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Year : 2021  |  Volume : 36  |  Issue : 2  |  Page : 214-216  

IgG4 related kidney disease with extra-renal involvement demonstrated on FDG PET/CT

Department of Nuclear Medicine, MIOT International, Chennai, Tamil Nadu, India

Date of Submission06-Oct-2020
Date of Decision12-Oct-2020
Date of Acceptance15-Oct-2020
Date of Web Publication21-Jun-2021

Correspondence Address:
Dr. Piyush Chandra
Department of Nuclear Medicine, MIOT International, Manapakkam, Chennai - 600 056, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijnm.ijnm_206_20

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Immunoglobulin G4 (IgG4)-related kidney disease is a relatively rare clinical entity and usually occurs as an extra-pancreatic manifestation of IgG4-related autoimmune pancreatitis. We describe here the imaging findings of a patient who presented with recurrent multiorgan IgG4-related disease, involving bilateral kidneys/ureters, proximal small bowel, and multiple abdominal and extra-abdominal lymph nodes.

Keywords: 2-Fluoro-2-deoxy glucose, immunoglobulin G4, immunoglobulin G4-related kidney disease, inflammatory, kidney, positron emission tomography/computed tomography

How to cite this article:
Chandra P, Nath S. IgG4 related kidney disease with extra-renal involvement demonstrated on FDG PET/CT. Indian J Nucl Med 2021;36:214-6

How to cite this URL:
Chandra P, Nath S. IgG4 related kidney disease with extra-renal involvement demonstrated on FDG PET/CT. Indian J Nucl Med [serial online] 2021 [cited 2022 Aug 20];36:214-6. Available from:

   Case History Top

A 52-year-old male with a history of immunoglobulin G4 (IgG4)-related biliary stricture, treated with steroids in 2017, presented with increased stool frequency and weight loss for 2 months. Blood reports revealed normal total white blood cell count with increased differential eosinophil counts - 13% (normal <6%), absolute eosinophil count - 1190 cells (normal <450 cells/cumm), normal lactate dehydrogenase - 142 IU/L (normal <450 IU/L), and elevated serum IgG4 subclass - 353 mg/dL (normal range for >18 years old: 3–201 mg/dL). Routine urine analysis does not reveal any infections/proteinuria, and urine cytology was negative malignancy. Colonoscopy-guided biopsy was suggestive of colitis. In view of known history of IgG4-related disease, a whole-body 18F-2-fluoro-2-deoxy glucose (FDG) positron emission tomography/computed tomography (PET/CT) was done which revealed FDG uptake in the diffuse thickening of the pelvis of bilateral kidneys [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g, yellow arrows, pelvis of left kidney - SUVmax - 11.32] and bilateral proximal ureter [white arrows, [Figure 1]e, left ureter - SUVmax - 4.45] with smooth and patent urinary lumen [[Figure 1]b, [Figure 1]c, [Figure 1]d, red arrows]. On delayed post-intravenous (IV) furosemide, delayed PET/CT images showed soft tissue density in the bilateral pelvis without any delayed contrast retention [[Figure 1]f, yellow arrow heads] and with persistent FDG uptake in the bilateral pelvis/ureteric wall [[Figure 1]g, white arrow head]. In addition, FDG PET/CT showed increased FDG uptake in the multiple enlarged mesenteric/retroperitoneal/pelvic nodes (with surrounding mesenteric fat stranding) and mild wall thickening in the proximal small bowel and proximal large bowel [[Figure 2]a, [Figure 2]b, [Figure 2]c, yellow arrows]. Extra-abdominal increased FDG uptake was also noted in the bilateral tonsils and enlarged bilateral axillary and cervical nodes [Figures 1a and 2d]. In view of patient's history, current imaging, and blood investigations, diagnosis of IgG4-related kidney disease (IgG4-RKD) relapse was established without need for re-biopsy. The patient was treated steroids which subsequently showed good clinical improvement.
Figure 1: (a) Whole-body 2-fluoro-2-deoxy glucose positron emission tomography MIP image showing increased 2-fluoro-2-deoxy glucose uptake in the bilateral kidneys (black arrows), bilateral palatine tonsils, and bilateral cervical, axillary, and pelvic nodes. (b and c) Trans-axial computed tomography and positron emission tomography/computed tomography images showing enhancing soft tissue thickening in the bilateral renal pelvis (small white arrows) with smooth intraluminal urinary lumen (dashed white arrow). (d and e) Coronal computed tomography and positron emission tomography/computed tomography images showing bilateral pelvis and proximal ureteric thickening (long white arrow). (f and g) Delayed computed tomography and positron emission tomography/computed tomography images showing no contrast retention on computed tomography and persistent accumulation of 2-fluoro-2-deoxy glucose in the bilateral renal pelvis and proximal ureters (white arrow heads) suggestive of inflammation

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Figure 2: (a and b) Coronal computed tomography and positron emission tomography/computed tomography images showing 2-fluoro-2-deoxy glucose uptake in the thickened duodenal wall (long white arrows) with adjacent mesenteric fat stranding and multiple enlarged mesenteric nodes (short white arrow). (c and d) Trans-axial positron emission tomography/ computed tomography images showing increased 2-fluoro-2-deoxy glucose uptake in the enlarged pelvic and axillary nodes

