IgG4-Related Kidney Disease PDF
Preview IgG4-Related Kidney Disease
IgG4-Related Kidney Disease Takao Saito John H. Stone Hitoshi Nakashima Takako Saeki Mitsuhiro Kawano Editors 123 IgG4-Related Kidney Disease Takao Saito (cid:129) John H. Stone (cid:129) Hitoshi Nakashima (cid:129) Takako Saeki (cid:129) Mitsuhiro Kawano Editors IgG4-Related Kidney Disease Editors TakaoSaito JohnH.Stone FacultyofMedicine RheumatologyClinic/Yawkey2 FukuokaUniversity MassachusettsGeneralHospital Fukuoka Boston,Massachusetts Japan USA HitoshiNakashima TakakoSaeki DepartmentofInternalMedicine DepartmentofInternalMedicine FacultyofMedicine NagaokaRedCrossHospital FukuokaUniversity Nagaoka Fukuoka Japan Japan MitsuhiroKawano DivisionofRheumatology DepartmentofInternalMedicine KanazawaUniversityHospital Kanazawa Japan ISBN978-4-431-55686-2 ISBN978-4-431-55687-9 (eBook) DOI10.1007/978-4-431-55687-9 LibraryofCongressControlNumber:2016956873 ©SpringerJapan2016 Thisworkissubjecttocopyright.AllrightsarereservedbythePublisher,whetherthewholeorpartof the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilarmethodologynowknownorhereafterdeveloped. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publicationdoesnotimply,evenintheabsenceofaspecificstatement,thatsuchnamesareexempt fromtherelevantprotectivelawsandregulationsandthereforefreeforgeneraluse. Thepublisher,theauthorsandtheeditorsaresafetoassumethattheadviceandinformationinthis book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained hereinorforanyerrorsoromissionsthatmayhavebeenmade. Printedonacid-freepaper ThisSpringerimprintispublishedbySpringerNature TheregisteredcompanyisSpringerJapanKK Preface Thelatenineteenthcenturywasagoldenageforthedescriptiononnewinflamma- tory diseases. Both Sj€ogren’s syndrome (SjS) and Mikulicz disease (MD), the representative immune-mediated diseases of the lacrimal and salivary glands, were described first in 1888. SjS was later described in detail by Henrik Sj€ogren in 1933, and ever since has borne his name. Beginning in the 1950s, MD was erroneouslybelievedtocompriseassubsetofSjS.However,immunologicalstudies commencing in the 1960s slowly untangled the differences between SjS andMD, whichbythebeginningofthiscenturyhadbecomeincontrovertible. ThecategorizationofIgGintofoursubclassesmarkedanimportantstepinthis process. Subsequently, a peculiar increase of IgG4, normally the least abundant subclass,wasrecognizedinseraofpatientswithautoimmunepancreatitisandMD, but not in sera from patients with SjS. Moreover, serum IgG4 elevation was reported in various organ manifestations showing lymphoplasmatic infiltration and a specific form of fibrosis termed “storiform” by pathologists. Following many important case reports and series describing a consistent histopathology acrossmanyorgansandthetypicalfindingofincreasednumbersofIgG4-positive plasma cells, multiple types of inflammatory processes once believe confined to individual organs were recognized as comprising a single disease entity. Wide- spreadrecognitionof“IgG4-relateddisease(IgG4-RD)”asaunifieddiseaseentity was achieved at the First International Symposium on IgG4-Related Disease in Bostonin2011. Renal manifestations have a prominent role in IgG4-RD, as they do in other systemic conditions such as systemic lupus erythematosus and ANCA-associated vasculitis. Renal involvement by IgG4-RD has now been termed “IgG4-related kidneydisease”(IgG4-RKD)byinternationalconsensus.Tubulointerstitialnephri- tis(TIN),themostcommonIgG4-RKDlesionofIgG4-RKD,hasseveralhistologic characteristics that are not found in TIN associated with other immune-mediated conditions,e.g.,SjS. Regions of involvement of the tubulointerstitium tend to be well-demarcated from non-involved areas. The affected regions are occupied by an intense lymphoplasmacyticinfiltrate,typicallyincludingapreponderanceofIgG4-positive v vi Preface plasmacells,and“storiform”or“bird’seye”fibrosiswithminimalinvolvementof the tubulesthemselves. Distinctiveimagingfindingsoncontrast-enhancedCT are often instrumental in the identification of IgG4-related TIN, because the clinical testsmostoftenemployedtodetectkidneydiseasearemoresensitiveforglomer- ular lesions. However, a diverse array of renal manifestations including glomerulopathies, have also been recognized to occur in association with IgG4- RD.ThemajorotherformsofIgG4-RKDconsistofmembranousnephropathyand tumor-likelesionsmimickingurologicdiseasesthatextendtothepelvisandurinary tract.Toinvestigatethesecomplicatedconditionsindepthandestablishthedisease entity,aworkinggroupwasorganizedintheJapaneseSocietyofNephrologyanda set of diagnostic criteria for IgG-RKD was proposed in 2011, simultaneous with anotherproposalbyagroupinNorthAmerica. In this book, leading researchers in the field present the latest insights into the broad spectrum of IgG4-RKD. In addition, they provide detailed explanations of