IgG4-related kidney disease - FUE |...

Seminars in Diagnostic Pathology (2012) 29, 245-250

IgG4-related kidney disease

Lynn D. Cornell, MD

From the Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester,
Minnesota.
IgG4-related kidney disease is a term that refers to any form of renal involvement by IgG4-relateddisease (IgG4-RD), a recently recognized systemic immune-mediated disease. The most common renalmanifestation is IgG4-related tubulointerstitial nephritis (IgG4-TIN), which presents as acute or chronicrenal insufficiency, renal mass lesions, or both. On biopsy, IgG4-TIN shows a plasma cell–richinterstitial inflammatory infiltrate with increased IgG4� plasma cells, along with expansile interstitialfibrosis; tubular basement membrane immune complex deposits are common. IgG4-TIN usually showsa brisk response to immunosuppressive therapy. Glomeruli may be affected by IgG4-RD, usually in theform of membranous glomerulonephritis. Other patterns of glomerular disease include IgA nephrop-athy, membranoproliferative glomerulonephritis, and endocapillary or mesangioproliferative immunecomplex glomerulonephritis. IgG4-related plasma cell arteritis has also been observed in the kidney.This review describes the histopathologic and immunophenotypic patterns of renal involvement byIgG4-RD, with associated clinical, radiographic, and serologic features.© 2012 Elsevier Inc. All rights reserved.

KEYWORDSInterstitial nephritis;Immune complex;IgG4-related disease;Membranousglomerulonephritis;Membranousnephropathy

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IgG4-related kidney disease (IgG4-RKD) is the termused to refer to any pattern of renal involvement by IgG4-related disease (IgG4-RD).1 As with other medical kidneydiseases, IgG4-RKD can be described in terms of changes tothe different “compartments” in the kidney: the tubules andinterstitium, the glomeruli, and the vessels. In the kidney,IgG4-RD manifests most commonly as tubulointerstitialnephritis (TIN), which may be mass forming and detectedon radiographic examination. Glomerular disease, in partic-ular membranous glomerulonephritis (MGN), may also beseen in IgG4-RD, with or without concurrent IgG4-relatedTIN (IgG4-TIN). A lesion of the arteries, IgG4-relatedplasma cell arteritis, has also been observed.2 The kidneymay also be affected by extrarenal manifestations of IgG4-RD, including ureteral inflammatory pseudotumor or retro-peritoneal fibrosis.3-5 This article will review the different

Address reprint requests and correspondence: Lynn D. Cornell,MD, Division of Anatomic Pathology, Department of Laboratory Medicineand Pathology, Mayo Clinic, Rochester, MN 55905.

E-mail address: [email protected].

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patterns of renal involvement by IgG4-RD, with associatedclinical, radiographic, and serologic features.

Clinical presenting features of IgG4-RKD

IgG4-TIN in the kidney may present as masses evident onradiographic studies, as acute or progressive chronic renalfailure, or both.6 Tissue samples of radiographic lesionseveal TIN.7 Patients with TIN may have mild proteinuria

and microscopic hematuria on urinalysis, and those withMGN usually present with heavy proteinuria or nephroticsyndrome.8 IgG4-related retroperitoneal fibrosis or ureteralinflammatory pseudotumor may lead to renal failure due toobstruction.3-5

IgG4-related tubulointerstitial disease

IgG4-TIN, the most commonly recognized pattern ofrenal involvement by IgG4-RD, is a specific type of im-
mune-mediated TIN that can be distinguished from other
ax Hospital - JCon June 07, 2016.right ©2016. Elsevier Inc. All rights reserved.

