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Objective:To explore the clinical characteristics and outcomes of type 2 autoimmune pancreatitis (AIP) and compare with type 1 AIP.Methods:Clinical data of the patients diagnosed with type 2 AIP by the International Consensus on diagnostic criteria of AIP at Peking Union Medical College Hospital from January 2001 to December 2022 were retrospectively analyzed, and type 1 AIP patients diagnosed in Peking Union Medical College Hospital from January 1985 to December 2016 were collected as controls. The clinical symptoms, treatments and follow-ups were analyzed.Results:A total of 25 patients with type 2 AIP were included, of which 16 cases (64.0%) were pathologically confirmed cases (13 cases by endoscopic ultrasound puncture, 2 cases by surgery, and 1 case by interventional puncture), and 9 cases (36.0%) were suspected. The average age of onset was 40 years old. Most patients ( n=23, 92.0%) had abdominal pain along with emaciation to a various degree. Among them, 3 cases primarily presented as acute pancreatitis. Two cases were diagnosed after surgery for pancreatic masses. Eighteen cases were complicated with inflammatory bowel disease, including 16 cases with ulcerative colitis, one case with Crohn's disease, and one case with indeterminate colitis. All patients had typical imaging manifestations, including 13 cases (52.0%) with diffuse pancreatic enlargement, 12 cases (48.0%) with focal or multifocal pancreatic lesions, and 5 cases (20.0%) with simultaneous focal pancreatic masses and diffuse enlargement. All patients had normal serum IgG4 levels, anti-neutropil cytoplasmic antibodies (ANCA) positivity rate was 35.3% (6/17), and anti-nuclear antibody (ANA) positivity rate was 29.2% (7/24). Two surgical patients recovered well after surgery, and the other patients all achieved clinical and imaging relief after hormone therapy, and no recurrence was seen during follow-up. Compared with type 1 AIP, type 2 AIP had younger onset age, main manifestation as abdominal pain without jaundice, rare involvement with extra-pancreatic organs, the lesions mainly located in the intestine and normal IgG4 level with statistically significant differences. The recurrence rate of type 2 AIP was lower than that of type 1 AIP (0 vs 16%). Conclusions:Type 2 AIP has different clinical characteristics from type 1 AIP. Due to the lack of specific serum markers, the diagnosis is more difficult. It responds well to glucocorticoids and has a low recurrence rate.
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Objective:To investigate the role of endoscopic ultrasonography (EUS) in differentiating between autoimmune pancreatitis (AIP) and pancreatic cancer (PC).Methods:Data of 133 patients with AIP and 113 patients with PC who underwent EUS because of obstructive jaundice at Peking Union Medical College Hospital from January 2013 to December 2018 were retrospectively analyzed in the study, and were randomly divided into either a derivation sample or a validation sample using 1∶1 allocation according to the random number. In the derivation sample, 10 EUS characteristics were used to construct a prediction model to distinguish between AIP and PC, in which predictors were identified by multivariate stepwise logistic regression analysis and predictive efficacy was evaluated by receiver operating characteristics (ROC) curve analysis. The predictive efficacy was assessed in the validation sample. In view of the subjectivity in the judgment of diffuse/focal hypoechogenicity, 2 prediction models were designed in order to avoid bias.Results:By multivariate stepwise logistic regression analysis, diffuse hypoechogenicity ( OR=591.0, 95% CI: 98.8->999.9, P<0.001) and vessel involvement ( OR=11.9, 95% CI: 1.4-260.2, P=0.023) were identified as statistically significant predictors for distinguishing AIP from PC. EUS characteristics excluding diffuse/focal hypoechogenicity were stepped by logistic regression, which showed that hyperechoic foci/strands ( OR=177.3, 95% CI: 18.7->999.9, P<0.001), pancreatic duct dilation ( OR=60.5, 95% CI: 6.2->999.9, P=0.004), bile duct wall thickening ( OR=35.4, 95% CI: 3.7->999.9, P=0.009), lymphadenopathy ( OR=16.8, 95% CI: 1.7-475.2, P=0.038) and vessel involvement ( OR=22.7, 95% CI: 2.0-725.7, P=0.028) were statistically significant predictors to distinguish the two diseases. Both prediction models were built in the derivation sample, with area under the ROC curve of 0.995 and 0.979 respectively. In the validation sample, sensitivity, specificity, accuracy, positive predictive value and negative predictive value of both prediction models were all >90% by using the optimal cutoff value. Even for discrimination between focal AIP and PC, sensitivity and accuracy of both models were >90%, and specificity, positive predictive value and negative predictive value were all >85%. Conclusion:The 2 prediction models have good differential predictive value, and EUS is a useful tool to differentiate between AIP and PC.