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   Discussion Top

IgG4-RKD is a relatively newly recognized disease after many reports associated its occurrence in patients with autoimmune pancreatitis.[1],[2] Early recognition of the disease is important as it is a condition associated with dramatic response to IV steroids.[3] Imaging features, best described on CT, are the characteristics and often the first recognized manifestation of this disease. Most common pattern reported on CT appears to be multiple rounded low-density lesions in the bilateral renal cortex. Other less common patterns reported are diffuse enlargement of both kidneys or diffuse thickening of the pelvis with smooth intraluminal surface (as see in in our case), or rarely, the disease may present as a solitary hypovascular kidney mass. These characteristic radiology appearances along with serological profile can help differentiate IgG4-related kidney disease with other mimicking systemic illness (such as pyelonephritis, lymphoma, sarcoidosis, vasculitis, renal malignancies, and Castleman's disease).[4],[5]

IgG4-related fibroinflammatory disease may virtually involve every other organs including pancreas (most common), liver, bile ducts, stomach, salivary glands, thyroid, kidneys, and colon.[6],[7],[8] Due to the same reason, the clinical symptoms are variable/nonspecific, often leading to delay in diagnosis and thereby threatening organ function. Growing evidence over the past half-decade favors the use of PET/CT as a one-stop shop for imaging IgG4-related diseases for diagnosis, guiding biopsy, and treatment response evaluation.[9],[10] Detecting extra-renal involvement at baseline appears to be the most important indication of using FDG PET/CT in IgG4-RKD.[11],[12] Appropriate use of PET/CT should be guided by the serum IgG4 levels, as the probability of detecting multiorgan disease is >80% if the serum IgG4 levels are two times the upper limit of normal (i.e., >270 mg/dL, normal limit ≤135 mg/dL).[13] Another potential use of FDG PET/CT in this disease would be in avoiding invasive tissue diagnosis, especially in recurrent disease setting.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Uchiyama-Tanaka Y, Mori Y, Kimura T, Sonomura K, Umemura S, Kishimoto N, et al. Acute tubulointerstitial nephritis associated with autoimmune-related pancreatitis. Am J Kidney Dis 2004;43:e18-25.  Back to cited text no. 1
Watson SJ, Jenkins DA, Bellamy CO. Nephropathy in IgG4-related systemic disease. Am J Surg Pathol 2006;30:1472-7.  Back to cited text no. 2
Saeki T, Nishi S, Imai N, Ito T, Yamazaki H, Kawano M, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int 2010;78:1016-23.  Back to cited text no. 3
Takahashi N, Kawashima A, Fletcher JG, Chari ST. Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 2007;242:791-801.  Back to cited text no. 4
Kawano M, Saeki T, Nakashima H, Nishi S, Yamaguchi Y, Hisano S, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol 2011;15:615-26.  Back to cited text no. 5
Obiorah IE, Henao Velasquez A, ízdemirli M. The clinicopathologic spectrum of IgG4-related disease. Balkan Med J 2018;35:292-300.  Back to cited text no. 6
Vadi SK, Parihar AS, Kumar R, Singh H, Mittal BR, Bal A, et al. IgG4-related disease simulating carcinoma colon with diffuse peritoneal carcinomatosis on 18F-FDG PET/CT. Clin Nucl Med 2018;43:e247-9.  Back to cited text no. 7
Chandra P, Nath S, Jain D. Pancreatitis, cholangitis, and gastritis: The triumvirate of immunoglobulin G4-related disease identified simultaneously on18F-fluorodeoxyglucose positron emission tomography/computed tomography. Indian J Nucl Med 2019;34:335-7.  Back to cited text no. 8
[PUBMED]  [Full text]  
Zhang J, Chen H, Ma Y, Xiao Y, Niu N, Lin W, et al. Characterizing IgG4-related disease with 18F-FDG PET/CT: A prospective cohort study. Eur J Nucl Med Mol Imaging 2014;41:1624-34.  Back to cited text no. 9
Lee J, Hyun SH, Kim S, Kim DK, Lee JK, Moon SH, et al. Utility of FDG PET/CT for differential diagnosis of patients clinically suspected of IgG4-related disease. Clin Nucl Med 2016;41:e237-43.  Back to cited text no. 10
Fu Z, Liu M, Zhang J, Li Z, Li Q. IgG4-related renal lesions detected by delayed 18F-FDG PET/CT scan. Clin Nucl Med 2017;42:66-7.  Back to cited text no. 11
Krebs S, Monti S, Seshan S, Fox J, Mannelli L. IgG4-related kidney disease in a patient with history of breast cancer: Findings on 18F-FDG PET/CT. Clin Nucl Med 2016;41:e388-9.  Back to cited text no. 12
Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, et al. IgG4-related disease: Dataset of 235 consecutive patients. Medicine (Baltimore) 2015;94:e680.  Back to cited text no. 13


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