1) The document describes a case report of a 66-year-old female who presented with backache and renal failure. Imaging revealed a contrast-enhancing mass in the left renal hilum encasing the aorta, and hydronephrosis. 2) An initial biopsy was suggestive of lymphoma but treatment did not improve symptoms. A subsequent biopsy found elevated IgG4 levels and histological features consistent with IgG4-related kidney disease (IgG4-RKD). 3) The patient was started on oral steroids and follow-up imaging showed partial resolution of the mass and hydronephrosis, demonstrating that IgG4-RKD can present as a renal mass and be treated with steroids.

1. Poster Number:
Title: IGG4-RELATED KIDNEY DISEASE OF RENAL PELVIS,
MIMIKING MALIGNANCY : A CASE REPORT
Authors:
Devesh Bansal, Manas Babu, Sony Mehta, Biju Pillai, Mohan P sam , H Krishnamoorthy
Institution: Department of Urology Lourdes Hospital Kochi

2. INTRODUCTION-
Immunoglobulin G4-related related disease is a fibro-inflammatory condition most commonly
associated with autoimmune pancreatitis. The disease can also manifest as extra-pancreatic
disease involving the kidney, bile ducts, lung, and retroperitoneum. Immunoglobulin G4-related
disease was first described by Hamano et al, who described elevated serum IgG4 concentrations
in patients with autoimmune pancreatitis. We describe the clinical, radiographic and pathologic
findings in a patient with isolated renal involvement in IgG4-related sclerosing disease which
resulted in renal failure.
CASE REPORT
A 66-year-old female presented with backache, and renal failure. She had no fever, hematuria
The routine investigation showed (Hb- 9.2 g/l, creatinine, 3.1 mg/dl). Urine cytology for
malignant cells was negative. Bilateral ureteric stenting was performed initially. CT urogram
revealed 4.3x3.5cm contrast enhancing mass lesion infiltrating the left renal hilum with
hydronephrosis with encasement of aorta. Open biopsy from mass lesion revealed suspicion of
Non Hodgkins lymphoma. Rituximab was started for treatment of Non Hodgkins lymphoma.
However after two cycle of therapy no improvement was observed. The patient serum IgG4
level was raised (384mg/dl). Repeat CT guided trucut biopsy was suggestive of IgG4RKD
according to a diagnostic criteria proposed in the literature. Oral prednisolone was started after
diagnosis at a dose of 40 mg daily, follow up investigations suggested partial resolution of mass
lesion and hydronephrosis.
CT urogram revealed 4.3x3.5cm contrast
enhancing mass lesion infiltrating the left
renal hilum and aorta with B/L
hydronephrosis
Kidney showing a lymphoplasmacytic
infiltrate in the renal interstitium with
obliterative phlebitis and storiform fibrosis
within the renal interstitium.

3. DISCUSSION
IgG4-RD is a systemic inflammatory condition characterised
by mass-forming lesions. This was first described by
Hamano et al, who described elevated serum IgG4 in
patients with autoimmune pancreatitis.
Kawano et al proposed the diagnostic criteria of IgG4-
RKD based on the presence of typical radiological
appearances, raised serum IgG4 levels and specific
histological findings like dense lymphoplasmacytic infiltrate,
fibrosis, arranged at least focally in a storiform pattern,
obliterative phlebitis, increased numbers of eosinophils.
Though there is no definitive treatment strategy for, IgG4-
RKD, various treatment methods which including “Watch-
and-wait” strategy, systemic glucocorticoids, and steroid-
sparing immunomodulators indicated for this disease
condition.
CONCLUSION
IgG4-RKD may present as hydronephrosis
arising in the renal pelvis and ureter, mimicking a
ureteral/renal tumor. Early recognition of this
disease is important for reversal of renal
deterioration and overall prognosis. Large
clinical trials are needed for studying clinical
manifestations, approaches to diagnosis, and
treatment of patients with IgG4-RKD.
REFERENCES
Stone J, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539–51.
Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing
pancreatitis. N Engl J Med. 2001;344:732–8
Kawano M, Saeki T, Nakashima H et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol
2011;15:615–26.
S. Shoji, M. Nakano, and Y. Usui, “IgG4-related inflammatory pseudotumor of the kidney,” International Journal of Urology, vol. 17,
no. 4, pp. 389–390, 2010
Partial resolution of mass lesion at
left renal hilum (1.6x1 cm) on follow
up CT scan.