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Objective To evaluate the diagnostic efficacy of narrow band imaging (NBI) international colorectal endoscopic (NICE) classification in distinguishing neoplastic from non-neoplastic colorectal polyps during routine clinical practice. Methods A total of 224 lesions detected by white light colonoscopy by non-expert endoscopists were collected in this retrospective study. Each lesion was assessed by NBI and classified by NICE classification. The results were compared with pathological findings from endoscopic or surgical resected specimen. Results Among these 224 polyps, there were 59 of type 1, 159 of type 2 and 6 of type 3 according to NICE classification. There were 58 non-tumorous and 166 tumorous polyps according to pathological diagnosis. The total diagnostic sensitivity, specificity, positive predictive value, negative predictive value and accuracy of NICE classification for colorectal tumor were 91. 6%, 77. 6%, 92. 1%,76. 3%and 87. 9%, respectively.Diagnostic sensitivity and accuracy in big (＞10 mm in diameter), small (＞5-10 mm in diameter) and mini (≤5 mm in diameter) polyp groups were 100. 0%, 97. 0% and 80. 9%, as well as 95. 7%, 87. 8%, and 83. 3%, respectively. Diagnostic accuracy showed a decreasing tendency on polyp size, without significant difference between the three groups ( P＝0. 694). Conclusion NICE classification with non-magnified NBI is effective in distinguishing neoplastic and non-neoplastic colorectal polyps by non-expert endoscopists and is potentially worth popularizing for routine clinical practice.
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The research on autoimmune pancreatitis (AIP) has progressed rapidly in recent years, causing a lot of clinical concerns. This article reviews the history of the understanding of AIP and the changes in the diagnostic criteria for AIP, with highlights on the new international diagnostic criteria and the diagnostic criteria for type 1 and type 2 AIP. This article also elaborates on the new advances in the treatment of AIP, from hormone therapy to the application of immunosuppressants, points out the issues which need to be considered in clinical practice, and proposes the future research directions for AIP.
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Objective To compare the performance of the revision of Atlanta classification (RAC) and determinant-based classification (DBC) in acute pancreatitis. Methods Consecutive patients with acute pancreatitis admitted to a single center from January 2001 to January 2015 were retrospectively analyzed. Patients were classified into mild, moderately severe and severe categories based on RAC and were simultaneously classified into mild, moderate, severe and critical grades according to DBC. Disease severity and clinical outcomes were compared between subgroups. The receiver operating curve (ROC) was used to compare the utility of RAC and DBC by calculating the area under curve (AUC). Results Among 1120 patients enrolled, organ failure occurred in 343 patients (30.6%) and infected necrosis in 74 patients (6.6%). A total of 63 patients (5.6%) died. Statistically significant difference of disease severity and outcomes was observed between all the subgroups in RAC and DBC (P<0.001). The category of critical acute pancreatitis (with both persistent organ failure and infected necrosis) had the most severe clinical course and the highest mortality (19/31, 61.3%). DBC had a larger AUC (0.73, 95%CI 0.69-0.78) than RAC (0.68, 95%CI 0.65-0.73) in classifying ICU admissions (P=0.031), but both were similar in predicting mortality(P=0.372) and prolonged ICU stay (P=0.266). Conclusions DBC and RAC perform comparably well in categorizing patients with acute pancreatitis regarding disease severity and clinical outcome. DBC is slightly better than RAC in predicting prolonged hospital stay. Persistent organ failure and infected necrosis are risk factors for poor prognosis and presence of both is associated with the most dismal outcome.
