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the Genitourinary System Spectrum of Imaging Findings and ClinicalPathologic Features Department of Radiology 1 SEOUL ST MARYS HOSPITAL and 2 Eunpyeong ST Marys Hospital college of medicine ID: 931601

renal igg4 arrows kidneys igg4 renal kidneys arrows mass year image shows prostate ureter lesions contrast nodular axial multiple

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Slide1

IgG4-related Disease of the Genitourinary System: Spectrum of Imaging Findings and Clinical-Pathologic Features

Department of Radiology 1SEOUL ST MARY’S HOSPITAL and 2Eunpyeong ST Mary’s Hospital, college of medicine,The Catholic university of Korea

Ji Woon Oh, MD1; Sung Eun Rha, MD, PhD1; Moon Hyung Choi, MD, PhD2; Soon Nam Oh, MD, PhD1; Seo Yeon Youn, MD, PhD1; Joon Il Choi, MD, PhD1

Slide2

Introduction

Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic

infiltrates rich in IgG4+ plasma cells.IgG4-RD is characterized histologically by extensive infiltration of IgG4-positive plasma cells and lymphocytes and fibrosis of the involved organ, usually associated with an elevated serum level of IgG4.IgG4-RD was initially characterized as a form of autoimmune pancreatitis, but IgG4-RD involvement has now been described in almost every organ system, including the genitourinary system.

IgG4-RD involving the genitourinary system may show variable imaging findings that can mimic other benign and malignant diseases.

Slide3

Introduction

Epidemiology Average age of disease onset: 50–70 yearsIncidence according to sex: male

> female (5:1 to 1.6:1)Involving organs All organs throughout the body can be candidates for IgG4-RD Incidence of involved organs : pancreas > biliary tree > salivary gland > orbit > retroperitoneum > kidneyIgG4-RD can involve one or multiple sites in the body. For

multiorgan involvement, multiple sites can be affected simultaneously or sequentially.

Slide4

IgG4-RD

Retroperitoneum and mesentery

Hepatobiliary system

Thorax

Salivary, lacrimal, and thyroid glands

Central Nervous System and Orbits

IgG4-RD of the lungs, pleuritis, or pericarditis

Riedel thyroiditis

Sclerosing

sialadenitis and dacryoadenitis

Hypertrophic pachymeningitis

Hypophysitis

Orbital inflammatory pseudotumor

Fibrosing mediastinitis

IgG4-RD of the kidneys

IgG4-RD of the ureter

IgG4-RD of the prostate

Retroperitoneal fibrosis

Periaortitis and inflammatory aortic aneurysm

Genitourinary system

Sclerosing mesenteritis

Type I autoimmune pancreatitis

IgG4-RD hepatopathy

Sclerosing

cholangitis

Slide5

Introduction: Histologic Features of IgG4-RD

A dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cellsFibrosis in a storiform patternObliterative phlebitis: the venous channels are obliterated by a dense

lymphoplamacytic infiltrate in both the venous wall and the lumen

* IgG

* IgG4

* Lymphocyte infiltration with

storiform

fibrosis

*

Obliterative

phlebitis

Slide6

Introduction:

Diagnosis and TreatmentThe diagnosis of IgG4-RD is based on a combination of clinical, serologic, radiologic, and characteristic histologic findingsSerum IgG or IgG4

Variable from normal to marked elevation in IgG4-RDNeither sensitive nor specific for diagnosisHigh serum IgG4 concentration (>135 mg/dL) is commonly seen in patients, but a serum IgG4 level may be normal in 20%–40 % of cases of biopsy-proven IgG4-RDThe elevated serum IgG4 level is not pathognomonic for the diagnosis of IgG4-RDTreatmentGlucocorticoids are effective and rapid for IgG4-RD Immunosuppressants such as methotrexate, azathioprine, or mycophenolate mofetil as alternatives or maintenance treatment

