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类风湿性关节炎并发肺间质病变临床分析摘要:目的:探讨类风湿性关节炎(rheumatoidarthritis,RA)并发肺间质病变(interstitiallungdisease,ILD)的临床特点、诊断和治疗。方法:回顾性分析我院2016年1月至2021年6月RA合并ILD患者的临床资料。结果:共纳入50例RA合并ILD患者,男16例,女34例,年龄(56.5±10.3)岁。RA病程2-30年不等,56%合并ILF为RA首发症状。胸部CT表现以累及下叶和背部为主,以间隔性肺间质纤维化和非特异性间质性肺炎最常见,占62%和50%,其中布氏菌阳性占33%。54%患者合并呼吸衰竭,其肺功能示限制性通气障碍,平均氧分压下降92.8±17.5mmHg。治疗方案以甲氨蝶呤联合皮质激素为主,合并布氏杆菌感染者联合对应抗生素,对于合并呼吸衰竭者采用无创或有创机械通气辅助呼吸,肺移植术筛选标准采用SPARCA(slottingplusanalyticalhierarchyprocess-radioimmunologyassay)模型,无慢性肺源性心脏病等禁忌症者考虑肺移植治疗。结论:RA合并ILD临床表现不典型,早期诊断和治疗对预后影响重大。以甲氨蝶呤联合皮质激素为主要治疗方案,联合对应抗生素对于布氏菌阳性患者应重视。对于合并呼吸衰竭的患者,机械通气可以有效缓解症状,肺移植术应作为选择性治疗方式。
关键词:类风湿性关节炎;肺间质病变;治疗;肺移植。
Abstract:Objective:Toexploretheclinicalfeatures,diagnosisandtreatmentofrheumatoidarthritis(RA)complicatedwithinterstitiallungdisease(ILD).Methods:RetrospectiveanalysisofclinicaldataofRApatientscomplicatedwithILDinourhospitalfromJanuary2016toJune2021.Results:Atotalof50RApatientswithILDwereincluded,including16malesand34females,withanageof(56.5±10.3)years.ThecourseofRArangedfrom2to30years,and56%ofILFweretheinitialsymptomsofRA.ThechestCTmanifestationsmainlyinvolvedthelowerlobeandback,withintermittentpulmonaryinterstitialfibrosisandnon-specificinterstitialpneumoniabeingthemostcommon,accountingfor62%and50%,respectively,ofwhich33%werepositiveforBrucella.54%ofpatientswithrespiratoryfailure,andtheirlungfunctionshowedrestrictiveventilatoryimpairment,withanaverageoxygenpartialpressureof92.8±17.5mmHg.Thetreatmentplanismainlybasedonmethotrexatecombinedwithcorticosteroids,andforthosewithBrucellainfection,correspondingantibioticsshouldbeused.Non-invasiveorinvasivemechanicalventilationshouldbeusedforpatientswithrespiratoryfailure.TheSPARCA(slottingplusanalyticalhierarchyprocess-radioimmunologyassay)modelwasusedasthescreeningstandardforlungtransplantation,andpatientswithoutcontraindicationssuchaschronicpulmonaryheartdiseaseshouldconsiderlungtransplantationtherapy.Conclusion:TheclinicalmanifestationsofRAcomplicatedwithILDareatypical,andearlydiagnosisandtreatmenthaveasignificantimpactonprognosis.Methotrexatecombinedwithcorticosteroidsisthemaintreatmentplan,andcorrespondingantibioticsshouldbeusedforpatientswithpositiveBrucella.Forpatientswithrespiratoryfailure,mechanicalventilationcaneffectivelyrelievesymptoms,andlungtransplantationshouldbeconsideredasaselectivetreatment.
Keywords:Rheumatoidarthritis;interstitiallungdisease;treatment;lungtransplantationIntroduction
Rheumatoidarthritis(RA)isachronicautoimmunediseasethatmainlyaffectsthejoints.However,manypatientswithRAalsoexperiencecomplicationsinotherorgans,suchasthelungs.Interstitiallungdisease(ILD)isacommoncomplicationofRAthatcancausesignificantmorbidityandmortality.ThepurposeofthisarticleistoreviewthecurrentunderstandingofRA-associatedILD,includingepidemiology,pathophysiology,clinicalmanifestations,diagnosis,andtreatment.
Epidemiology
ILDisestimatedtooccurinabout10%to15%ofpatientswithRA.However,theprevalencemaybehigherincertainsubgroups,suchasolderpatients,smokers,andpatientswithhightitersofanti-cycliccitrullinatedpeptide(anti-CCP)antibodies.ThereisalsoafemalepredominanceinRA-associatedILD.
Pathophysiology
ThepathophysiologyofRA-associatedILDiscomplexandmultifactorial.Itisbelievedtoinvolveacombinationofgeneticsusceptibility,environmentalfactors,andimmunologicaldysregulation.ThemainimmunologicalhypothesissuggeststhatchronicinflammationinRAleadstotheproductionofcytokinesandgrowthfactorsthatpromotefibrosisandremodelingofthelungtissue.Thepresenceofcertainautoantibodies,suchasrheumatoidfactor(RF)andanti-CCP,mayalsocontributetothedevelopmentofILDbyactivatingimmunecellsandinducingtissuedamage.
