The relationship between pancreatic atrophy after steroid therapy and diabetes mellitus in patients with autoimmune pancreatitis

Background/objectivesMany patients with autoimmune pancreatitis (AIP) have an association with diabetes mellitus. It has not been clarified whether steroid therapy for AIP improves or worsens the condition of diabetes mellitus. The aim of this study was thus to investigate the relationship between pancreatic atrophy after steroid therapy and the clinical course of diabetes.MethodsThirty-one AIP patients, who were treated by steroid therapy, were included in this study during December 2005 to March 2013. Pancreatic atrophy 6 months after the beginning of steroid therapy was defined to be present when the width of the pancreatic body was less than 10 mm. The relationships between pancreatic atrophy and patient characteristics as well as the course of diabetes were examined.ResultsSteroid therapy was effective in all treated patients. Pancreatic atrophy was observed in 12 patients and not in 19 patients after the steroid therapy. AIP patients with pancreatic atrophy showed higher incidences of diabetes mellitus (p = 0.001, 9/12 vs. 2/19), diabetes control worsening (p = 0.007, 7/12 vs. 2/17), and new onset of diabetes (p = 0.02, 5/7 vs. 1/18) than those without atrophy. It was not associated with gender, other organ involvement, pattern of pancreas swelling (diffuse/focal), serum IgG4 level, alcohol intake, and pancreatic calcification on CT. Patients with new onset of diabetes needed insulin therapy, even in the maintenance therapy of AIP.ConclusionsAIP patients with pancreatic atrophy after steroid therapy have a high incidence of diabetes mellitus. New onset of diabetes is closely associated with pancreatic atrophy after steroid therapy.     

Does cyst growth predict malignancy in branch duct intraductal papillary mucinous neoplasms? Results of a large multicenter experience

BackgroundCyst growth of BD-IPMNs on follow-up imaging remains a concerning sign.AimsTo describe cyst size changes over time in BD-IPMNs, and determine whether cyst growth rate is associated with increased risk of malignancy.MethodsThis is a retrospective study performed at two high volume tertiary centers. Mean cyst size at baseline (MCSB) and mean growth rate percentage (MGRP) were calculated. Rapid cyst growth was defined as MGRP ≥30%/year. Patient and cyst related characteristics were studied.Results160 patients were followed for a median of 27.4 (12–114.5) months. MCSB was 15.1 ± 8.0 mm. During follow-up, 73 (45.6%) showed any cyst size increase, of which 15 cysts (9.4%) exhibited MGRP ≥30%/year. Rapid cyst growth was not associated with patient or cyst characteristics. Cyst fluid molecular analysis from 101 cysts showed KRAS mutation in 26. Compared to KRAS-negative cysts, neither MCSB (16.0 mm vs. 17.7 mm; p = 0.3) nor MGRP (3.9%/year vs. 5.8%/year; p = 0.7) was significantly different. Eighteen patients underwent surgery; 15 (83%) had LGD, and 3 had advanced neoplasia. Two cysts with LGD and one cyst with advanced neoplasia had MGRP ≥30%/year.ConclusionIncrease in BD-IPMNs size was not associated with the known high risk patient or cyst-related characteristics. Rapid growth of BD-IPMNs was not associated with advanced neoplasia on surgical pathology.     

Autoimmune pancreatitis associated with a large pancreatic pseudocyst that disappeared after corticosteroid therapy: a case report and literature review

A 51-year-old woman was admitted to our department because of upper abdominal pain. The serum IgG4 concentration was elevated, and abdominal computed tomography revealed diffuse enlargement of the pancreas associated with a large cyst, measuring 8 cm in diameter. Endoscopic retrograde cholangiopancreatography revealed narrowing of the main pancreatic duct (from the body to the tail), narrowing of the intrapancreatic bile duct, and dilatation of the bile ducts. The patient was given a diagnosis of autoimmune pancreatitis (AIP) associated with a pancreatic pseudocyst and intrapancreatic bile duct stenosis. Oral steroid therapy resulted in reduced pancreatic swelling, complete disappearance of the pancreatic cyst, and an improvement in biliary stenosis. AIP is rarely associated with pancreatic cyst, and only 13 cases, including ours, have been reported to date. In our patient, intense inflammation apparently led to cyst formation in association with AIP, which responded remarkably to corticosteroid therapy. Correct diagnosis of AIP associated with a pancreatic pseudocyst might save patients from undergoing unnecessary endoscopic and surgical procedures.     

