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2014 ; 3
(1
): 18-23
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Membranous nephropathy associated with type 1 autoimmune pancreatitis and
dominant glomerular IgG4 deposit
#MMPMID28509238
Sueta S
; Kondo M
; Matsubara T
; Yasuhara Y
; Akiyama S
; Imai E
; Amaike H
; Tagawa M
CEN Case Rep
2014[May]; 3
(1
): 18-23
PMID28509238
show ga
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.