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Table of Contents
Year : 2020  |  Volume : 31  |  Issue : 6  |  Page : 285-287

Immunoglobulin g4-related bilateral kidney disease: The clinical course and outcome: A case report

Department of Surgery, Division of Urology, Faculty of Medicine, Songklanagarind Hospital, Prince of Songkla University, Songkhla, Thailand

Date of Submission23-Mar-2020
Date of Decision27-May-2020
Date of Acceptance20-Jun-2020
Date of Web Publication26-Dec-2020

Correspondence Address:
Worapat Attawettayanon
Department of Surgery, Division of Urology, Faculty of Medicine, Songklanagarind Hospital, Prince of Songkla University, Songkhla
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/UROS.UROS_31_20

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Immunoglobulin G4-related disease (IgG4-RD) is uncommon and is characterized by dense tissue infiltration of IgG4-positive plasma cells or mass-like sclerosing lesions. In addition, it can involve any anatomic site, which has frequently elevated serum IgG4 concentrations. Japanese gastroenterologists are the ones who first discussed the concept of IgG4-RD; they first introduced the term IgG4-related kidney disease (IgG4-RKD), which refers to the involvement of the kidney and its surrounding tissues. Common urologic indicators of IgG4-RKD typically include subacute pain, on the detection of a tumor-like mass or enlargement, with or without tubulointerstitial nephritis. Conventional imaging computed tomography and magnetic resonance imaging are of limited usefulness in determining IgG4-RKD. The gold-standard for the diagnosis of IgG4-RD is the identification of typical histopathological features of dense lymphoplasmacytic infiltration, storiform fibrosis (an irregular cartwheel like fibrotic pattern), and obliterative phlebitis; these are the three hallmarks of IgG4-RD. The current report discusses a case of IgG4-RKD, who came into our hospital with chronic flank pain.

Keywords: Bilateral renal tumor, immunoglobulin G4-related kidney disease, renal failure, repeated renal biopsies

How to cite this article:
Anukoolphaiboon A, Attawettayanon W. Immunoglobulin g4-related bilateral kidney disease: The clinical course and outcome: A case report. Urol Sci 2020;31:285-7

How to cite this URL:
Anukoolphaiboon A, Attawettayanon W. Immunoglobulin g4-related bilateral kidney disease: The clinical course and outcome: A case report. Urol Sci [serial online] 2020 [cited 2021 Jan 17];31:285-7. Available from: https://www.e-urol-sci.com/text.asp?2020/31/6/285/305092

  Introduction Top

IgG4-related kidney disease (IgG4-RKD) is an inclusive term for kidney lesion associated with IgG4-related disease. The incidence of IgG4-RKD is rare at approximately 15% of IgG4-related disease (IgG4-RD) with age range at diagnosis from 50 to 75 years. The most common of renal feature in IgG4-RD are IgG4-related tubulointerstitial nephritis and membranous glomerulonephropathy.[1] Patients may present with acute or chronic renal dysfunction, unilateral or bilateral renal mass. The diagnosis of IgG4-RD is based on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4+ plasma cells.[2],[3] Genitourinary involvement in IgG4-RD has been reported in short series and isolated in case reports, most often involving the kidneys in so-called IgG4-RKD. Radiographic imaging of disease can mimic malignancies and is often misdiagnosed due to its rarity.[4] Serum IgG4 concentration elevations are considered the essential condition in the diagnosis of IgG4-RD, whereas normal serum IgG4 concentrations are also reported in active biopsy-proven disease.[5] Recognition of this disease is important to ensure diagnoses, which can prevent unnecessary surgical procedure.

  Case Report Top

A 53-year-old, Thai male presented with chronic bilateral flank pain for 4 months. He had dull pain, but this was not related to the position. He denied having a history of gross hematuria, and his underling disease was a chronic obstructive pulmonary disease and occasional use of a salbutamol inhaler. He had no organ-specific signs or symptoms. On examination, we palpated his kidney on both sides by bimanual palpation. An abdominopelvic computed tomography (CT) scan revealed an infiltrative left renal mass and a ureteric mass with moderate left hydronephrosis. On the right kidney, we found a wedge-shape in the mid-to-lower pole with right renal pelvic involvement, multiple mediastinal, hilar, intraabdominal, and paraaortic and iliac lymph nodes enlargement of up to 1.5 cm, as shown in [Figure 1]. We diagnosed the patient as having bilateral upper urinary tract urothelial carcinoma. We then sent the patient for an initial workup, with the first step of the workup consisting of cystoscopy and bilateral retrograde pyelography (RP). The findings were normal bladder mucosa, and both ureteric orifices were normal. The RP revealed no abnormal filling defect; moderate left hydronephrosis, and urine cytology were collected from both sides of the ureter and bladder. The results of the cytology were all negative for malignancy.{Figure 1}

The patient was then sent to an interventionist for tissue diagnosis. The first renal biopsy was done using the percutaneous technique, with an ultrasound-guided biopsy at the bilateral sites. The pathology revealed the end-stage glomerular disease of the left side kidney specimen coupled with dense fibrotic tissue and infiltration of predominant small-to-medium-sized T-lymphocytes of the right retroperitoneum.