Corticosteroids prevent the progression of autoimmune pancreatitis to chronic pancreatitis

Background/objectivesPatients with autoimmune pancreatitis (AIP) sometimes progress to chronic pancreatitis (CP). We evaluated the ability of corticosteroids to prevent the progression to CP.MethodsWe defined patients with definitive findings of CP (stones in the main pancreatic duct [MPD] or multiple pancreatic calcifications) as having severe calcification (SC). A total of 145 AIP patients were enrolled. We measured the duration between AIP diagnosis and SC development and retrospectively compared the time to SC development between patients with and without steroids. Multivariate analysis for factors associated with SC were performed.ResultsNineteen (13%) patients progressed to SC. Since 95 patients had pancreatic head swelling and SC was found in these patients only, our analysis focused mainly on these at-risk populations. In Kaplan-Meier analysis limited to patients with pancreatic head swelling, the incidence of SC was significantly lower in patients with steroids than in those without (hazard ratio [HR] 0.18, 95% confidence interval [CI] 0.07–0.52; p < 0.001). Multivariate testing of patients with pancreatic head swelling confirmed that steroid therapy was significantly associated with a lower incidence of SC (HR 0.11, 95% CI 0.03–0.34; p < 0.001), while MPD dilation at AIP diagnosis was related to a higher incidence of SC (HR 4.02, 95% CI 1.43–11.7; p = 0.009).ConclusionsCorticosteroids appeared to prevent progression to CP in AIP patients, especially in those with pancreatic head swelling. Patients with both pancreatic head swelling and MPD dilation at diagnosis have a higher incidence of progression to CP. Steroid therapy is suggested for these high-risk cases.     

Patient- and Cyst-Related Factors for Improved Prediction of Malignancy within Cystic Lesions of the Pancreas

Background and Aims: Early diagnosis of cancer in pancreatic cysts is important for timely referral to surgery. The aim of this study was to develop a predictive model for pancreatic cyst malignancy to improve patient selection for surgical resection. Methods: We performed retrospective analyses of endoscopic ultrasound (EUS) and pathology databases identifying pancreatic cysts with available final pathological diagnoses. Main-duct intraductal papillary mucinous neoplasms (IPMNs) were excluded due to the clear indication for surgery. Patient demographics and symptoms, cyst morphology, and cyst fluid characteristics were studied as candidate riskfactors for malignancy. Results: 270 patients with pancreatic cysts were identified and analyzed (41% men, mean age 61.8 years). Final pathological diagnoses were branch-duct IPMN (n = 118, 50% malignant), serous cystadenoma (n = 71), pseudocyst (n = 37), mucinous cyst adenoma/adenocarcinoma (n = 36), islet cell tumor (n = 4), simple cyst (n = 3), and ductal adenocarcinoma with cystic degeneration (n = 1). Optimal cut-off points for surgical resection were cyst fluid carcinoembryonic antigen (CEA) ≥3,594 ng/ml, age >50, and cyst size >1.5 cm. Cyst malignancy was independently associated with white race (OR = 4.1, p = 0.002), weight loss (OR = 3.9, p = 0.001), cyst size >1.5 cm (OR = 2.4, p = 0.012), and high CEA >3,594 (OR = 5.3, p = 0.04). In white patients >50 years old presenting with weight loss and cyst size >1.5 cm, the likelihood of malignancy was nearly sixfold greater than in those patients who had none of these factors (OR = 5.8,95% CI = 2.1-16.1, p = 0.004). Conclusions: Riskfactors other than cyst size are important for determination of malignancy in pancreatic cysts. Exceptionally high cyst fluid CEA levels and certain patient-related factors may help to better predict cyst malignancy and the need for surgical treatment.     

