Case Review of lgG4-Related Disease Patient with Combined
Tubulointerstitial Nephritis and Membranous Nephropathy
Coexisting with Cholangiocarcinoma:
Case Report:
Wipada Kraikriangsri, MD¹, Suchin Worawichawong, MD²
Affiliation : ¹ Department of Medicine, Chulabhorn Hospital, HRH Princess Chulabhorn College of Medical Science, Chulabhorn Royal Academy, Bangkok, Thailand ² Department of Pathology Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as an autoimmune disorder involving multiple organs.
The kidney is a represented organ with a wide range of renal manifestations. The authors report a case of an 83-year-old Thai
male with combined IgG4 tubulointerstitial nephritis and membranous nephropathy coexisting with cholangiocarcinoma. The
patient presented with proteinuria, acute renal failure, eosinophilia, hypocomplementemia, and high serum IgG4 concentration.
The diagnosis was IgG 4-related tubulointerstitial nephritis and membranous nephropathy on renal biopsy, with negative
immunohistochemistry for anti-phospholipase A2 receptor antibodies. Magnetic resonance imaging (MRI) abdomen showed two
wedge shaped arterial enhancing lesions of liver. Liver biopsy revealed adenocarcinoma, compatible with cholangiocarcinoma.
Proteinuria and renal failure were resolved with initial steroid treatment. Meanwhile, IgG4-related membranous nephropathy
should be considered in the differential diagnosis for patients with proteinuria. Potentially, IgG4-RD may be rarely associated
with carcinoma development. However, further studies are recommended to ratify and confirm the association between IgG4-
RD and incidence of malignancies.
Received 13 March 2020 | Revised 19 May 2020 | Accepted 22 May 2020
doi.org/10.35755/jmedassocthai.2020.11.10685
Keywords : IgG4-related disease, Membranous nephropathy, Secondary membranous nephropathy, Tubulointerstitial nephritis, Cholangiocarcinoma
