BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is an immune -mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large panEuropean cohort and study the effectiveness of current treatment regimens. METHODS: We retrospectively analyzed adults diagnosed since 2005 with type 1 or not -otherwisespeci fi ed AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using speci fi c diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary end point was complete remission, de fi ned as the absence of clinical symptoms and resolution of the index radiologic pancreatic abnormalities attributed to AIP. RESULTS: We included 735 individuals with AIP (69% male; median age, 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, whereas 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher ( double dagger 0.4 mg/kg/day) corticosteroid doses were no more effective than lower ( < 0.4 mg/kg/day) doses (odds ratio, 0.428; 95% con fi dence interval, 0.054 - 3.387) and neither was a starting dose duration > 2 weeks (odds ratio, 0.908; 95% con fi dence interval, 0.818 - 1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (odds ratio, 0.639; 95% con fi dence interval, 0.427 - 0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid -tapering duration, induction treatment duration, and total cumulative dose. CONCLUSIONS: Patients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens.
Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment
Carrara, Silvia;
2024-01-01
Abstract
BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is an immune -mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large panEuropean cohort and study the effectiveness of current treatment regimens. METHODS: We retrospectively analyzed adults diagnosed since 2005 with type 1 or not -otherwisespeci fi ed AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using speci fi c diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary end point was complete remission, de fi ned as the absence of clinical symptoms and resolution of the index radiologic pancreatic abnormalities attributed to AIP. RESULTS: We included 735 individuals with AIP (69% male; median age, 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, whereas 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher ( double dagger 0.4 mg/kg/day) corticosteroid doses were no more effective than lower ( < 0.4 mg/kg/day) doses (odds ratio, 0.428; 95% con fi dence interval, 0.054 - 3.387) and neither was a starting dose duration > 2 weeks (odds ratio, 0.908; 95% con fi dence interval, 0.818 - 1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (odds ratio, 0.639; 95% con fi dence interval, 0.427 - 0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid -tapering duration, induction treatment duration, and total cumulative dose. CONCLUSIONS: Patients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.