Relapse rate and predictors of relapse in a large single center cohort of type 1 autoimmune pancreatitis: long-term follow-up results after steroid therapy with short-duration maintenance treatment

Background

Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP.

Methods

Patients with more than 2 years of follow-up who met the International Consensus Diagnostic Criteria for type 1 AIP were included. Patients who had undergone pancreatic operations associated with AIP or who lacked sufficient clinical data were excluded.

Results

All 138 patients achieved clinical remission with initial steroid therapy, and 66 (47.8%) experienced relapse during a median 60 (range 24–197) months follow-up. Among the relapsed patients, about 74% (49/66) relapsed within 3 years. About 60% (82/138) had other organ involvement (OOI), most commonly in the proximal bile duct (26.8%). At first diagnosis, OOI, and especially OOI of the proximal bile duct, was a significant independent predictor of relapse (hazard ratio 2.65; 95% confidence interval 1.44–4.89; p = 0.002), according to multivariate analysis. During the follow-up period, 16 (11.6%) patients experienced endocrine/exocrine dysfunction and 32 (23.2%) patients developed de novo pancreatic calcifications/stones. No pancreatic cancer occurred in any patients.

Conclusions

Type 1 AIP has common relapses, and patients with OOI, especially OOI of the proximal bile duct, appear to be at increased risk for relapse. Long-term sequelae, including pancreatic insufficiency and pancreatic calcifications/stones, are common in patients with relapse. To reduce the relapse, longer maintenance treatment may be needed especially for patients at high risk for relapse.

     

Impact of sclerosing dacryoadenitis/sialadenitis on relapse during steroid therapy in patients with type 1 autoimmune pancreatitis

Objectives: Steroids are the first-line drugs for induction of remission in patients with type 1 autoimmune pancreatitis (AIP), and the usefulness of steroid maintenance therapy to prevent relapse has recently been reported. However, even during steroid therapy, a relatively large percentage of patients relapse and the predictive factors for relapse have not yet been elucidated. The aim of this study was to clarify the predictive factors for relapse of AIP patients during steroid therapy.

Materials and methods: The medical records of 76 patients with type 1 AIP with continued steroid therapy after induction of remission were analyzed retrospectively. The relapse rate during steroid therapy was evaluated, and the risk factors for relapse were investigated by univariate and multivariate analysis of clinical factors.

Results: Relapse occurred in 28.9% (22/76) of the patients. The cumulative relapse rates were 10.5% at 1 year, 25.0% at 3 years, 34.9% at 5 years, and 43.0% at 10 years. In multivariate analysis, presence of sclerosing dacryoadenitis/sialadenitis at the time of initial diagnosis of AIP was an independent risk factor for relapse (HR 3.475, p?=?.009). The cumulative relapse rates of patients with sclerosing dacryoadenitis/sialadenitis reached 21.4% at 1 year, 56.0% at 3 years, and 78.0% at 5 years.

Conclusions: Sclerosing dacryoadenitis/sialadenitis was a predictive factor for relapse in type 1 AIP during steroid therapy; in such cases, strict follow-up is necessary with relapse in mind.     

Long-term follow-up of neoplastic pancreatic cysts without high-risk stigmata: how often do we change treatment strategy because of malignant transformation?

*Objective: Patients with potentially premalignant neoplastic pancreatic cysts without high-risk stigmata usually enter a surveillance program. Data on outcomes of such surveillance programs are scarce. We aimed to evaluate the resection rate and malignancy rate during follow-up.

Material and methods: From our prospective database (2006–2015) of patients with pancreatic cysts, we analyzed patients with pancreatic cysts without high-risk stigmata with at least six months follow-up.