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246 Seminars in Diagnostic Pathology, Vol 29, No 4, November 2012

types of TIN by clinical, radiographic, laboratory, histo-logic, and immunophenotypic features.9 IgG4-TIN has beenbserved in IgG4-RD patients both with and without pan-reatic involvement; in some patients the disease is confinedo the kidney. A lesion similar to IgG4-TIN, chronic scle-osing pyelitis, an inflammatory mass that affects the renalelvis, has also been described.10 Raissian et al and Saeki etl have collected data on the two largest biopsy series ofgG4-TIN at 35 and 23 cases, respectively.6,11 Both of these

series showed many of the clinical and histologic featuresthat have been encountered in other organs affected byIgG4-RD, namely, radiographic tumoral lesions, plasmacell–rich inflammatory infiltrates with increased IgG4�plasma cells, elevated total IgG or IgG4 levels in the serum,presence of other organ involvement, and rapid response tosteroid therapy in most patients. Features specific to thekidney are detailed below.

Clinical features of IgG4-TINThe average age of patients with IgG4-TIN is 65 years;

most (�80%) are male. The Saeki et al study from Japanincluded only Japanese patients; the Raissian series fromNorth America included patients from other racial and eth-nic groups, although the majority of cases were Cauca-sian.6,11 Most patients (57%-76%) had acute or progressivechronic renal failure. In the remaining patients, the primaryindication for biopsy or nephrectomy was a renal masslesion. Many patients had both renal failure and kidneymass lesions. Other organs were involved by IgG4-RD in�80% of patients in the Raissian et al biopsy series, eitherconcurrently or before the recognition IgG4-TIN. The mostcommon extrarenal sites involved were the pancreas, liver,and salivary or lacrimal glands.

Laboratory features of IgG4-TINSimilar to autoimmune pancreatitis, almost 80% of

IgG4-TIN patients had elevated serum total IgG or IgG4levels.12 Not all of these patients had serum IgG subclassevels measured; of the subset that did, 92% had anlevated serum IgG4.6 Elevated serum IgG4 alone is notpecific for IgG4-RD, and the results of these serumtudies should be interpreted with caution.13 Other com-

mon laboratory features are decreased serum C3 and/orC4 levels (56%-78% of IgG4-TIN patients), peripheralblood eosinophilia (33%-48%), and low-titer positiveanti-nuclear antibody (�30%).6,11

Radiographic features of IgG4-TINRadiographic lesions of IgG4-TIN are best visualized on

contrast-enhanced computed tomography scan. The lesionsare commonly bilateral and multiple and predominantlyinvolve the renal cortex. Renal parenchymal lesions can beclassified as small peripheral cortical nodules, round orwedge-shaped lesions, diffuse patchy involvement, or alarge solitary mass.14 The radiographic differential diagno-is of renal parenchymal lesions includes lymphoma, vasc-
litis, pyelonephritis, and metastatic cancer. t

istologic, immunophenotypic, and ultrastructuraleatures of IgG4-TIN

By light microscopy, IgG4-TIN shows a plasma cell–richnterstitial inflammatory cell infiltrate. There is a spectrumf histologic appearances, ranging from acute TIN withinimal fibrosis, to an intermediate pattern with some in-

erstitial fibrosis but a marked inflammatory infiltrate, to aensely fibrotic paucicellular pattern with extensive tubularestruction and atrophy.6 The fibrosis is expansile, pushing

apart the tubules, and often has a “storiform” pattern as seenin other organs involved by IgG4-RD.15 Along with plasmacells and mononuclear cells, some tissue specimens shownumerous eosinophils and thus may be confused with aller-gic TIN due to a drug. Focal mild mononuclear cell tubulitisis seen in most cases, and eosinophilic or plasma celltubulitis may also be seen. In some cases, tubules aredestroyed and only fragments of tubular basement mem-branes (TBMs) can be appreciated on periodic acid-Schiff(PAS)- or silver-stained sections (Figure 1).

By immunofluorescence, �80% of cases show focal ordiffuse TBM immune complex deposits, which stain forIgG and kappa and lambda light chains, usually for C3,albeit lesser intensity, and in approximately 10% of cases,for C1q in approximately 10% of cases.6 TBM deposits arefound more frequently in specimens with interstitial fibro-sis. Deposits are found only in areas of the fibroinflamma-tory process and not in adjacent unaffected areas.6

Specimens with deposits seen by immunofluorescenceshow corresponding amorphous TBM electron-dense de-posits by electron microscopy.6 Glomeruli are negative byimmunofluorescence and electron microscopy unless thereis a concurrent immune complex glomerulonephritis.

