IgG4-related Sclerosing Cholangitis: Report Of 2 Cases And Literature Review

Objective To investigate the clinical manifestations,serological changes,imaging findings,pathological features,diagnosis,differential diagnosis,and treatment of IgG4-related sclerosing cholangitis(IgG4-SC)and to raise awareness of this disease.Methods Two cases of IgG4-related sclerosing cholangitis(IgG4-SC),suspected to be hilar cholangiocarcinoma diagnosed in our hospital from 2016 to 2017 were studied and compared with the 38 patients with IgG4-SC diagnostic criteria in China whom reported by others kinds of literature,Then to discuss the disease,include it's clinical manifestations,imaging findings,histological findings,diagnosis,differential diagnosis,treatment,and prognosis.Results Among 40 patients,30 males and 10 females.The main clinical manifestations were obstructive jaundice(32,80%),abdominal discomfort and pain in 18 cases(45%),weight loss in 4 cases(10%)and fever in 3 cases(8%).Serology IgG4 examination was elevated in 33 cases(83%),7 cases were normal(17%),the CA199 examination was elevated in 24 cases(60%),and 16 cases were normal(40%).31 patients combined with autoimmune pancreatitis,3 diabetes,4 multiple lymphadenopathies,and 2 without any other diseases.Imaging examination: There were only 5 cases(12%)with hilar stenosis,2 cases(5%)with extrahepatic bile duct stenosis at the distal porta hepatis,and 3 cases(8%)with multiple intrahepatic bile duct stenosis.There were only 30 cases(75%)of distal stenotic extrahepatic bile ducts.18 patients underwent surgery,IgG4-SC confirmed by postoperative pathology.7 patients received hormone therapy after surgery.Symptoms resolved after treatment.21 patients were diagnosed with IgG4-SC by clinical diagnosis,and one patient received biliary stent implantation with hormone therapy,29 patients were treated with hormones,and symptoms were relieved after treatment.Follow-up for six months,follow-up of 36 patients,32 patients within did not recrudesce with six 0months,without obstructive jaundice and upper abdominal pain discomfort,fever and other symptoms.Conclusions IgG4-SC should be carefully identified with cholangiocarcinoma,pancreatic cancer,and PSC before surgery to avoid unnecessary surgical treatment.If necessary,diagnostic hormone therapy may be available.The diagnosis of IgG4-SC needs to be combined with serological changes,imaging findings,histological features and other organ involvement,hormone therapy,etc.IgG4-SC treatment is mainly based on glucocorticoid treatment,Most patients were effectively treated and recurrent hormone therapy was effective.