Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis

Abstract

Objective Autoimmune pancreatitis (AIP) is a treatable form of chronic pancreatitis that has been increasingly recognised over the last decade. We set out to better understand the current burden of AIP at several academic institutions diagnosed using the International Consensus Diagnostic Criteria, and to describe long-term outcomes, including organs involved, treatments, relapse frequency and long-term sequelae. Design 23 institutions from 10 different countries participated in this multinational analysis. A total of 1064 patients meeting the International Consensus Diagnostic Criteria for type 1 (n=978) or type 2 (n=86) AIP were included. Data regarding treatments, relapses and sequelae were obtained. Results The majority of patients with type 1 (99%) and type 2 (92%) AIP who were treated with steroids went into clinical remission. Most patients with jaundice required biliary stent placement (71% of type 1 and 77% of type 2 AIP). Relapses were more common in patients with type 1 (31%) versus type 2 AIP (9%, p<0.001), especially those with IgG4-related sclerosing cholangitis (56% vs 26%, p<0.001). Relapses typically occurred in the pancreas or biliary tree. Retreatment with steroids remained effective at inducing remission with or without alternative treatment, such as azathioprine. Pancreatic duct stones and cancer were uncommon sequelae in type 1 AIP and did not occur in type 2 AIP during the study period. Conclusions AIP is a global disease which uniformly displays a high response to steroid treatment and tendency to relapse in the pancreas and biliary tree. Potential long-term sequelae include pancreatic duct stones and malignancy, however they were uncommon during the study period and require additional follow-up. Additional studies investigating prevention and treatment of disease relapses are needed.

IgG4-related sclerosing cholangitis: all we need to know

“…In Asian countries including Japan, after achieving remission, high-dose steroid administration is followed by a slow taper over several months to a low maintenance dose (equivalent of 2.5-10 mg of prednisone per day), which is continued for at least 1-3 years [82,83]. In contrast, in the West, steroid therapy is completely withdrawn after the successful induction of remission and the tapering period (typically 5 mg each weak) [14,84,85]. A retrospective study of type 1 AIP showed that the relapse rate was slightly higher in patients with maintenance steroids than in those with no maintenance therapy [86].…”

Autoimmune Pancreatitis – Diagnosis, Management and Longterm Follow-up

“…is paper adds to the available data from the United States [5,10,12,13] and Europe [14][15][16] and supports the fact that this condition is not con ned to certain geographical regions in the world. ere has been a case series from the UK in the literature [17] and patients from the UK have been reported as part of large multicentre series [18]. ough our rst case was diagnosed in 2005, there has been increased awareness of this condition, which has resulted in a gradual increase in the diagnosis.…”

“…Relapse is common (40-53% of patients) and is predicted by the presence of proximal extra-and intrahepatic biliary stricturing and HLA DQβ1 [18]. ere is limited data on the role of immunosuppression in patients with AIP as radiological and clinical improvement may also occur without any treatment and there is no placebo controlled trial available to con rm the bene t of steroids [44].…”

Autoimmune Diseases of Digestive System

“…Минималь-но эффективной дозой преднизолона при АИП является 35-40 мг/сут (0,6-1 мг/кг веса тела пациента). Клиниче-ский эффект наступает обычно через 2-3 нед от начала лечения, нормализация лабораторно-инструментальных данных -через несколько недель-месяцев [18].…”