Immunostaining for IgG4� plasma cells is helpful indistinguishing IgG4-TIN from other types of plasma cell–rich tubulointerstitial inflammatory infiltrates that couldmimic IgG4-TIN clinically and histologically.6 Using acutoff of focal moderate (11-30 IgG4� cells/40� field) tomarked (�30 IgG4� cells/40� field) increase in IgG4�plasma cells, the authors of one study found a sensitivity of100% (95% confidence interval: 0.9-1) and specificity of92% (95% confidence interval: 0.86-0.95) for distinguishingIgG4-TIN from other forms of TIN, after excluding cases ofinflammatory infiltrates in pauci-immune necrotizing andcrescentic glomerulonephritis.6

In kidney biopsies with interstitial inflammation relatedto pauci-immune glomerulonephritis, approximately 30% ofcases showed a focal moderate to marked increase inIgG4� plasma cells6; others have made this observationin the kidney as well.16 Similar findings have been noted inranulomatosis with polyangiitis (Wegener’s granulomato-is) affecting other organs.17 The absence of a serum anti-eutrophil cytoplasmic antibody (or anti-myeloperoxidaser -proteinase 3 antibodies) and necrotizing or crescenticlomerulonephritis on the tissue specimen helps to excludeauci-immune glomerulonephritis as a cause of the intersti-
ial inflammation. Focally increased IgG4� plasma cells

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247Cornell IgG4-Related Kidney Disease

were seen in a few other causes of interstitial inflammation,including chronic pyelonephritis; these other causes usuallycan be distinguished on clinical and histopathologicgrounds. Notably, nearly all cases of Sjögren syndrome-related TIN did not show increased IgG4� plasma cells.Clinicians and pathologists should keep in mind that IgG4plasma cell staining alone is not diagnostic of IgG4-RD.

Diagnosis of IgG4-TINRecent publications from Japan and North America have

proposed diagnostic criteria for IgG4-TIN.6,18 Both proposemultimodal approach to the diagnosis (Tables 1 and 2).

Table 1 Proposed diagnostic criteria for IgG4-TIN

Histology Plasma cell–rich TIN with �10 IgG4�plasma cells/hpf field in the mostconcentrated field*TBM immune complex deposits byimmunofluorescence,immunohistochemistry, and/or electronmicroscopy†

Imaging Small peripheral low-attenuation corticalnodules, round or wedge-shaped lesions,or diffuse patchy involvementDiffuse marked enlargement of kidneys

Serology Elevated serum IgG4 or total IgG levelOther organ

involvementCharacteristic findings of IgG4-RD inother organs

Raissian et al histologic criteria for IgG4-TIN.6

Diagnosis of IgG4-TIN requires the histologic feature of plasmacell–rich TIN with increased IgG4� plasma cells and at least one otherfeature from the imaging, serology, or other organ involvement cate-gories.

*Mandatory criterion.

Figure 1 IgG4-TIN in a young man who presented with acuultrasound examination. On biopsy, there is a marked interstitiamethenamine-silver). An IgG4 immunoperoxidase stain showimmunofluorescence, there is TBM and interstitial granular staideposition (right panel); a similar staining pattern was also see(Color version of figure is available online.)

†Supportive criterion, present in �80% of cases.


oth articles emphasize the need to exclude other diagnoseshat may show increased IgG4� plasma cells, in particularranulomatosis with polyangiitis, Churg–Strauss syndrome,nd plasma cell myeloma or lymphoproliferative disordersith plasmacytic differentiation.

al failure. The kidneys were markedly enlarged bilaterally onmatory cell infiltrate with expansile fibrosis (left panel; Jonesarked increase in IgG4� plasma cells (middle panel). By

or IgG, whereas a glomerulus is negative for immune complexIgG4 and for kappa and lambda light chains (data not shown).

