A 78-year-old-man was admitted to our hospital because of
renal insufficiency 20 months after the onset of
autoimmune pancreatitis. He had
cerebral infarction and
prostatic hypertrophy as complications. He had been previously diagnosed with
autoimmune pancreatitis (AIP). The initial
therapy was started with oral
prednisolone at the dose of 0.8 mg/kg (40 mg/day).
Prednisolone had been tapered off gradually through a one-year period. Four months later from terminating
prednisolone, a follow-up CT showed multiple low-density areas in both kidneys without swelling of the pancreas. Furthermore, 4 months later, laboratory findings showed progressive
renal insufficiency. On admission, BP was 167/77 mmHg, and the bilateral submaxillary glands were swollen. He did not have pretibial
edema. Laboratory findings were as follows. BUN 55.9 mg/dL, Cre 6.17 mg/dL, Amy 65 mg/dL, TP/Alb 9.5/4 g/dL, gamma-gl 43.7%,
IgG/
IgA/
IgM 3,395/112/74 mg/dL,
IgG4 1,460 mg/dL, urinary
protein 1.38 g/day, and 24 hr-Ccr 11.8 mL/min/1.73 m2. Percutaneous renal needle biopsy was conducted. Light microscopic findings demonstrated
tubulointerstitial nephritis (
TIN) and membranous change. Immunofluorescent microscopic findings indicated diffuse deposition of
IgG2 and
IgG4 in the renal interstitium. On the basis of these findings, the condition was diagnosed as IgG4-related
tubulointerstitial nephritis. As
renal insufficiency was progressing,
hemodialysis was started soon after admission and oral
prednisolone was also started at the dose of 0.4 mg/kg (20 mg/day). However, improvement of renal function has not been obtained and
hemodialysis and
prednisolone tapering are still being conducted. This case showed severe
tubulointerstitial nephritis requiring
hemodialysis after a cure for
autoimmune pancreatitis. IgG4-related renal disease rarely needs
hemodialysis. This case indicates that the prognosis of IgG4-related systemic disease is not necessarily good and further accumulation of cases is required.