variousaspectsofIgG4-RKDincludinghistopathologicalcomparisonsbetweenthe kidney and other affected organs, such as the lacrimal, salivary glands and pan- creas. IgG4-RKD is already having a major impact on immunological and nephrological studies and offers a useful resource not only for nephrologists but alsogeneralphysiciansandinvestigatorsinrelatedfields. Fukuoka,Japan TakaoSaito Boston,MA,USA JohnH.Stone Fukuoka,Japan HitoshiNakashima Nagaoka,Japan TakakoSaeki Kanazawa,Japan MitsuhiroKawano Acknowledgements Japaneseeditors–T.S,H.N,T.S.andM.K-thanktheeditorintheUnitedStates–J. H.S-forhiscarefuleditingtoallmanuscripts.WearealsoverygratefultoMr.John Gelblumforhishelpwiththetranslationandotheraspectsofthisbook.Wewould like to appreciate the supports of the Japanese Society of Nephrology and the Research Projects for Study of Intractable Diseases provided by the Ministry of Health,LaborandWelfareofJapan. vii Contents PartI GeneralRemarks 1 IgG4-RelatedDisease:HistoryandOverview. . . . . . . . . . . . . . . . . 3 ShigeyukiKawa 2 IgG4-RelatedDisease:PathologicalFeaturesandDifferential Diagnosis. . . . . . .. . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . .. . . . 25 YohZen 3 PotentialPathwaysinthePathogenesisofIgG4-RelatedDisease. . . 43 MasafumiMoriyamaandSeijiNakamura 4 DoesIgG4-RelatedDiseaseHaveanAutoimmuneBasis?. . . . . . . . 55 TakakoSaekiandMitsuhiroKawano 5 Plasmablasts:APromisingBiomarkerinIgG4-RelatedDisease. . . 65 MarcoLanzillotta,JohnH.Stone,andEmanuelDella-Torre PartII FeaturesofIgG4-RelatedKidneyDiseaseandAssociated Conditions 6 OverviewofIgG4-RelatedKidneyDisease. . . . . . . . . . . . . . . . . . . 75 TakaoSaito 7 IgG4-RelatedKidneyDisease:ClinicalandLaboratoryFeatures. . . 85 ShinichiNishiandTakakoSaeki 8 ImagingFindings. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 DaiInoue,YohZen,MitsuhiroKawano,OsamuMatsui, andToshifumiGabata 9 CharacteristicTubulointerstitialNephritisinIgG4-RelatedKidney Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105 MichioNagataandSatoshiHara ix x Contents 10 ElectronMicroscopicFindings. . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 NobuakiYamanaka 11 IgG4-RelatedKidneyDisease:PathologicalFeatures:Membranous Glomerulonephritis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125 MariamPriyaAlexanderandLynnD.Cornell 12 IgG4-RelatedKidneyDisease:PathologicalFeatures:Other GlomerularDisease. . . . . . .. . . . . . . . .. . . . . . . . .. . . . . . . . .. . . 139 SatoshiHisano 13 IgG4-RelatedKidneyDiseasesandConditions:RenalPelvicand UreteralDiseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145 KenichiHaradaandYoshifumiUbara 14 RetroperitonealFibrosis/PeriaortitisandHydronephrosis. . . . . . . 159 IchiroMizushima,DaiInoue,andMitsuhiroKawano 15 OtherUrogenitalLesions(Testis,ParatesticularLesion,Bladder, Prostateetc.). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173 TakeshiUehara,YasunariFujinaga,TeruyukiOgawa, andShigeyukiKawa 16 CharacteristicDistributionofInflammatoryLesionsinIgG4- RelatedKidneyDisease:FindingsfromAutopsyCaseSeries. . . . . 187 SatoshiHara,MitsuhiroKawano,IchiroMizushima,KenichiHarada, TakumaTakata,TakakoSaeki,YoshifumiUbara,YasuharuSato, andMichioNagata 17 DiagnosisofIgG4-RelatedKidneyDisease. . . . . . . . . . . . . . . . . . . 193 MitsuhiroKawanoandKazunoriYamada 18 PathogenesisofIgG4-RelatedKidneyDisease. . . . . . . . . . . . . . . . . 203 HitoshiNakashima PartIII TreatmentofIgG4-RelatedDiseaseandIgG4-Related KidneyDisease 19 CorticosteroidTherapyforIgG4-RelatedKidneyDisease. . . . . . . 219 TakakoSaeki 20 TargetingtheBCellLineageinIgG4-RelatedDisease. . . . . . . . . . 229 JohnH.Stone PartIV DifferentialDiagnosisofIgG4-RelatedKidneyDisease 21 DifferentialDiagnosisofIgG4-RelatedTubulointerstitialNephritis: AnOverview. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237 YutakaYamaguchi,SatoshiHara,andMitsuhiroKawano Contents xi 22 DifferentialDiagnosis(1):ANCAAssociatedVasculitis. . . . . . . . . 251 MitsuhiroKawano,SatoshiHara,andYoshifumiUbara 23 DifferentialDiagnosis(2):CastlemanDisease. . . . . . . . . . . . . . . . . 261 TakeshiZoshima,MitsuhiroKawano,SatoshiHara,andMichioNagata 24 DifferentialDiagnosis(3):Sj€ogrenSyndrome. . . . . . . . . . . . . . . . . 271 TakaoSaito 25 DifferentialDiagnosis:LupusNephritisandIgG4-RelatedKidney Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 ShinichiNishi,ShigeoHara,andNaofumiImai PartV LessonsfromCases 26 MulticentricCastleman’sDiseaseMimickingIgG4-RelatedDisease: ACaseReport. .. . . . . .. . . . . . .. . . . . .. . . . . .. . . . . .. . . . . . .. 293 EikoHasegawa,AkinariSekine,Jun-ichiInenaga,TakeshiFujii, KenichiOhashi,YasuharuSato,andYoshifumiUbara 27 ACaseofConcurrentIgG4-RelatedKidneyDiseaseandLupus Nephritis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 YokoWada,KojiMatsuo,YumiIto,NaofumiImai,MasaakiNakano, TakakoSaeki,andIchieiNarita
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