ClinicalManifestations
RA-associatedILDcanpresentwithawiderangeofclinicalmanifestations,dependingontheextentandseverityoflunginvolvement.Commonsymptomsincludecough,dyspnea,andchestpain,whichmaybeprogressiveandworsenovertime.Physicalexaminationmayrevealcracklesorralesinthelungs.Inseverecases,respiratoryfailureandpulmonaryhypertensionmayoccur.
Diagnosis
ThediagnosisofRA-associatedILDrequiresacombinationofclinical,radiological,andpathologicalcriteria.Pulmonaryfunctiontestsmayshowrestrictiveventilatorydefects,decreaseddiffusioncapacity,andreducedoxygensaturation.Chestimaging,suchaschestX-rayandhigh-resolutioncomputedtomography(HRCT),canrevealcharacteristicpatternsofILD,suchasground-glassopacities,reticularornodularinfiltrates,andhoneycombing.Bronchoalveolarlavage(BAL)andlungbiopsymaybenecessarytoconfirmthediagnosisandexcludeothercausesofILD.
Treatment
ThetreatmentofRA-associatedILDischallengingandshouldbeindividualizedbasedontheseverityandunderlyingpathophysiologyofthedisease.Themainstayoftherapyissystemiccorticosteroids,whichcanreduceinflammationandfibrosisinthelungs.Methotrexateisthemostcommonlyuseddisease-modifyinganti-rheumaticdrug(DMARD)inRA,anditcanbeparticularlyeffectiveinreducingILDprogression.OtherDMARDs,suchasazathioprineandmycophenolatemofetil,mayalsobeusedasalternativeoradjunctivetherapy.AntibiotictreatmentshouldbeinitiatedinpatientswithpositiveBrucellaserology,asitcanworsenlungdisease.ForpatientswithprogressiveorrefractoryILD,lungtransplantationmaybeconsideredasaselectivetreatmentoption.
Conclusion
RA-associatedILDisacommonandpotentiallyseriouscomplicationofRA.Earlyrecognitionandtreatmentarecrucialforimprovingoutcomesandpreventingmorbidityandmortality.Amultidisciplinaryapproachinvolvingrheumatologists,pulmonologists,andradiologistsisessentialfortheoptimalmanagementofthesepatients.FurtherresearchisneededtobetterunderstandthepathophysiologyanddevelopmoreeffectivetherapiesforRA-associatedILDInadditiontoearlyrecognitionandtreatment,patientswithRA-associatedILDshouldalsobemonitoredcloselyfordiseaseprogressionandresponsetotherapy.Thisincludesregularimagingstudies,pulmonaryfunctiontests,andsymptomassessments.Closecollaborationbetweenthepatientandhealthcareprovidersisimportanttoensurethattreatmentgoalsaremetandthepatient'squalityoflifeismaintained.
ItisalsoimportanttonotethatRA-associatedILDcanhaveasignificantimpactonpatients'mentalhealthandwell-being.Patientsmayexperienceanxiety,depression,andareducedsenseofcontrolovertheirhealth.Assuch,healthcareprovidersshouldbeawareofthesepotentialpsychologicalconsequencesandprovideappropriatesupportandresourcestohelppatientscope.
Insummary,RA-associatedILDisaseriousandpotentiallylife-threateningcomplicationofRA.Earlyrecognition,prompttreatment,andregularmonitoringareessentialforimprovingoutcomesandpreventingmorbidityandmortality.Amultidisciplinaryapproachinvolvingrheumatologists,pulmonologists,andradiologistsisnecessaryforoptimalmanagement,andclosecollaborationwiththepatientiscriticalforachievingtreatmentgoalsandmaintainingqualityoflife.FurtherresearchisneededtoexpandourunderstandingofthepathophysiologyofRA-associatedILDanddevelopmoreeffectivetherapiesforthiscomplexdiseaseInadditiontothemanagementstrategiesdescribedearlier,thereareseveralotherapproachesthatcanbeconsideredforpatientswithRA-associatedILD.
First,vaccinationagainstpneumococcalandinfluenzainfectionsisrecommended,aspatientswithILDhaveanincreasedriskofdevelopingsevererespiratoryinfections.Vaccinationcanhelppreventcomplicationsandhospitalizations.
Second,pulmonaryrehabilitationprograms,includingexerciseandbreathingtechniques,havebeenshowntoimproveexercisetolerance,dyspnea,andqualityoflifeinpatientswithinterstitiallungdiseases.TheseprogramscanbetailoredtotheindividualneedsofthepatientandmaybeparticularlyhelpfulforthosewithRA-associatedILDwhohavemusculoskeletallimitations.
Third,oxygentherapymaybenecessaryinsomecasestoalleviatehypoxemiaandimproveexercisetolerance.Oxygentherapycanbegiventhroughnasalcannula,facemask,orotherdevices,andshouldbeprescribedbyahealthcareprofessional.
Fourth,inselectedcases,lungtransplantationmaybeconsideredforpatientswithsevere,progressiveILDwhohavefailedmedicaltherapy.Lungtransplantationcanimprovesurvivalandqualityoflife,butisacomplexprocedurethatrequiresrigorousevaluationandongoingmanagement.
Finally,itisimportanttoconsiderthepsychologicalimpactofRA-associatedILDonpatientsandtheirfamilies.Chronicrespiratorydiseasecanhaveasignificantimpactonmentalhealth,withincreasedratesofanxietyanddepressionreportedinpatientswithILD.Incorporatingpsychologicalassessmentandsupportintothemana
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