IgG4相关性疾病(IgG4-relateddisease)临床病理特征

2025/07/23PathologicalandClinicalFeaturesofIgG4-AssociatedDisease(IgG4-RD)Reporter:CONTENTSCatalogue01OverviewofIgG4-RD02ClinicalManifestationsofIgG4-RD03PathologicalFeaturesofIgG4-RD04DiagnosticMethodsforIgG4-RDOverviewofIgG4-RD01DefinitionandEtiologyUnderstandingIgG4-RDIgG4相关性疾病是一种以血清IgG4水平升高和组织内IgG4阳性浆细胞浸润为特征的慢性炎症性疾病。ComplexEtiologyThepreciseoriginsofIgG4-relateddiseaseremainelusive,yetit'sthoughttostemfromamixofgeneticsusceptibilityandexternalinfluences.DefinitionandEtiologyAutoimmuneComponentIgG4相关性疾病被认为具有自身免疫性质，免疫系统错误攻击正常组织，从而导致纤维化和器官功能障碍。CommonAffectedOrgansThePancreasandBiliaryTractIgG4相关性疾病通常侵袭胰腺和胆管系统，引发自身免疫性胰腺炎和硬化性胆管炎等状况。SalivaryandLacrimalGlandsThesalivaryglands,particularlythesubmandibularglands,andthelacrimalglandsarefrequentlyinvolved,causingxerostomiaanddryeyes.CommonAffectedOrgansLymphNodesandSoftTissuesIgG4相关性疾病还可能侵害淋巴结和软组织，导致身体各部位的淋巴结肿大和纤维化病变。KidneysandAortaTheaortaandkidneysmaysometimesbeimpacted,possiblyresultingintubulointerstitialnephritisandaortitis,respectively.ManifestationsCliniquesdelaPathologieIgG4-RD02OrganSwellingandPainLymphNodeEnlargementIgG4相关性疾病常导致无疼痛的淋巴结肿大，这可能是疾病存在的迹象。SalivaryGlandInvolvementinConditionsSwellingandpaininthesalivaryglandsarecommonsymptoms,oftenleadingtoadiagnosisofIgG4-RD.OrganSwellingandPainPancreaticSwellingThepancreasmightswellandfeelsore,aphenomenonreferredtoasautoimmunepancreatitis,ahallmarkofIgG4-relateddisease.OrganSwellingandPainTubulointerstitialRenalInflammationIgG4相关性疾病可能损害肾脏，引发肿胀和疼痛，若未得到妥善治疗，可能导致肾功能受损。FunctionalImpairmentandAssociatedSymptomsSwellingofOrgansIgG4相关性疾病常导致受累器官，如胰腺或唾液腺，出现肿胀，进而引发功能障碍和不适。LymphNodeEnlargementPatientsmayexperienceswollenlymphnodes,whichcanbeasignofIgG4-RDandcontributetothedisease'sclinicalmanifestations.SymptomsandObstructionsinFunctionalitySystemicSymptomsSymptomssuchasexhaustion,adecreaseinbodyweight,andanelevatedtemperaturemayaccompanyIgG4-relateddisease,indicativeofitssystemiccharacteristics.TissueFibrosisProgressivefibrosisinaffectedtissuescanleadtoorgandysfunctionandisasignificantclinicalfeatureofIgG4-RD.PathologicCharacteristicsinIgG4-RelatedDisease03IgG4-PositivePlasmaCellInfiltrationHistopathologicalCharacteristicsIgG4相关性疾病以密集的淋巴浆细胞浸润为特征，其中包含IgG4阳性的浆细胞，通常伴随纤维化和闭塞性静脉炎。ImmunohistochemicalExaminationUseimmunohistochemistrytodetectelevatednumbersofIgG4-positiveplasmacellswithinaffectedtissues,whichisakeydiagnosticcriterion.IgG4-PositivePlasmaCellInfiltrationClinicalCorrelationAssociatethedegreeofIgG4-positiveplasmacellinfiltrationwiththeclinicalsymptomsinordertograsptheprogressionandintensityoftheillness.TreatmentImplicationsExaminetheimpactofIgG4-positiveplasmacellsontherapeuticchoicesandthelikelihoodofatherapeuticoutcomeinindividualssufferingfromIgG4-relateddisease.FibrosisandScarringSwellingofOrgansIgG4相关性疾病通常在受影响的器官，如胰腺或唾液腺，引起肿胀，进而导致功能阻塞。LymphNodeEnlargementPatientsmightencounterenlargedlymphnodes,potentiallyleadingtopressureonnearbytissuesandresultinginuneaseorpain.FibrosisandScarringSystemicSymptomsSymptomssuchasexhaustion,decreasedbodyweight,andelevatedtemperaturesmightarise,suggestingabodilyimmunereactiontoIgG4-RD.MethodsofDiagnosingIgG4-RelatedDisease04SerumIgG4LevelTestingUnderstandingIgG4-RDIgG4相关性疾病是一种慢性炎症性病症，其特征为血清中IgG4水平升高以及由IgG4阳性浆细胞引起的组织浸润。ComplexEtiologyThepreciseoriginofIgG4-RDremainsunspecified;however,it'sthoughttoresultfromamixofhereditaryinclinationsandexternalinfluences.SerumIgG4LevelTestingAutoimmuneComponentIgG4相关性疾病被认定为一种自身免疫性疾病，其特征为免疫系统错误地攻击正常组织，进而引发纤维化和器官功能障碍。ImagingandPathologicalExaminationHistologicalFeaturesIgG4-RDischaracterizedbydenselymphoplasmacyticinfiltration,withapredominanceofIgG4-positiveplasmacells.FibrosisandStoriformPatternTheillnessfrequentlymanifestsasstoriformfibrosis,whichisaunique,swirlingarrangementoffibrosiswithinthedamagedtissues.Imaging&PathologicalStudiesTissueEosinophiliaEosinophilicaccumula