| Part I.Long-term outcome after non-operative treatment of type 1 autoimmune pancreatitis and analysis of associated factorsObjective: Autoimmune pancreatitis(AIP)is a relatively rare form of pancreatitis,with type 1 predominating in China.In general,patients with AIP can achieve significant relief of clinical symptoms,laboratory tests and imaging manifestations after steroid treatment,with a good short-term outcome.However,the research history of AIP is relatively short and there are still different views on its long-term clinical outcome.The aim of this study was to review a large single-centre sample of cases,to summarise information on the long-term outcome of patients with type 1 AIP who received non-operative treatment,and analyse the associated risk factors.Methods: The clinical data of type 1 AIP patients who received non-operative treatment in the autoimmune pancreatitis database of our center were retrospectively analyzed,and the long-term follow-up and prognosis data were collected.The long-term outcome was divided into six aspects: recurrence,endocrine insufficiency,exocrine insufficiency(replaced by steatorrhea),pancreatic duct stones(including pancreatic calcification),occurrence of pancreatic and extrapancreatic malignant tumors,adverse reactions to steroid therapy and related death events.COX proportional hazard model was used to predict risk factors.Results: A total of 253 patients with type 1 AIP treated non-operatively were included,with a male to female ratio of 5.49:1 and a mean age of onset of(57.9 ± 12.2)years.After a median follow-up period of 45 months(17.5-84 months),long-term follow-up data were finally obtained in 233 patients,of whom 35.2% presented with recurrence,16.3% with endocrine insufficiency,4.3% with steatorrhea,5.2% with pancreatic duct stones or enlargement compared to the previous one,and 5.6% with malignancy,of whom 3 were diagnosed at the same time as AIP and no pancreatic cancer was seen.There were 0.8% of patients who died from organ failure associated with AIP.Adverse reactions to steroid therapy occurred in 3.5% of the 172 patients who received hormone therapy.The independent risk factors for recurrence included combined extra-pancreatic organ involvement(HR=2.409,p<0.001),Ig G4 at follow-up two times higher than the upper limitof normal(HR=1.760,p=0.040),and time to first steroid treatment more than 6 months(HR=0.443,p=0.002)were protective factors for various adverse prognoses.The independent risk factor for endocrine insufficiency was the combination of extra-pancreatic organ involvement(HR=3.130,p=0.003),and a first diagnosis of Ig G4 level two times higher than the upper limit of normal(HR=0.385,p=0.012)was a protective factor.History of diabetes(HR=4.411,p=0.032)and recurrence(HR=8.507,p=0.006)were independent risk factors for steatorrhea.History of steatorrhea(HR=16.914,p=0.002)was an independent risk factor for pancreatic duct stones.Alcohol consumption(HR=4.345,p=0.049)was an independent risk factor for the development of malignancy.History of malignancy was an independent risk factor for death(HR=6.244,p=0.006).Conclusion: Recurrence and endocrine insufficiency are more common in long-term follow-up of type 1 AIP,especially in patients with extra-pancreatic organ involvement need to strengthen follow-up.Initial steroid maintenance therapy should be more than 6 months to reduce the risk of recurrence.Part II.Long-term outcome after operative treatment of type 1 autoimmune pancreatitisObjective: The differential diagnosis of autoimmune pancreatitis is difficult as the clinical symptoms and imaging presentations are similar to those of pancreatic malignancies.In actual clinical work,there are cases where patients with AIP are suspected or misdiagnosed as malignant cancer and undergo surgery.The aim of this study,based on a single-centre case data,was to assess the clinical characteristics and long-term outcome of patients with type 1 AIP who underwent operative treatment.Methods: A retrospective case-control study was used to collect patients who underwent partial pancreatectomy for suspected pancreatic cancer with a final pathologically confirmed diagnosis of type 1 AIP,based on a single-centre,long-term follow-up AIP database.They were compared with patients with type 1 AIP who underwent non-surgical treatment to assess the usefulness of routine clinical diagnostic tests received by patients with AIP.To further report on the long-term outcome of patients with type 1 AIP after operative treatment.Results: A total of 38 patients were included,with a mean age of(55.8 ± 9.2)years and 92% were male.Symptoms at presentation included jaundice(58%),abdominal pain(21%)and weight loss(8%).Postoperative pathology diagnosed one patient with type 1 AIP combined with poorly differentiated pancreatic ductal adenocarcinoma,five patients with type 1 AIP combined with pancreatic intraepithelial neoplasia and the others with type 1 AIP.Compared to patients who received non-operative treatment,patients who underwent operative treatment had less prior history of acute pancreatitis(31% vs.13%,p=0.036),less history of diabetes(26% vs.8%,p=0.027),and higher prevalence of smoking(32% vs.53%,p=0.024)and BMI(21.6 vs.23.1,p=0.002).Localised enlargement of the pancreas was more common in the operative group(38% vs.95%,p<0.001),and patients in the non-operative group had higher levels of eosinophils(4.0 vs.1.8,p<0.001),serum globulin(34.3 vs.31.4,p=0.040)and Ig G4(10.7 vs.5.4,p=0.028)than in the operative group.With a median follow-up of 78 months(53-121),long-term follow-up information was obtained for 33 patients,and patients with AIP combined with pancreatic ductal adenocarcinoma were lost to follow-up.Among them,18.2% recurred,42.4% had endocrine insufficiency,24.2% had steatorrhea,and 6.1% had pancreatic ductal calculi formation or were larger than before.12.1% had extra-pancreatic malignancies(including liver,bladder,oral cancer,and lymphoma),and 15.1% died during the follow-up period,with no AIPrelated deaths.Conclusion: Only a few patients with AIP will benefit from operative treatment,and preoperative diagnosis and differential diagnosis should be enhanced to avoid operation where possible.If treated with surgery,attention should be paid to recurrence,endocrine insufficiency,exocrine insufficiency and malignancy during follow-up.If a relapse occurs,it is safe to apply steroids after excluding contraindications. |
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