IgG4-Related Kidney Disease

298 Pages·2016·13.332 MB·English

by  Takao Saito, John H. Stone, Hitoshi Nakashima, Takako Saeki, Mitsuhiro Kawano (eds.)

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
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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