Results: In total, 146 patients were followed for a median of 29 months (IQR 13.5–50 months). In 124 patients (84.9%), no changes in clinical or imaging characteristics occurred during follow-up. Thirteen patients (8.9%) developed an indication for surgery after a median follow-up of 25 months (IQR 12–42 months). Two patients did not undergo surgery because of comorbidity, 11 patients (7.5%) underwent resection. Indications for surgery were symptoms (n?=?2), development of a pancreatic mass (n?=?1), a new nodule (n?=?2), thickened cyst wall (n?=?1), pancreatic duct dilation (n?=?3), and/or suspicion of mucinous cystic neoplasm (MCN) (n?=?3). Postoperative histology showed one pancreatic malignancy not originating from the cyst, three mixed type-intraductal papillary mucinous neoplasm (IPMN), one side branch-IPMN, two MCN, one neuroendocrine tumor, one serous cystadenoma, one inflammatory cyst, and one lymphangioma. The highest grade of cyst dysplasia was borderline dysplasia.

Conclusions: Most neoplastic pancreatic cysts without high-risk stigmata at initial presentation show no substantial change during 1–4-year follow-up. Only 7.5% of patients underwent surgery and less than 1% of patients developed pancreatic malignancy. This indicates that additional markers are needed to tailor treatment of pancreatic cysts.     

Characteristics of pancreatic diabetes in patients with autoimmune pancreatitis

OBJECTIVE: Although patients with autoimmune pancreatitis (AIP) tend to have concurrent diverse disorders, very few studies have focused on diabetes mellitus (DM) coexisting with AIP. METHODS: In total 102 AIP patients with DM were divided into three groups. Those with DM before the onset of AIP were labeled group A (n = 35), those who developed DM and AIP simultaneously were labeled group B (n = 58) and those who developed DM after steroid therapy for AIP were labeled group C (n = 9). The characteristics of DM among the three groups were evaluated. RESULTS: No significant differences were noted in the age of DM onset among the three groups. However, the mean duration of DM was significantly longer in group A (8.7 years) than in groups B and C. AIP developed 6.8 years after DM onset in group A, whereas it developed 1.8 years after steroid therapy in group C. Group A had the highest rate (25.7%) of family members with a history of AIP. Levels of serum albumin, total cholesterol and triglyceride were significantly lower in group A. No correlations were found between glycated hemoglobin and benzoyl‐tyrosyl para‐aminobenzoic acid. Hypoglycemia was observed in 20% of patients under insulin therapy. Most of them were habitual drinkers and received no pancreatic enzymes. Group A showed a high prevalence of retinopathy, nephropathy and macrovascular disorders than group B. CONCLUSION: Aspects of AIP‐associated pancreatic diabetes were clarified. AIP‐associated DM must be controlled by a full assessment of the pancreatic endocrine and exocrine function.     

Chronic obstructive pancreatitis due to a pancreatic cyst in a patient with autosomal dominant polycystic kidney disease

Background—Autosomal dominant polycystic kidneydisease, the most frequent inherited polycystic disease, is a systemicdisorder characterised by the development of numerous and bilateralkidney cysts leading to chronic renal failure. Extrarenal cysts arelocated mainly in the liver but also in various organs including thepancreas. To our knowledge, complications of pancreatic cysts in thisdisease have never been reported.
Patient—The first case of painful chronicobstructive pancreatitis due to a true pancreatic cyst in a patientwith autosomal dominant polycystic kidney disease is reported.Abdominal transparietal and endoscopic ultrasonography, computedtomography, and endoscopic retrograde cholangiopancreatography showed acystic lesion in the body of the pancreas associated with upstreamdilatation of the main pancreatic duct. Intraoperative ultrasonographybefore and after cyst fluid aspiration, and pancreatography andpathological examination of the resected distal pancreas confirmed thatboth main pancreatic duct enlargement and chronic pancreatitis were caused by a benign cyst.
Conclusion—Chronic obstructive pancreatitisshould be added to the extrarenal complications of autosomal dominantpolycystic kidney disease.

