndromewithpalpablekidneys,hepatomegaly,andventricularhypertrophy(suggestedbyanaudiblefourthheartsound)inthesettingofchronicinflammatorydisease(recurrentpulmonaryinfections,bronchiectasis)issuggestiveofsecondaryamyloidosis(AA)asacommonunifyingdiagnosis.Treatmentisusuallydirectedattheunderlyinginflammatorydisease.ColchicineisapprovedforboththetreatmentandprophylaxisofAA.(ChoiceB)Hepatorenalsyndromereferstoacuterenalfailureinpatientswithacuteorchronicliverdiseaseandportalhypertension.Itusuallypresentsinpatientswithsevereliverdiseasewithprogressivelyrisingserumcreatininewithminimalproteinuria(500mg/day).(ChoiceC)HypertensivenephrosclerosisisseeninpatientswithchronichypertensionandIsassociatedwithretinopathy,leftventricularhypertrophy,progressiverenalfailure,andmildproteinuria(generally1g/day).Kidneysareusuallysmall,andhepatomegalyisnotpresent.(ChoiceD)IgAnephropathyisatypeofnephritisthattypicallypresentswithhematuriaonurinalysis.Unlikeinthispatient,IgAnephropathyIsusuallyrestrictedtothekidneys.(ChoiceE)Patientswithpolycystickidneydiseasetypicallypresentwithflankpain,hematuria,renalfailure,hypertension,andlargepalpablekidneys.Hepatomegalycanalsobepresentduetoco-occurrenceofhepaticcystsinsomepatients.However,theyusuallydonothavetheproteinuriaorgeneralizededemaseeninthispatient.Educationalobjective:Secondaryamyloidosisisacomplicationofachronicinflammatorycondition(eg,chronicinfections,inflammatoryboweldisease,rheumatoidarthritis)resultinginextracellulartissuedepositionoffibrilsintotissuesandorgans.Patientscandevelopmulti-organdysfunction(eg,kidneys,liver,gastrointestinaltract).TreatmentusuallyinvolvestreatingtheunderlyingInflammatorydisease.Q2A24-year-oldwomancomestotheofficewithseveralmonthsofslowlyprogressivelowbackandbuttockpain.Herpainismostsevereearlyinthemorningandfadesasthedayprogresses.Thepainisrelievedwithawarmshowerorover-the-counteranalgesics.Associatedsymptomsincludestiffness,butthepatienthasnofeverorgastrointestinalsymptoms.Thereisnorecenthistoryofsignificantillnessortrauma.Vitalsignsarenormal.Onexamination,thereislimitedrangeofmotioninthelumbarspine.Musclestrengthisnormalandequalinallextremities.Deeptendonreflexesare2+intheupperandlowerextremities.Otherexaminationfindingsareunremarkable.Plainx-rayfindingsareshownintheimagebelow.Whichofthefollowingconditionsisthispatientatgreatestriskofdeveloping?A.AnterioruveitisB.AorticcoarctationC.EpiscleritisD.IschemicopticneuropathyE.ObstructivelungdiseaseF.OralulcersA2Correctanswer:AThisyoungpatienthaschronicbackpainandstiffnessthatimprovewithactivity(stretching),warmshowers,andnonsteroidalagents,suggestiveofinflammatorybackpain.Inaddition,shehasreducedspinalrangeofmotionandanx-rayshowingarthritisanderosionsinvolvingthesacroiliacjoints.Thisisdiagnosticofankylosingspondylitis(AS).Theincidenceishighestatage20-30,andthereIsastrongassociationwithHLA-B27.Anterioruveitis(iritis)isthemostcommonextraarticularmanifestationofASandoccursin25%-4G%ofpatients.ItIscharacterizedbyinflammationoftheuvealtract(iris,ciliarybody,andchoroid).Anterioruveitistypicallypresentswithintensepainandphotophobiainoneeye.(ChoiceB)ASisassociatedwithaorticvalvedisease,typicallyaorticregurgitation,duetoscarringofthevalvecusps.CoarctationisassociatedwithTurnersyndromebutnotAS.'(ChoiceC)Episcleritisischaracterizedbyinflammationseenatthewhiteoftheeyerwithoutinvolvementoftheuvealtract.ItismoststronglyassociatedwithrheumatoidarthritisandinflammatoryboweldiseasebutisnotassociatedwithAS.(ChoiceD)Ischemicopticneuropathyisafearedcomplicationoftemporalarteritis.Itresultsfromvasculitisoftheretinalarteryandmaycauseblindness.(ChoiceE)AScancauserestrictivelungphysiologyduetoimpairedchestexpansionandapicalpulmonaryfibrosis,itdoesnotcauseobstructivelungdisease.(ChoiceF)Oralulcersareassociatedwithanumberofautoimmunedisorders(eg,systemiclupuserythematosus,Behcetdisease.Crohndisease).Educationalobjective:Anterioruveitisisthemostcommonextraskeletaicomplicationofankylosingspondylitis.Itcausesinflammationintheiris,ciliarybodyandchoroid,andpresentswithunilateralocularpainandphotophobia.Q3A23-year-oldwomancomestotheofficeduetoprogressivelowbackpainandstiffness.Thepainhasbeenpresentforthelastfewyearsandhasworsenedsomewhatinr