Slide7

IgG4-RD of the Genitourinary System

Ⅰ. Kidneys Ⅱ. UreterⅢ. BladderⅣ. Prostate glandⅤ. Testes

Slide8

. IgG4-RD of the KidneysIgG4-RD of the kidneys is the

most common among IgG4-RD involvement of genitourinary organsIgG4-RD of the kidneys manifests pathologically asTubulointerstitial nephritisMembranous glomerulonephritisObstructive nephropathy caused by postrenal obstruction

Clinical symptoms Usually mildly symptomatic or even asymptomatic

Hematuria and elevated serum creatinine level as acute renal injuryObstructive nephropathy

Slide9

. IgG4-RD of the KidneysRenal parenchymal involvement - Multiple nodular lesions: the most common pattern - Diffuse patchy infiltrative lesions

- Single nodular lesionRenal pelvic and perinephric involvement- Diffuse wall thickening of the renal pelvis- A rim of soft tissue around the kidney

Slide10

IgG4-RD of the Kidneys

– Multiple Nodular Type

A 78-year-old woman with IgG4-RD involving the kidneys (tubulointerstitial nephritis). Her serum IgG4 level was 69.2 mg/dL (0.692 g/L), with a reference range of 3.9–86.4 (0.039–0.864 g/L). Contrast-enhanced axial (A,B) and coronal (C) CT images show several well-defined round hypoattenuating parenchymal nodules (arrows) in both kidneys

. (D) US image shows a few well-defined hypoechoic nodular lesions in the renal parenchyma. US-guided biopsy of the kidney allowed confirmation of the diagnosis of IgG4-RD of the kidney.

A

B

C

D

Slide11

B

C

AA 57-year-old man with IgG4-RD of the kidneys. His serum IgG4 level was 13 200 mg/dL (132 g/L) with a reference range of 3–201 mg/dL (0.03–2.01 g/L). (A-C) Axial T2-weighted MR images show several small hypointense nodular lesions (arrows) in both renal cortices. US-guided biopsy allowed confirmation of IgG4-RD of the kidneys.

IgG4-RD of the Kidneys – Multiple Nodular Type

Slide12

IgG4-RD of the Kidneys – Patchy Infiltrative Type

A 64-year-old man with IgG4-RD of the kidneys (tubulointerstitial nephritis). (A, B) Axial (A) and coronal (B) CT images show multiple ill-defined patchy and confluent hypoattenuating lesions (arrows) in both kidneys.

B

C

A

(C) Contrast-enhanced CT image obtained after completion of steroid treatment shows improvement of bilateral renal parenchymal lesions. However, multifocal tiny cortical scars remain (arrows).

Slide13

IgG4-RD of the Kidneys

– Single Nodular TypeSingle nodular IgG4-RD of the kidneys in a 58-year-old man with left flank discomfort. His serum IgG4 level was 222.3 mg/dL (2.22 g/L) with a reference range of 3.9–86.4 mg/dL (0.039–0.86 g/L). (A) Axial contrast-enhanced nephrographic phase CT image shows a 1.7-cm round hypoattenuating nodular lesion (

arrow) in the left kidney. (B) Axial fat-suppressed T2-weighted MR image shows a hypointense nodular lesion (arrow) in the left kidney. (C, D) Axial diffusion-weighted image (b = 1000 sec/mm2) (C) and apparent diffusion coefficient (ADC) map (D) show a single nodular lesion (arrow), which appears as hyperintensity and hypointensity, respectively, and represents diffusion restriction. A

B

B

C

D

Slide14

IgG4-RD of the Kidneys

– Pelvic or Sinus Type

T2

B

= 1000

ADC

CET1

NECT

CMP

NP

EP

A 68-year-old man with IgG4-RD of the kidneys manifesting as a unilateral

renal pelvic lesion (arrow)

. Axial dynamic contrast-enhanced CT images (top row) show an

ill-defined soft-tissue lesion encasing the renal pelvis without hydronephrosis

. MR images (second row) also show an infiltrative lesion in the renal pelvis with mild diffusion restriction,

mimicking cancer of the renal pelvis

. Microscopic finding shows marked lymphoplasmacytic infiltration with storiform fibrosis and obliterative phlebitis, which is highly suggestive of IgG4-RD.