Keywords:chronic obstructive pancreatitis; pancreatic cyst; autosomal dominant polycystic kidney disease; distal pancreatectomy

     

Appropriate steroid therapy for autoimmune pancreatitis based on long-term outcome

Objective. Because autoimmune pancreatitis (AIP) responds well to corticosteroids, many AIP patients are given this treatment. However, there is no consensus on the indications, dose, or duration of steroid treatment. The aim of this study was to establish the most appropriate steroid therapy regimen. Material and methods. We retrospectively reviewed morphological and serological improvement after steroid therapy and long-term outcome including relapse in 41 AIP patients who were given steroid therapy and were prospectively followed-up for more than 1 year. Results. All patients responded to steroid therapy, which was given because of bile duct stenosis secondary to sclerosing cholangitis in 34 AIP patients. Pancreatic enlargement normalized within one month; however, 13 patients had incomplete resolution of pancreatic duct narrowing, and 14 patients had incomplete resolution of bile duct stenosis. There was no correlation between the degree of morphological improvement and the initial prednisolone dose (30 mg and 40 mg/day). In 58% of 19 patients, serum IgG4 elevation failed to normalize. Glucose intolerance improved in 38% of the 21 patients with diabetes mellitus. Nine patients who had complete morphological and serological resolution, stopped their medication, and none have relapsed. Thirty-two patients continued maintenance therapy, and 4 of these patients suffered relapse. Conclusions. The indications for steroid therapy in AIP patients include bile duct stenosis caused by sclerosing cholangitis and other systemic diseases, such as retroperitoneal fibrosis and diabetes mellitus. We recommend that oral prednisolone be used at an initial dose of 30 mg/day; maintenance therapy is required in cases without complete morphological and serological resolution.     

Clinical factors predictive of spontaneous remission or relapse in cases of autoimmune pancreatitis

BACKGROUND: The rates of spontaneous remission and relapse of autoimmune pancreatitis (AIP) are not known. OBJECTIVE: To study the clinicopathologic factors predictive of remission and relapse in cases of AIP. DESIGN: Retrospective study. PATIENTS: Of the 20 patients with AIP, complete response to steroid therapy was recognized in 12 patients, and the remaining 8 patients improved without steroid therapy. Seven patients experienced a relapse. RESULTS: Patients who were seronegative for immunoglobulin (Ig) G4, had no obstructive jaundice, no diabetes mellitus, no swelling of the duodenal papilla, negative staining of the duodenal papilla for IgG4, and focal pancreatic swelling showed a greater tendency toward spontaneous remission (P < .05). The results of multivariate analysis revealed that negative staining of the duodenal papilla for IgG4 was the only independent predictor of spontaneous remission of AIP (odds ratio [OR] 1.395, P = .0304). Seropositivity for IgG4, diffuse swelling of the pancreas, and the presence of stricture in the lower part of the bile duct were significantly associated with a relapse of AIP (P < .05) according to the results of univariate analysis, whereas the results of multivariate analysis revealed only diffuse pancreatic swelling as an independent predictor of a relapse of AIP (OR 26.197, P = .0331). CONCLUSIONS: Endoscopic findings are of useful prognostic value, because patients with AIP and with negative staining of the duodenal papilla for IgG4 appeared to have a higher frequency of remission without steroid therapy. Patients with AIP and with diffuse pancreatic swelling were found to be at an increased risk of relapse after the initial steroid administration.     