Table 2 Japanese Society of Nephrology criteria for IgG4-RKD18

Clinicalfeatures

Clinical or laboratory evidence of kidneydamage, including abnormal renalfunction or abnormal urinalysis withelevated serum IgG or IgE level orhypocomplementemia

Imaging Abnormal radiographic findings:Multiple low-density lesions oncontrast-enhanced computedtomography scan, diffuse kidneyenlargement, hypovascular solitarykidney mass, hypertrophic lesion of therenal pelvic wall

Serology Elevated serum IgG4 or total IgG levelHistology a. Dense lymphoplasmacytic infiltrate

with �10 IgG4� plasma cells/hpf and/or IgG4/IgG� plasma cell ratio of�40%b. Characteristic storiform fibrosis

Other organinvolvement

Characteristic histologic findings ofIgG4-RD in other organs

“Definite” IgG4-RKD occurs with three of the following: clinicalfeatures, serology, and histologic features (a and b); imaging, serol-ogy, and histologic features (a and b); imaging, serology, or otherorgan involvement; or clinical features, serology, histologic features (aonly), and other organ involvement.

“Probable” and “possible” disease occurs with fewer criteria.

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IgG4-related glomerular disease

Glomerular diseases have been described in patients withIgG4-RD, mostly in the form of case reports or as a part ofIgG4-TIN case series. In a clinical series of Kawano et al,11 of 28 (39%) patients were reported to have some type ofglomerular lesion.18

IgG4-related MGNMGN is a glomerular disease pattern characterized by

subepithelial glomerular basement membrane (GBM) im-mune complex deposits, and may be a primary (“idio-pathic”) disease or secondary to a number of conditions,including autoimmune diseases, infections, medications,and neoplasms.19 MGN in the setting of IgG4-RD is re-ferred to as “IgG4-related MGN” (IgG4-MGN)1 although its recognized that primary MGN is also an IgG4-dominantisease.20,21

In the two largest series of IgG4-TIN, MGN was presentin approximately 7% of patients, and this form of glomer-ular disease has been noted in several case reports aswell.6,11,15,18,22-28 Patients with IgG4-MGN all presentedwith proteinuria, typically nephritic range. IgG4-MGN thusshould be suspected in IgG4-RD patients with significantproteinuria, and conversely, patients with MGN on renalbiopsy, and an appropriate clinical history should be eval-uated for IgG4-RD.8

Histopathologically, in IgG4-MGN, the glomeruli appearnormal or show thickened glomerular capillary loops onH&E-stained sections (Figure 2). GBM “spikes” can some-times be seen using silver or PAS stains, and subepithelialimmune complex deposits may be seen on a trichrome stain.One biopsy in our series showed segmental endocapillaryhypercellularity in addition to the MGN pattern.8 By immu-nofluorescence, glomeruli typically show segmental orglobal granular GBM bright staining for IgG, C3, and both

Figure 2 IgG4-MGN in an elderly woman with heavy proteinurJones methenamine-silver). Immunofluorescence shows global gridentified despite presence of concurrent IgG4-TIN in some areas
(arrows, right panel). (Color version of figure is available online.)

kappa and lambda light chains. Immunofluorescence stain-ing for IgG subclasses or immunoperoxidase staining forIgG4 revealed that the glomerular deposits contained IgG4.Immunostaining for the phospholipase A2 receptor (a find-ing typically seen in primary MGN) was negative in all 8biopsies stained, which argues that this is a secondaryMGN.8,29 In our series, 5 of 9 patients (56%) showedoncurrent IgG4-TIN on biopsy. Compared with IgG4-TIN,BM deposits were less common in IgG4-MGN, present in3% of cases.8

Other IgG4-related glomerular lesionsOther glomerular diseases have been variably reported in

IgG4-RD, including IgA nephropathy, Henoch-Schönlein pur-pura, and membranoproliferative glomerulonephritis.11,18,30-32

A few cases of endocapillary proliferative glomerulonephritis,sometimes with crescents, have been described.11,18,27,32 In theetting of IgG4-TIN, nephropathologists have occasionally ob-erved a pattern of mesangial proliferative glomerulonephritisith IgG-containing mesangial immune complex deposits,ithout a more specific diagnosis.7,11 Diabetic glomeruloscle-

osis may be identified in cases of autoimmune pancreatitisith pancreatic endocrine insufficiency. In addition, there haveeen three cases of minimal change disease among 116 IgG4-KD cases presented in regional meetings in Japan between004 and 2011 (Takako Saeki, personal communication).

