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Sueta S, Kondo M, Matsubara T, et al. Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit. CEN Case Rep. 2013;3(1):18-23doi: 10.1007/s13730-013-0077-y.
Sueta, S., Kondo, M., Matsubara, T., Yasuhara, Y., Akiyama, S., Imai, E., Amaike, H., & Tagawa, M. (2014). Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit. CEN case reports, 3(1), 18-23. https://doi.org/10.1007/s13730-013-0077-y
Sueta, Shinichi, et al. "Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit." CEN case reports vol. 3,1 (2014): 18-23. doi: https://doi.org/10.1007/s13730-013-0077-y
Sueta S, Kondo M, Matsubara T, Yasuhara Y, Akiyama S, Imai E, Amaike H, Tagawa M. Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit. CEN Case Rep. 2014 May;3(1):18-23. doi: 10.1007/s13730-013-0077-y. Epub 2013 May 03. PMID: 28509238; PMCID: PMC5411534.
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CEN Case Rep. 2014 May;3(1):18-23. doi: 10.1007/s13730-013-0077-y. Epub 2013 May 03.

Membranous nephropathy associated with type 1 autoimmune pancreatitis and dominant glomerular IgG4 deposit.

CEN case reports

Shinichi Sueta, Makiko Kondo, Takeshi Matsubara, Yumiko Yasuhara, Shinichi Akiyama, Enyu Imai, Hisashi Amaike, Miho Tagawa

Affiliations

  1. Department of Nephrology, Kyoto Katsura Hospital, 17 Yamada-Hirao-cho, Nishikyo-ku, Kyoto, 6158256, Japan.
  2. Department of Nephrology, Kyoto University Graduate School of Medicine, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 6068507, Japan.
  3. Department of Diagnostic Pathology, Kyoto Katsura Hospital, 17 Yamada-Hirao-cho, Nishikyo-ku, Kyoto, 6158256, Japan.
  4. Department of Nephrology, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, Aichi, 4668550, Japan.
  5. Department of Surgery, Kameoka Municipal Hospital, 1-1 Shino-Noda, Shino-cho, Kameoka, Kyoto, 6218585, Japan.
  6. Department of Nephrology, Kyoto Katsura Hospital, 17 Yamada-Hirao-cho, Nishikyo-ku, Kyoto, 6158256, Japan. [email protected].

PMID: 28509238 PMCID: PMC5411534 DOI: 10.1007/s13730-013-0077-y

Abstract

We report a case of membranous nephropathy associated with type 1 autoimmune pancreatitis. A 58-year-old man presented with anorexia. Work-up revealed a mass in the pancreatic head, which was subsequently resected. Pathological examination showed diffuse infiltration of immunoglobulin (Ig) G4-positive plasma cells, which was compatible with the diagnosis of type 1 autoimmune pancreatitis. Serum IgG4 was elevated. He developed nephrotic syndrome around the time of the surgery. Kidney biopsy confirmed the diagnosis of membranous nephropathy. Immunofluorescent staining showed predominant glomerular IgG4 deposit among IgG subclasses. Tubulointerstitial nephritis, which is usually a dominant feature of renal involvement in IgG4-related disease, was not observed. The patient was treated with prednisolone and several immunosuppressants. During the course, the degree of proteinuria was associated with the serum IgG4 level. Serum antibody against phospholipase A2 receptor was negative. These findings together with IgG4-dominant glomerular deposit suggest that IgG4 may play a unique role in the pathogenesis of secondary membranous nephropathy caused by IgG4-related diseases.

Keywords: IgG4; IgG4-related disease; Membranous nephropathy

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