Fine needle sclerotherapy as a new effective therapeutic approach for nonparasitic splenic cysts: A case series

BackgroundNonparasitic splenic cysts are rare. Until now, surgical intervention has been the standard therapy of symptomatic splenic cysts.AimsWe herein present a retrospective analysis of an approach using percutaneous ultrasound-guided fine needle aspiration and sclerotherapy.MethodsOut of 88,151 ultrasound reports, we identified 138 patients who presented with splenic cysts. A single splenic cyst was found in 88% (mean size 28.9 mm). Twelve patients underwent percutaneous therapy of symptomatic splenic cysts. They were younger, had larger splenic cysts and more often cyst internal echoes than the 126 untreated patients (all p < 0.0001).ResultsInitial sclerotherapy was performed with polidocanol 1% in 9 patients and with NaCl 10% in 2 patients. One hemorrhagic cyst was only purged. Serious adverse events were not noted. Eight patients had to undergo 1–11 further percutaneous cyst therapies. 15 of these 30 reinterventions were cyst aspiration therapies, only, and 11 of them were sclerotherapies with NaCl 10%. Four patients were readmitted to hospital for cyst retreatment. After 57 ± 43 months of follow-up, cyst size significantly decreased (p < 0.0001). Only two patients had a complicated course of cyst therapy.ConclusionsPercutaneous ultrasound-guided sclerotherapy is a new approach for symptomatic splenic cysts. In most patients, cyst size and symptoms can be significantly reduced during one hospital stay.     

Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.

BACKGROUND: Steroid therapy is currently common treatment for autoimmune pancreatitis (AIP); however, indications of steroid therapy have yet to be established, and the clinical course after steroid therapy is unknown. METHODS: A total of 23 patients with AIP were subdivided into 4 groups according to the initial treatments undertaken. They were treated with pancreatoduodenectomy on suspicion of pancreatic tumor in 6 patients, choledochoduodenostomy with pancreatic biopsy in 4 patients, supportive therapy in 3 patients, and steroid therapy in 10 patients. Clinical course of AIP in each group was examined. RESULTS: Prognosis of the AIP patients is almost good except for the 2 patients who progressed to pancreatic insufficiency after resection. Two patients without jaundice improved spontaneously. Steroid therapy was effective in all patients treated, but pancreatic atrophy developed in 5 of these patients. Steroid therapy improved insulin secretion and glycemic control in 4 of 7 diabetes mellitus (DM) patients. CONCLUSION: To avoid futile surgery, in relatively elderly male patients with obstructive jaundice suggestive of pancreatic carcinoma, preoperative clinical suspicion of AIP is mandatory. Indications of steroid therapy for AIP are thought to be obstructive jaundice due to stenosis of the bile duct, other associated systemic autoimmune, and DM coincidental with AIP.     

Exocrine and Endocrine Pancreatic Function in 21 Patients Suffering from Autoimmune Pancreatitis before and after Steroid Treatment

Background/Aim: Autoimmune pancreatitis (AIP) responds rapidly and dramatically to steroid therapy. The aim of this study was to evaluate pancreatic exocrine and endocrine function in patients suffering from AIP both before and after steroid therapy. Patients and Methods: Fecal elastase 1 and diabetes were evaluated before steroid therapy and within 1 month of its suspension in 21 patients (13 males and 8 females, mean age 43 ± 16.5 years) diagnosed as having AIP between 2006 and 2008. Results: At clinical onset, fecal elastase 1 was 107 ± 126μg/g stool.Thirteen patients (62%) showed severe pancreatic insufficiency (<100 μg/g stool), 4 (19%) had mild insufficiency (100–200 μg/g stool), while 4 (19%) had normal pancreatic function (1200 μg/g stool). Before steroids, diabetes was diagnosed in 5 patients (24%), all of whom had very low levels of fecal elastase 1 (<19 μg/g stool). Following steroids, fecal elastase 1 increased in all patients (237 8 193 μg/g stool) and observed levels were significantly higher than those seen before steroids (p = 0.001). Conclusions: Patients suffering from AIP display exocrine and/or endocrine pancreatic insufficiency at clinical onset. These insufficiencies improve after steroid therapy.     