CET1

= contrast-enhanced T1-weighted image,

CMP

= corticomedullary phase,

EP

= extended phase,

NECT

= nonenhanced CT,

NP

= nephrographic phase.

Slide15

IgG4-RD of the Kidneys – Multiple Nodular and

Peripelvic

Upper row. A

36-year-old woman with IgG4-RD of the kidneys (tubulointerstitial nephritis), manifesting as several bilateral renal parenchymal nodules (yellow arrows) and

peripelvic soft-tissue infiltration (green arrow), mimicking lymphoma.

Lower row.

CT images

at 3-month follow-up after steroid treatment in the same patient show complete regression of bilateral renal parenchymal and left

peripelvic

lesions.

Slide16

Differential

DiagnosisBenign diseaseAcute

pyelonephritis, renal infarctionIgG4-RD of the Kidneys – Differential Diagnosis (1)

Acute pyelonephritis in a 63-year-old woman with fever and chills. Axial contrast-enhanced CT image shows several ill-defined patchy hypoattenuating lesions (arrows) in the right kidney.

Renal infarction

in a 57-year-old woman with acute flank pain. (A) Axial contrast-enhanced corticomedullary phase CT image shows

a few wedge-shaped lesions of poor contrast material enhancement

(arrows) in the left kidney. (B) Axial contrast-enhanced nephrographic phase CT image shows well-demarcated, wedge-shaped lesions of poor contrast material enhancement (arrows) in the left kidney. The right kidney is mildly atrophied and shows a few small cortical scars, presumably caused by chronic pyelonephritis.

A

B

Slide17

Differential

Diagnosis - Renal ParenchymaMalignant disease

lymphoma, renal cell carcinoma, and metastasisA 64-year-old man with multiple nodular renal lymphoma manifesting as several small low-attenuation nodular lesions (arrows) in the right kidney and multiple enlarged retroperitoneal lymph nodes (arrowheads).

IgG4-RD of the Kidneys – Differential Diagnosis (2)

A 62-year-old man with

multiple renal cell carcinoma

manifesting as

multiple round renal masses (yellow arrows),

with an ancillary finding of renal vein thrombosis

(orange arrow)

.

A 24-year-old woman with

multiple nodular lymphoma

. Axial contrast-enhanced CT image shows

multiple variably sized low-attenuating round mass lesions

(arrows) in both kidneys.

Slide18

Differential

Diagnosis – Renal Pelvis and Perirenal InvolvementMalignant disease

Lymphoma, urothelial cell carcinoma, Erdheim-Chester diseaseAn 82-year-old woman with mucosa-associated lymphoid tissue lymphoma (arrows), mimicking perinephric and renal pelvic involvement of IgG4-RD.Lymphoma involvement of the mesentery (arrows in lower right image) is also noted in this patient.

IgG4-RD of the Kidneys

– Differential Diagnosis (3)

A 77-year-old man with a

malignant urothelial tumor of the renal pelvis

(arrows)

, mimicking renal pelvic involvement of IgG4-RD.

Slide19

. IgG4-RD of the UreterLocalized ureter involvement of IgG4-RD is uncommon relative to

IgG4-RD of the kidneysClinical symptoms at presentationUreteral obstructive symptomsFlank pain , hematuria, proteinuria, and renal dysfunction with elevated creatinine levelSize of ureteral lesions: 1-4 cm (average, 2.6 cm) The ureteral IgG4-RD is usually diagnosed after surgical resection because it cannot be easily differentiated from urothelial carcinoma at radiographic or endoscopic evaluation

Slide20

Imaging findings of IgG4-RD of the ureter can be classified into

three types on the basis of morphologic features.Three types of gross and imaging findings(1) Polypoid mass-forming lesions (inflammatory pseudotumor)(2) Segmental ureteral wall thickening

(3) Periureteral fibrosisⅡ. IgG4-RD of the Ureter

Slide21

IgG4-RD of the Ureter - Polypoid Mass-forming Lesion

A 22-year-old man with IgG4-RD involving the ureter, manifesting as a polypoid ureteral mass (arrow) with hydronephrosis.

