In 1986, a 45-year-old man, who had a past medical history of swollen submandibular lymph nodes and colon cancer, was admitted for an evaluation of eyelid swelling and generalized edema. On admission, in addition to massive proteinuria, increased total protein (9.2 g/dL) and low albumin (2.7 g/dL) were noted. By several radiological and endoscopic examinations, a diagnosis of chronic pancreatitis was made. And the finding of multiple peritoneal lymph nodes raised the possibility of a cancer recurrence. An open biopsy of several involved organs was performed. The diagnostic conclusions in 1986 were as follows: (1) reactive follicular hyperplasia (lymph node); (2) reactive hepatitis; (3) chronic pancreatitis; and (4) idiopathic membranous nephropathy (MN). These findings were considered to represent a state of hyperimmune reactivity, but no precipitating factor could be identified at that time. When the diagnosis was revisited 25 years later, reinterpretation of the histology, performance of IgG4 staining, and recognition of the features of IgG4-RD in other organs led to reclassification of the patient’s diagnosis as IgG4-related MN without tubulointerstitial nephritis.
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