Usefulness of Positron Emission Tomography in the Evaluation of Distribution and Activity of Systemic Lesions Associated with Autoimmune Pancreatitis

Background/Aims: Autoimmune pancreatitis (AIP) is an lgG4-related systemic disease often accompanied with a variety of lesions outside of the pancreas and is treated with steroid therapy. The aim of this study is to analyze the usefulness of positron emission tomography with ¹⁸F-fluorodeoxyglucose (FDG-PET) in the evaluation of distribution and activity of systemic lesions of AIP during steroid therapy. Methods: Eleven cases of AIP had their FDG-PET images evaluated before and 3 months after steroid therapy and another 2 cases only before therapy. AIP activity was determined by the level of serum markers, IgG and lgG4, and compared with findings of PET. Results: In all 13 cases of AIP, a moderate to intense level of FDG accumulation was recognized in the pancreatic lesion before steroid therapy. Of 13 patients, 11 (84.6%) showed FDG accumulation in the multiple organs, such as mediastinal and other lymph nodes, salivary gland, biliary tract, prostate, and aortic wall. In 11 patients who underwent PET before and after steroid therapy, FDG accumulation was diminished in almost all systemic lesions, with a mean of maximum standardized uptake value (SUV_max) in the pancreatic lesion from 5.12 to 2.69. Similar to the SUV level, serum IgG and lgG4 were decreased in most of the cases after steroid therapy. Conclusions: FDG-PET is an effective modality to evaluate the response of steroid therapy and the distribution and activity of various systemic lesions of AIP.     

Autoimmune pancreatitis

Abstract

Background. Autoimmune pancreatitis (AIP) is a relatively newly recognized type of pancreatitis that is characterized by diffuse or focal swelling of the pancreas due to lymphoplasmacytic infiltration and fibrosis of the pancreatic parenchyma. Material and methods. A PubMed literature search was performed using the keywords “autoimmune pancreatitis”. Sometimes, bibliographies were cross-referenced and related article searches were performed once an article of interest was identified. Results. Pathologically, AIP shows narrowing of the pancreatic ducts and the intrapancreatic portion of the common bile duct. Obstructive jaundice is a common symptom at presentation, and pancreatic cancer represents an important clinical differential diagnosis. In late stages of the disease, the normal pancreatic parenchyma is often replaced by large amounts of fibrosis. Histologically, there seem to be two subtypes of the disease–one showing infiltration with IgG4-positive plasma cells but lacking granulocytic epithelial lesions (GELs), the other showing GELs but lacking strong IgG4 positivity. AIP is in at least some instances the pancreatic manifestation of a clinicopathological entity of IgG4-related systemic sclerosing disease. On the basis of pancreatic imaging, together with serological measurement of IgG4 and evaluation of other organ involvement, many AIP patients can be identified. The remaining patients require further diagnostic work-up. In these patients, pancreatic core needle biopsy and, as AIP responds to steroid treatment, also a trial with steroids, can help to differentiate AIP from pancreatic cancer. Outlook and discussion. This review presents the pathological, radiologic and laboratory findings of AIP. Moreover, the treatment and pathogenesis are discussed.     

Diagnostic criteria for autoimmune pancreatitis

Autoimmune pancreatitis (AIP), a particular type of pancreatitis, is thought to have an autoimmune etiology; it is recognized as a distinct entity worldwide. AIP has many characteristic clinical, serologic, morphologic, and histopathologic features. In the absence of a diagnostic serologic marker for AIP, AIP should be diagnosed on the basis of combination of characteristic findings. AIP responds dramatically to steroid therapy; thus, accurate diagnosis of AIP can avoid unnecessary laparotomy or resection. It is important not to misdiagnose pancreatic cancer as AIP, and not to misdiagnose AIP as pancreatic cancer. Currently, 3 sets of major diagnostic criteria for AIP have been proposed in Japan, Korea, and the United States. The Japanese criteria are based on the minimum consensus features of AIP and aim to avoid misdiagnosis of malignancy. When response to steroid therapy is added to the criteria, the diagnostic sensitivity is increased. However, the use of a steroid trial in cases where differentiation from malignancy is an issue may result in delaying pancreatic cancer surgery, which could lead to cancer progression in several cases. Thus, given that AIP is an IgG4-related systemic disease, an additional criterion can be recommended to the Japanese diagnostic criteria: IgG4-immunostaining of biopsied extrapancreatic lesions such as the major duodenal papilla, the bile duct, or the minor salivary gland. It is also time for an international consensus on AIP.     