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Objective To differentiate colorectal sessile serrated adenoma(SSA)and large hyperplastic polyp(HP)with white light endoscopy and narrow band imaging. Methods Retrospective analysis was performed on the clinical data of 953 patients who underwent endoscopic screening for colorectal cancer at digestive endoscopy center of Peking Union Medical College Hospital from January 2015 to December 2015.Endoscopic features were compared with white light endoscopy and narrow band imaging between SSA and large HP in location, size, Paris classification, surface mucus, NICE classification and varicose microvascular vessel(VMV). Results A total of 28(2.9%)SSAs and 25(2.6%)HPs of diameter not less than 0.5 cm were detected in the total of 953 patients.Statistically significant difference was found in right hemi-colon, diameter not less than 1.0 cm and VMV in two groups(P<0.05). The sensitivities of the above three markers for SSA were 89.3%, 67.9% and 46.4% respectively. The specificities were 64.0%,76.0% and 84.0% respectively. Diagnostic accuracies were 77.4%,71.7% and 64.2% respectively. And the sensitivity, specificity and accuracy of three markers combined(at least two markers)were 75.0%, 88.0% and 81.1%, respectively. Conclusion SSA and large HP could be differentiated with a systemic consideration of lesion location, size, and surface of microvessels by using white light endoscopy and image-enhanced technique.
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Objective To evaluate the effects of infective necrosis (IN) on prognosis in moderately severe or severe acute pancreatitis (AP).Methods According to the revision of Atlanta classification,from January 2001 to January 2015,admitted patients with moderately severe or severe AP were retrospectively analyzed.According to whether with the presence of persistent organ failure (POF) and / or IN,the patients were divided into four groups:group one with weither IN nor POF,group two with IN but without POF,group three with POF but without IN,group four with both IN and POF.The differences in disease severity and prognosis among groups were compared.Logistic regression and Cox proportional hazard regression model were used to analyze the effect of IN on prognosis.Results A total of 375 moderately severe or severe AP patients were enrolled.There were 211,43,90 and 31 patients in group one,two,three and four,respectively.A total of 121 (32.3％) patients with POF,74 (19.7％) patients with IN,and death in 63 (16.8％) patients.The mortality rate in patients with IN was 32.4％ (24/74),and which was 13.0％(39/301) in patients without IN.The mortality rates of group one,two,three and four were 1.9％(4/211),11.6％(5/43),38.9％(35/90) and 61.3％(19/31),respectively;mortality rate was in a trend of increasing,and the difference was statistically significant (x2 =109.672,P＜0.01).Both IN (OR=8.24,95％CI2.09 to 32.46) andPOF (OR=8.31,95％ CI2.48 to 27.87)were independent risk factors of mortality of AP patients (both P＜0.01).Both IN (OR=2.04,95 ％CI 1.19 to 3.48,0.002) and POF (OR=5.25,95％CI 2.36 to 11.65) also were independent risk factors of shortened survival time of AP patients (both P＜0.01).Conclusions IN is an independent risk factor of disease severity and poor prognosis in AP.The prognosis is the worst in AP patients with both POF and IN.
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Objective The type 1 autoimmune pancreatitis is gradually being recognized, but the type 2 AIP is still very rare in Asia.This paper summarizes the clinical characters of type-2 AIP patients in Peking Union Medical College Hospital.Methods From January 2001 to December 2016,all type 2 AIP hospitalized patients who met the ICDC were included in the study.The clinical data, laboratory results and imaging features of all patients were recorded, verified and follow-up.Results Six patients with type 2 AIP were included in the study.The ratio of men and women was 2/1, with an average age of 38.4 years.67.7% (4/6) patients have UC.37.7% (2/6) of patients were asymptomatic.Three patients were diagnosed by pathology.50% (3/6) of patients showed mass of pancreas, and 50% (3/6) of patients showed pancreatic enlargement.Conclusions The clinical manifestations of the type 2 AIP patients in Peking Union Medical College Hospital are the same as those in foreign countries.
