Primary biliary cholangitis （PBC） is a chronic progressive autoimmune liver disease that is often comorbid with other connective tissue diseases （CTDs）， and such comorbidity can significantly alter the natural course or clinical phenotype of PBC or CTDs， limiting available therapeutic drugs and complicating clinical decision-making. Due to the involvement of the interdisciplinary subjects of hepatology， rheumatology， and clinical immunology and a paucity of large-scale cohort data and in-depth basic research， there is a limited understanding of such comorbidity in clinical practice， which increases the complexity of clinical diagnosis and treatment. This article summarizes the comorbidity of PBC with common CTDs such as Sjögren’‍s syndrome， systemic sclerosis， systemic lupus erythematosus， and idiopathic inflammatory myopathies， and analyzes related immune mechanisms， clinical manifestations， diagnostic challenges， treatment strategies， and prognosis. It is expected to establish PBC-CTD comorbidity cohorts through future multidisciplinary collaborations， focus on genetic background， immune mechanisms， and multi-omics approaches， elucidate pathogenesis and novel therapeutic targets， and improve the prognosis of patients by optimizing treatment strategies through precision medicine and artificial intelligence.

Objective To explore the effectiveness and safety of limited endoscopic sphincterotomy plus endoscopic papillary balloon dilation (ESBD)for patients with large common duct stones and periampul-lary diverticula (PAD).Methods Data of 40 patients with large common duct stones (diameter ≥10 mm) and PAD who underwent ESBD were retrospectively reviewed.The clinical feature,operation method,suc-cess rates of stone removal and early complications rates during hospitalization were evaluated according to PAD subtypes.Results The stone removal rate in first session was 90.0%(36 /40,with a median time of 29 minutes per procedure.Three patients underwent a second procedure to remove residual stones.The over-all stone removal rate was 97.5% (39 /40).The early complications rate was 15%(6 /40),including mild pancreatitis in two cases(5%),hyperamylasemia in two (5%),postoperative late bleeding in two (5%), which were cured with conservative treatment.No perforation,massive hemorrhage or death occurred.No significant differences in success stone removal rate and early complication rate were found between PAD sub-types.Conclusion ESBD is an effective and safe procedure for removing choledocholithiasis in patients with PAD,regardless of PAD subtypes.

Objective To evaluate the efficacy of emergency endoscopic intervention in acute obstructive suppurative cholangitis (AOSC) complicated with septic shock.Methods A total of 54 patients with AOSC and septic shock who underwent therapeutic emergency ERCP were included in this retrospective study,and were evaluated by the shock index (SI).Results ERCP was performed for all patients in 24hours after hospitalization,and the average ERCP operation time was 23.8 ± 12.5 min.All 54 patients underwent EST,46 of whom received ENBD,7 biliary stenting and 1 transferred to surgery due to bleeding.The post-ERCP mortality rate was 0,and the complications included 1 case of pancreatitis and 2 cases of pneumonia.The positive rate ofGram-Negative bacillus before ERCP was 46.9％ (15/32).The SI before ERCP was 1.250 ±0.200,which decreased to 0.950 ±0.119 at 2hr after the procedure (P ＜0.001 ),and decreased further to 0.598 ± 0.099 ( P ＜ 0.001 ) at 24 hours after ERCP.Conclusion Therapeutic emergency ERCP is of great importance in the treatments for AOSC complicated with septic shock.

Objective To summarize and analyze the clinical characteristic of blue rubber bleb nevus syndrome (BRBNS).Methods The clinical data of four cases treated since 2001 and 30 BRBNS cases reported by domestic literature were retrospectively analyzed.The clinical manifestation,family history,endoscopy and imageology examination,site of lesions,treatment and follow up were analyzed.Results The male to female ratio was 1.8∶1 and median age was 19.5 years.A total of 33 cases (97.1％) presented with gastrointestinal bleeding,median age of gastrointestinal bleeding detected was 9.0 years.Among the 33 cases,anemiawas found as the primary symptom in nine cases (27.3％),and one case complicated with intussusception and intestinal necrosis accompanied with abdominal pain.Two cases have family history.Gastroscopy (85.3 ％) and colonoscopy(73.5 ％) were mainly examinations for detection.Lesions mainly involved skin (100.0％) and digestive tract (97.1％),and the locations of the lesion in digestive tract was stomach (64.7％),small intestine (64.7％),colon (58.8％),esophagus (29.4％).Treatment methods included symptomatic treatment,endoscopic therapy and surgery.Banding ligation and polypectomy resection were common endoscopic therapies.Gastrointestinal bleeding did not recur in six cases with endoscopic therapy and four cases receiving surgery during short-term follow up.Conclusions BRBNS lesions mainly involve skin and digestive tract,mostly complicated with gastrointestinal bleeding.For gastrointestinal bleeding,so far endoscopic therapy and surgery are the effective therapies.