IgG4-related vascular disease

Plasma cell–rich renal arteritis has recently been de-scribed in a patient with IgG4-TIN.2 This lesion affectedsmall and medium-sized arteries on a biopsy, with markedintimal, medial, and adventitial inflammation by plasmacells and lymphocytes (Figure 3). Many IgG4� plasmacells were present in the arterial wall. Neither fibrinoid

g/d. By light microscopy, the glomeruli appear normal (left panel;GBM staining for IgG (middle panel). No TBM deposits wereon microscopy shows small subepithelial electron-dense deposits

ia at 4anular

. Electr


249Cornell IgG4-Related Kidney Disease

necrosis of the arteries nor rupture of the elastica waspresent, and neutrophils were not identified in the lesions.

Veins are usually not present in kidney needle corebiopsies but can be seen in nephrectomy specimens. Venu-litis, similar to that seen in other organs affected by IgG4-RD, can sometimes be seen in IgG4-TIN, but is not neces-sary for diagnosis.7

IgG4-RKD and response to therapy

Similar to other organ manifestations of IgG4-RD, IgG4-TIN also usually shows a swift response to steroid therapy.In both major series of IgG4-TIN, 90% of patients withelevated serum creatinine at presentation who were treatedwith steroids showed decreased creatinine at follow-up.Although TIN of any cause may respond to steroid therapy,IgG4-TIN tends to show a response even in cases withsevere interstitial fibrosis on the biopsy sample. IgG4-TINmay relapse after treatment, similar to other organ manifes-tations of IgG4-RD.33,34 A small case series described aresponse to rituximab in IgG4-RD patients refractory tosteroid treatment.35 We have also encountered one steroid-dependent patient with IgG4-TIN who showed a response torituximab (Cornell LD, unpublished data). Long-term fol-low-up data are needed to assess the impact of immunosup-pressive therapy on IgG4-TIN.

In a series of IgG4-MGN patients, six patients weretreated with various immunosuppressive drugs. All six pa-tients showed decreased proteinuria and most showed de-creased serum creatinine at an average of 39-month fol-low-up (range: 4-184 months). Although IgG4-TIN shows a

Figure 3 IgG4-related renal arteritis in an elderly man withacute on chronic renal failure. A representative artery shows severearteritis with numerous plasma cells in the intima, media, andadventitia, without fibrinoid necrosis (hematoxylin and eosin). Animmunoperoxidase stain for IgG4 showed numerous IgG4�plasma cells in the arterial lesion. Image courtesy of Dr ShreeSharma and Dr Vivette D’Agati, Columbia University. (Colorversion of figure is available online.)

swift response to therapy, we hypothesize that IgG4-MGN


has a different pathogenic mechanism and thus would not beexpected to show the same rapid treatment response withrespect to proteinuria.

Conclusions

The kidney can be affected by IgG4-RD in a variety ofpatterns. IgG4-TIN, the most common renal manifestationof IgG4-RD, is a plasma cell–rich immune-mediated dis-ease that may present as renal failure, renal mass lesions, orboth. IgG4-TIN may be distinguished from other forms ofTIN by histologic, immunophenotypic, clinical, serologic,and radiographic features. Glomerular disease may also beseen in IgG4-RD, most commonly with a pattern of MGN,although the glomerular disease may not be accompanied byIgG4-TIN. IgG4� plasma cell arteritis has recently beendescribed in the kidney. IgG4-TIN typically shows a swiftresponse to steroid therapy; refractory patients may respondto rituximab.

References

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