T2

CET1

T1

CET1

ADC

b

= 1000

Dynamic CET1

The polypoid ureteral mass shows

low signal intensity

on coronal

T2-weighted MR image

and

signal

isointensity

on axial T1-weighted MR image (top row).

The mass shows

homogeneous enhancement

on fat-suppressed gadolinium-enhanced T1-weighted MRI. The ureteral mass shows

no definite diffusion restriction

on high-

b

-value diffusion-weighted MR image and ADC map (middle row).

The mass shows gradual contrast enhancement

on

dynamic contrast enhanced T1-weighted

(CET1)

MR images (bottom row). Laparoscopic excision of the ureteral mass and end-to-end anastomosis of the ureter was performed, with the diagnosis pathologically confirmed as IgG4-RD, manifesting as an inflammatory pseudotumor.

Slide22

A 24-year-old man with IgG4-RD involving the ureter manifesting as (A) segmental concentric ureteral wall thickening

(arrows) with ureteral luminal narrowing and (B) obstructive hydronephrosis (arrow), mimicking urothelial cell carcinoma. (C) Left nephroureterectomy with bladder cuff excision was performed for the suspicion of urothelial cell carcinoma. Photograph of gross specimen shows encircling ureteral wall thickening (sclerosing ureteritis) with stenosis (arrows). Microscopic examination (not shown) confirmed IgG4-RD involving the ureter.

B

A

C

IgG4-RD of the Ureter

-

Segmental Concentric Ureteral Wall Thickening

Slide23

A 56-year-old man with IgG4-RD involving the ureter.(A-C) Coronal (A,B) and axial (C) contrast-enhanced CT images show a 2.8-cm length bulging soft-tissue-attenuation mass (arrows)

involving the left midureter and hydronephrosis.(D, E) Photograph of gross specimen shows a diffuse periureteral fibrotic mass and a smooth intact ureteral wall (arrows). Microscopic examination showed periureteric follicular lymphoid hyperplasia with increased IgG4 cells, which is suggestive of IgG4-RD.

A

B

D

E

IgG4-RD of the Ureter

- Infiltrative P

eriureteral Fibrosis (with Intact Ureter Wall)

C

** Teaching point

Discrimination between the segmental ureteral wall thickening and periureteral fibrosis on images is difficult and may be possible only at histopathologic evaluation. Direct visual examination and tissue biopsy by ureteroscopy may be helpful to rule out urothelial carcinoma.

Slide24

IgG4-RD of the Ureter – Differential Diagnosis.

Differential DiagnosisBenignUreteral papilloma, fibroepithelial polyp,

benign ureteral stricture with fibrotic scarMalignant

Urothelial cell carcinoma

A 79-year-old man with

urothelial cell carcinoma

of the left distal ureter.

Axial and sagittal contrast-enhanced CT images show a homogeneously enhancing soft-tissue-attenuation polypoid mass (arrow) and hydroureter.

Slide25

. IgG4-RD of the Bladder Bladder and urethral involvement of IgG4-RD is

much rarer than kidney involvement: only a few case reports of isolated IgG4-RD of the bladder exist:Intraluminal polypoid mass arising from the bladder wall – mimicking bladder cancerKufukihara R, Niwa N, Mizuno R, et al. Immunoglobulin G4-RD arising from the bladder wall. Urol

Int 2018:1-3. Transmural bladder wall thickening

- mimicking bladder cancer with invasion to the sigmoid colon Park S, Ro JY, Lee DH, Choi SY, Koo H. Immunoglobulin G4-associated inflammatory pseudotumor of urinary bladder: a case report. Ann Diagn Pathol

2013;17(6):540-543.