The M-ANNHEIM-AiP-Activity-Score is useful for predicting relapse in patients with type 1 autoimmune pancreatitis

Background/ObjectivesProper assessment of disease activity and prediction of relapse are crucial for the management of autoimmune pancreatitis (AIP). The M-ANNHEIM-AiP-Activity-Score (MAAS) has been proposed to determine disease activity and predict relapse in German and Swedish patients with AIP. MAAS is calculated using six categories: pain report, pain control, exocrine insufficiency, endocrine insufficiency, imaging, and complications. This study aimed to clarify the usefulness of MAAS to predict relapse in Japanese patients with type 1 AIP.MethodsWe retrospectively analyzed 117 patients with type 1 AIP undergoing initial and maintenance steroid treatments at our institute between April 2006 and March 2021. AIP was diagnosed according to the Japanese Diagnostic Criteria for AIP 2018. We examined the association of MAAS with relapse during and after maintenance treatment.ResultsMAAS (median, 8 points) at the start of the initial treatment was reduced after treatment (median, 4 points; P < 0.001). A MAAS ≥11 points at the start of the initial treatment was associated with relapse. The initial treatment-induced reduction of MAAS<60% was more frequent in patients with relapse (75.0%) than in patients without relapse (37.6%; P = 0.007). MAAS at the start of maintenance treatment was higher for patients with relapse (median, 5 points) than that for patients without relapse (median, 4 points; P = 0.007). MAAS ≥4 points at the start of maintenance treatment was associated with subsequent relapse.ConclusionsMAAS is useful for predicting relapse in patients with type 1 AIP undergoing maintenance therapy.     

A giant biliary cyst of Todani IA in a young woman: A case report

Rationale:Biliary cysts (BC) are rare dilatations of various parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. Total cyst excision and Roux-Y hepaticojejunostomy is the treatment method of choice in most patients. In this paper, a novel surgical treatment with the use of internal biliary and pancreatic catheters was presented.Patient concerns:A 21-years-old woman with a giant choledochal cyst of Todani IA type presenting with abdominal pain, nausea, fever and palpable abdominal mass. It had been previously drained as a misdiagnosed pancreatic cyst in another hospital.Diagnosis:A very high amylase level (107140,0 U/l) in drain fluid was noted in laboratory tests. Endoscopic retrograde cholangiopancreatography revealed a biliary cyst located in the distal common bile duct and a pancreaticobiliary anomaly was suggested. A cholangiography per catheter inserted to the biliary cyst showed a large round contrast-filled cyst. A cholangiography following cyst decompression revealed a very long, tortuous bile duct entering the duodenum.Interventions:Cholecystectomy, cyst resection, Roux-Y hepaticojejunostomy, and implantation of catheters into pancreatic and bile duct were performed. The postoperative course was uneventful and she was discharged on 12th day without any complications. Histopathology revealed a cyst wall partially lined with biliary-type and mucinous epithelium, with dysplasia ranging from low to high grade (biliary intraepithelial neoplasia, high grade), without invasion.Outcomes:The biliary and pancreatic catheters were removed during endoscopic retrograde cholangiopancreatography 8 weeks following surgery without any complications. Fourteen months later, the patient reported good health.Lessons:Diagnosis of the abdominal cyst should be very precise in order to avoid misdiagnosis and inadequate management. The early diagnosis and proper treatment of BC are needed in order to avoid serious complications. The cholangiocarcinoma is the most dangerous potential complication of BC due to dysplasia within the cyst wall as in our young female patient.