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Objective To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China.Methods IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016.All patients were followedup for more than 6 months.The demographic characteristics,symptoms,organ involvements,laboratory examinations and treatment efficacy were evaluated and analyzed.Results A total of 346 patients were finally enrolled,including 230 males (66.5％) and 116 females (33.5％).The mean age of disease onset was (53.8 ± 14.2) years old.The mostly common involved organs were lymph nodes (56.4％) and submandibular glands (52.6％).Other affected organs and manifestations included:swelling of the lacrimal glands (46.5％),autoimmune pancreatitis (38.4％),pulmonary involvement (28.0％),sclerosing cholangitis (25.4％),naso-sinusitis (23.4％),parotid gland swelling (21.7％),retroperitoneal fibrosis (19.9％),large arteries involvement (9.5％),kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9％),skin lesions (6.4％).Rare features consisted of thyroid glands,pituitary glands,gastrointestinal tract,pachymeningitis,pericardium,sclerosing mediastinitis and orchitis.The majority of patients had multi-organ involvement,such as 74.3％ patients with 3 and more,18.2％ and 7.5％ patients with 2 and single organ involvement respectively.The average IgG4-RD responder index (IgG4-RD RI) was 13.21 ±5.70.History of allergy was found in 172 (49.7％) patients.As to the laboratory tests,elevated serum IgG4 levels were confirmed in 285 (94.1％) patients,which was positively correlated with IgG4-RD RI.There were 33.5％ patients receiving monotherapy of glucocorticoid,52.6％ treated with glucocorticoids combined with immunosuppressive agents,4.9％ patients with immunosuppressant only,and 9.0％ patients with mild disease not receiving medication.The majority (336,97.1％) patients improved the above regimens.Conclusion IgG4-RD is a systemic fibro-inflammatory disease with multiple organ involvement.The mostly common involved organs include lymph node,submandibular glands,and pancreas.Glucocorticoids and immunosuppressive agents were effective for IgG4-RD.
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Objective To explore the long-term effect, prognosis and administration of corticosteroid treatment on autoimmune pancreatitis (AIP). Methods Clinical data were analyzed in 13 diagnosed and followed up AIP patients of Peking Union Medicine College Hospital during August 2004 to August 2008. Results Of 13 patients, 12 were males and 1 was female, with a mean age of 58.7 years old, and a mean follow-up of 30 months. Of 11 patients compliated with bile duct disease,biliary stents were placed in 9 patients and already taken out. Corticosteroid treatment was received by cured patients. The average corticosteroid therapeutic time was 9.2 months, 7.9 months in 6 biliary stent placed patients, 13.4 months in corticosteroid treated alone patients, the statistical difference was significant (P = 0. 023). Serum inflammatory parameters normalized range from 5. 3 to 8.8 weeks. After corticosteroid treatment, pancreas enlargement improved in all patients at the first imaging reexamination (1.0 to 11.3 weeks), pancreatic size normalized in 9 patients with an average of 16.6 weeks corticosteroid treatment. No relapsing sign was found with imaging examination during follow-up. Of 8 newly onset diabetes patients, glucose level normalized in 4 patients after corticosteroid treatment. Two patients complicated with autoimmune hepatitis developed early hepatic cirrhosis symptoms at the end of the follow-up. Swollen submandibular gland enlargement relapsed in one patient after corticosteroid withdrawn for six months. Conclsion AIP patients responsed well to corticosteroid treatment. Placement of biliary stent could shorten corticosteroid therapeutic time.Patients with bile duct complications and newly onset diabetes could partially relieve after the corticosteroid treatment, the prognosis of patients with autoimmune hepatitis complications was relatively poor.