Objective To evaluate and analyse the efficacy and safety of endoscopic papillectomy for tumors of major duodenal papilla.Methods The clinical data of thirty-four patients with tumors of major duodenal papilla who were treated by endoscopic papillectomy were retrospectively reviewed,and the clinical outcome was summarized.Results The success rate of endoscopic papillectomy was 94.12％ (32/34),and the complete resection was 66.67％ (20/30).Short-term complications occurred in 10 cases,including postoperative gastrointestinal bleeding in 7cases and postoperative pancreatitis in 3 cases.Five cases need further surgical intervention for the reason that tumor was too deep in 2 cases,bile duct was invaded in 2 cases and conservational medication was unsuccessful in 1 case.Thirty-three cases were pathologically diagnosed as low grade intraepithelial neoplasia (LGIN),17 cases high grade intraepithelial neoplasia (HGIN),1 case carcinoid and 3 cases adenocarcinoma.The recurrence rate was 23.33 (7/33).tumor recurrence rate of HGIN was higher than that of LGIN(42.86％ VS10％,P =0.078).Conclusion Endoscopic papillectomy is an effective method for treating tumors of major duodenal papilla,however,hemorrhage is the main postoperative complication,the recurrence rate is higher in HGIN group.

Objective To evaluate the technique of transpancreatic septum precut for cannulation of inaccessible common bile duct in endoscopic retrograde cholangiopancreatography (ERCP). Methods Data of 109 patients with difficult biliary cannulation in ERCP, of whom 56 underwent transpancreatic septum precut and 53 had needle-knife sphincterotomy from January 2006 to July 2008, were analyzed retrospectively, and the success rate of cannulation and the occurrence of complications were compared between the two methods. Results Of 109 patients accepted precut papillotomy, common bile duct cannulation was successfully achieved in 97. The success rates of transpancreatic septum precut group and needle-knife sphincterotomy group were 96.4% (54/56) and 81.1% (43/53) respectively, which was significantly different (P<0.05). Complications occurred in 11 cases, including bleeding(n =4), acute pancreatitis(n=5), cholangitis(n=2). The tolal frequency of complications of the transpancreatic septum pre-cut papillotomy group was lower than that of needle-knife sphincterotomy group(3.6% vs. 17.0%, P<0.05). Conclusion In patients with inaccessible bile ducts, transpancreatic septum precut is a safe and effective procedure in cannulation, exhibiting a higher success rate and lower occurrence of complication when compared with needle-knife sphincterotomy.

Objective To evaluate the influence of different cannulation technique in endoscopic retrograde cholangiopanereatography (ERCP) on success rate, risk of post-ERCP complication and operation time of the procedure. Methods The data of 120 patients who underwent ERCP from June 2000 to June 2008 because of biliary duct disorders were retrospectively studied. Conventional carmulation technique was applied in 60 patients and guide-wire eannulation was used in other 60. The success rate, total time of ERCP operation and the incidence of post-ERCP complications including acute pancreatitis and biliary system infec-tion within 7 days were assessed. Results Compared with conventional carmulation technique, selective can-nulation with a standard ERCP catheter under the assistance of guide-wire proved a higher success rate and a shorter operation time (P<0.05). Incidences of postoperative pancreatitis and infection with conventional cannulation were 10.0% (6/60) and 23.3% (14/60), respectively, while with guide-wire assisted cannu-lation were 3.3% (2/60) and 10.0% (6/60), respectively. No complication of bleeding was observed in either group. Conclusion Guide-wire assisted cannulation in ERCP can shorten operation time, improve success rate and reduce post-ERCP complications. Further evaluations are warranted.