Due to insufficient tissue for a definite diagnosis, a re-biopsy was done using an open technique. During the intraoperative process, we found an enlarged pale in color left kidney and a firm to hard consistency and thickening in the wall of the left proximal ureter with hydronephrosis. A biopsy was then performed from three spots: the upper pole kidney, the mid pole of the kidney, and the proximal ureter. The tissue pathology revealed immunoglobulin G4-related disease (IgG4-RD), as shown in [Figure 2]. Immunohistochemical techniques showed predominantly fibrosis with increased plasma cells and eosinophils infiltration, IgG4(+) plasma cells 30–80/HPF, IgG4/IgG(+), and a plasma cell ratio of about 30%, with polytypic plasma cell population. We also sent serum blood for serum IgG levels and the value was 3380 g/L (Ref, Adult 700–1600 g/L). Two days after the procedure, the patient developed left flank pain, with gross hematuria adjoined with a significant decrease in his hemoglobin level (12.7–7.0 mg/dL). We suspected this was due to parenchymal bleeding; therefore, the patient was selected for CT angiography. The finding was active contrast extravasation at the left upper pole, supplied by a branch of the posterior division of the left renal artery, minimal hemoperitoneum at perisplenic, and both paracolic gutters. We consulted an interventionist for embolization. After embolization, the patient improved and was subsequently discharged.{Figure 2}

We were planning to treat the patient with a corticosteroid; prednisolone (0.6 mg/kg/day) and step down to maintenance dose up to 2–3 years. The patient followed up at the outpatient department and was doing well. At 6-month follow-up imaging, by an abdominopelvic CT scan, revealed partially improving IgG4-RD, as smaller sized bilateral renal, perinephric lesions and para-aortic nodes, as well as an improvement of the left renal nephrography and excretion. After treatment for 6 months, the serum IgG4 was reduced to 1.014 g/L.

  Discussion Top

Immunoglobulin G4-related kidney disease (IgG4-RKD) is a rare disease at approximately 15% of IgG4-RD. The age range at diagnosis is from 50 to 75 years. The presentation mimics tumors making this condition a challenge to diagnose. The imaging features cannot identify reliable conditions between IgG4-RKD and cancer. The common findings from imaging may show bilateral peripheral cortical nodules, well-defined wedge-shaped lesions or diffuse involvement as well as enlargement of both kidneys.[6] Magnetic resonance imaging found well-demarcated on T2-weighted and contrast-enhanced T1-weighted.[7] The inflammatory lesion usually forms an infiltrative mass that can involve organ destruction. Due to the presentation and imaging mimicking malignancy, diagnosis is mostly established by nephrectomy.[8] Therefore, patients with bilateral infiltrative lesions on imaging should have awareness of these factors, so as to avoid unnecessary surgical operations.

In our case, the clinical and imaging findings at initial presentation were highly suspect of a systemic disease due to bilateral and infiltration involvement. However, this finding suspected an inflammatory response more so than malignancy, and fortunately, the pathologic finding was negative for malignancy. In so saying, the elevation of serum IgG4 levels and immunohistochemistry confirmed the diagnosis of IgG4-RKD.

The way in which tissue diagnosis is received is an important issue with an imaging-guided biopsy being the first choice for this procedure. The choice of which imaging modality used is dependent on the physician's preference and did not affect the sensitivity or negative predictive value.[9] Likewise, results show that core biopsy specimens are better than fine-needle aspiration specimens, in terms of sensitivity and specificity. In our case, we were not able to use the percutaneous technique for diagnosis due to tissue insufficiency. Thus, we followed an open technique for the 2nd procedure.

After confirming the diagnosis, the management process was a systemic glucocorticoid. The starting dose was 1 mg/kg/day, for 2–4 weeks, then gradually tapered to a maintenance dose of 2.5–5 mg/day, over a period of 2–3 months.[10] The response of treatment improved renal function and appearance of renal lesions on imaging.[11] Unlike evidence recorded in previous studies, our patient was improving in the appearance of renal lesions on imaging, and IgG4 levels, with stabilization in renal function.

In conclusion, due to the rarity of IgG4-RKD, the distinctive diagnosis of multiple and bilateral renal masses should be taken into consideration. Tissue diagnosis should be used to correctly detect the IgG4-RKD. Most patients with IgG4-RKD may have other organs involvement at diagnosis; hence, careful evaluation of multiple and bilateral renal mass lesions would provide important information to reach a definite diagnosis of the IgG4-RKD.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Quattrocchio G, Roccatello D. IgG4-related nephropathy. J Nephrol 2016;29:487-93.  Back to cited text no. 1
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.  Back to cited text no. 2
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. 3
Bianchi D, Topazio L, Gaziev G, Iacovelli V, Bove P, Mauriello A, et al. IgG4-related kidney disease: Report of a case presenting as a renal mass. Case Rep Surg 2017;2017:9690218.  Back to cited text no. 4
Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikuva M, Pillai S, et al. IgG4-related disease: Baseline clinical and laboratory features in 125 patients with biopsy-proven disease. Arthritis Rheumatol 2015;67:2466-75.  Back to cited text no. 5
Horger M, Lamprecht HG, Bares R, Spira D, Schmalzing M, Claussen CD, et al. Systemic IgG4-related sclerosing disease: Spectrum of imaging findings and differential diagnosis. AJR Am J Roentgenol 2012;199:W276-82.  Back to cited text no. 6
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. 7
Surintrspanont J, Sanpawat A, Sasiwimonphan K, Sitthideatphaiboon P. IgG4-related pseudo-tumor of the kidney and multiple organ involvement mimicked malignancy. Urol Case Rep 2019;26:100953.  Back to cited text no. 8
Caoili EM, Davenport MS. Role of percutaneous needle biopsy for renal masses. Semin Intervent Radiol 2014;31:20-6.  Back to cited text no. 9
Kamisawa T, Okazaki K. Diagnosis and treatment of IgG4-related disease. Curr Top Microbiol Immunol 2017;401:19-33.  Back to cited text no. 10
Saeki T, Kawano M, Mizushima I, Yamamoto M, Wada Y, Nakashima H, et al. The clinical course of patients with IgG4-related kidney disease. Kidney Int 2013;84:826-33.  Back to cited text no. 11


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