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.
Highlights
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is characterized by increased serum IgG4 levels, caused by the infiltration of IgG4-positive cells into various organs [1,2,3]
We report a case of membranous nephropathy associated with type 1 autoimmune pancreatitis
Tubulointerstitial nephritis (TIN) was a dominant feature and glomerular involvement was reported in 24 %: 3 cases with membranous nephropathy (MN), 1 with membranoproliferative glomerulonephritis, 4 with mesangial proliferative glomerulonephritis, and 1 with endocapillary proliferative glomerulonephritis
Summary
Shinichi Sueta • Makiko Kondo • Takeshi Matsubara • Yumiko Yasuhara • Shinichi Akiyama • Enyu Imai • Hisashi Amaike • Miho Tagawa Received: 15 November 2012 / Accepted: 3 April 2013 / Published online: 3 May 2013 Ó The Author(s) 2013. This article is published with open access at Springerlink.com
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