OBJECTIVETo evaluate the efficacy of endoscopic variceal sclerotherapy (EVS) for esophago gastric variceal bleeding.

METHODSA retrospective analysis was made on 1010 patients with esophagogastric variceal bleeding who underwent sclerotherapy, among whom there were 834 patients with cirrhosis, 160 with hepatocarcinoma, 12 with Budd-Chiari syndrome and 4 with congenital liver fibrosis. Totally, 3203 sessions of sclerotherapy were performed, including 602 sessions of emergency sclerotherapy and 2601 of selective surgery. The average number of sessions of sclerotherapy for the initial treatment in 710 cirrhosis patients who received continuous sclerotherapy was 3.2 +/- 1.1 times. Follow-up was done in 579 cirrhosis patients for 3-157 months, with an average period of 42.5+/- 32.8 months.

RESULTSThe rate of emergency hemostasis in the whole group was 97.0%. The rate of complications was 13.4%, and the mortality rate was 1.8%. The rate of complete eradication and basically complete eradication of esophagogastric varices in cirrhosis patients was 84.1%. The late rebleeding rate was 23.7%, and the survival rates were 95.8% +/- 0.8%, 86.1% +/- 1.6%, 74.5% +/- 2.4%, 53.6% +/- 3.8% at 1, 3, 5 and 10 years, respectively, according to Kaplan-Meier analysis.

CONCLUSIONEVS is an important method for the treatment of esophagogastric variceal bleeding.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Esophageal and Gastric Varices ; therapy ; Female ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; therapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Sclerotherapy

Objective To investigate the indication and therapeutic effect of endoscopic pancreatic stent insertion. Meth-ods 13 procedures of endoscopic sphincter incision,2 procedures of endoscopic pancreatic stone picking,3 procedures ofendoscopic papilla adenoma or carcinoma resection. 10 procedures of endoscopic stenosis dilatation and 20 procedures ofendoscopic pancreatic stent insertion were performed on 9 cases of chronic pancreatis (in which 3 cases had pancreaticstone), 1 case of acute recurrent pancreatis.4 cases of pancreatic cancer and 3 cases of papilla adenoma or carcinoma.The duration of stent preserve was 2 weeks to 8 months, average 3. 43 months. Results Remission of abdominal pain,improvement of appetite and digestive function was found in 16 cases. 12～35 months (average 26. 17 months) of follow-up was performed on patients of pancreatitis after the stents were removed. 7 cases had no recurrence of abdominal pain,3 patients still often had upper abdominal pain. Complications:3 cases had mild elevation of serum amylase and lipase. 1case had obstructive jaundice. Conclusion Endoscopc pancreatic stent insertion and drainage is suitable for pancreaticduct obstruction caused by chronic pancreatitis or cancer. It can alleviate symptoms and has reliable therapeutic effect andgood security.

Objective To evaluate the efficacy of scle rotherapy (EVS) for esophageal variceal bleeding. Methods Retrospective analysis was made in 1 010 patients with es ophageal variceal bleeding who underwent sclerotherapy, among them 850 patie nts were cirrhosis and 160 patients were hepatocellular carcinoma. The total num ber of procedures of sclerotherapy were 3 203, including 602 for emergency scler otherapy and 2 601 for selective sclerotherapy. Supplementary sclerotherapy was performed in 502 cases. Average procedures of sclerotherapy for initial treatme nt were 3.18?1.1 in 710 cirrhotic patients. Follow-up was made in 579 cirrhosi s patients for 3-157 months, with an average follow-up period of (42.47?32.78) months. Results 1. The hemostasis rate in the whole group was 97.0%, the c omplication rate was 13.4%, and the mortality was 1.8%. 2. Rate of complete and nearly complete elimination of esophageal varices in cirrhotic patients was 84 .1 %, and long term rebleeding rate was 23.7%. Survival rates were (95.8?0.8)% 、(86.1?1.6)%、(74.5?2.4)%、(53.6?3.8)% at 1,3,5 and 10 year according to t he Kaplan-Meier analysis. Conclusions EVS is an important method of treatment for esophageal variceal bleeding.