3)

Extravesical

and

periurethral

mass

with urinary distension

– resembling, at first glance, a

prostate gland in a female patient

Sangsoad

P,

Ramart

P,

Korpraphong

P, et al: Female urinary retention from a huge periurethral mass caused by immunoglobulin G4-RD.

Urol Case Rep 2019; 24:100844

Slide26

. IgG4-RD of the ProstateTwo types of radiologic findings (1) Diffuse infiltrative type(2) Periprostatic mass-forming type

Clinical symptoms No symptoms or symptoms associated with benign prostate hypertrophyIgG4-RD prostatitis can occur simultaneously with prostatic adenocarcinoma Mild increase or normal serum PSA (prostate-specific antigen) level – less than marked elevation in prostate cancer

Bourlon MT et al. IgG4-related autoimmune prostatitis: is it an unusual or underdiagnosed manifestation of IgG4-related disease? Case Rep Urol

2013; 2013:295472

Slide27

A 54-year old man with IgG4-RD of the prostate manifesting as

diffuse enlargement of the prostate gland

and a loss of normal zonal anatomy of the prostate gland on the T2-weighted MR image, mimicking diffuse prostatitis or lymphoma. Mild diffusion restriction is noted in the whole prostate gland on high-b-value diffusion-weighted image (bottom left) and ADC map (bottom right). IgG4-RD prostatitis was pathologically confirmed after transrectal US-guided core biopsy. T2

ADC

b = 1000

US

T2

IgG4-RD of the Prostate

- Diffuse Infiltrative Type

Slide28

A 61-year-old man with IgG4-RD of the prostate, manifesting as a

homogeneously enhancing periprostatic mass lesion (yellow arrows) surrounding the prostate gland (A) and increased FDG uptake on PET/CT image (B). (C) Normal prostate gland (green arrows) is surrounded by enhancing periprostatic mass lesions.

D. Bilateral ill-defined soft-tissue infiltrative lesions (orange arrows) involving the bilateral renal pelvis are also noted and are suggestive of renal pelvis involvement of IgG4-RD.

IgG4-RD of the Prostate

- Periprostatic Mass-forming Type

A

B

C

D

Slide29

Manifests as a form of paratesticular pseudotumor or

epididymo-orchitis Paratesticular pseudotumor: three case reports (age range, 23–52 years)Solitary or multiple intrascrotal nodules Composed of dense fibrous tissue with an inflammatory infiltrate of variable severityThis condition belongs to the list of IgG4-RDs.

Ⅴ. IgG4-RD of the TestesBrito-Zeron P, Ramos-Casals M, Bosch X, et al: The clinical spectrum of IgG4-related disease.

Autoimmun Rev 2014;13(12):1203-1210.Bosmuller H, von

Weyhern CH, Adam P, et al: Paratesticular fibrous pseudotumor--an IgG4-related disorder? Virchows

Arch 2011;458(1):109-113.

Slide30

IgG4-RD can involve almost every organ in the genitourinary system.

IgG4-RD involving the kidneys, ureter, bladder, urethra, prostate, and testes can show a broad spectrum of imaging findings in the forms of a localized mass in or surrounding the involved organ or diffuse enlargement of the involved organ, which may mimic a variety of both benign and malignant diseases.Although imaging findings are nonspecific for genitourinary system involvement of IgG4-RD, imaging has a key role in the detection of disease and monitoring treatment response. Therefore, radiologists should be aware of variable imaging manifestations, clinical-pathologic features, and differential diagnosis from the mimics of IgG4-RD for accurate diagnosis to avoid unnecessary surgery and provide timely effective treatment.

Summary