IgG4相关性硬化性胆管炎误诊为肝门部胆管癌原因分析OACSTPCD

Objective To investigate the clinical characteristics,types,clinical manifestations,diagnosis and treat-ment of IGG4-related sclerosing cholangitis(IgG4-SC)and the causes of misdiagnosis as hilar cholangiocarcinoma(HCCA),and to summarize the prevention measures of misdiagnosis.Methods The data of 2 patients with IgG4-SC misdiagnosed with HCCA admitted from March 2021 to June 2022 were retrospectively analyzed.Results The two patients were middle-aged and elderly males.The main manifestations were yellow skin and sclera with skin pruritus,epigastrica,loss of appetite and jaundice.The body weight was reduced,blood and liver function and some tumor markers were abnormal,and imaging exami-nations showed signs of hilar obstruction,suggesting space occupying lesions.The preliminary diagnosis was HCCA,and sur-gery was performed.No tumor cells were found in the post-surgical pathological examination,and a large number of plasma cells and lymphocytes were found in the bile duct wall in both patients.Therefore,the improved IgG4 examination showed ele-vated levels(14.3 g/L and 141.6 g/L,respectively),and then the revised diagnosis was IgG4-SC.After diagnosis,all pa-tients were treated with Methylprednisolone intravenous pulse therapy.After jaundice symptoms disappeared and liver function basically returned to normal after review,they were discharged from the hospital.All patients outside the hospital were given prednisone tablets orally,which were gradually reduced to a maintenance dose for long-term maintenance treatment.No recur-rence was reported since follow-up,and serum IgG4 levels remained normal.Conclusion IgG4-SC is rare in clinical prac-tice,and its clinical manifestations are similar to HCCA;therefore,it is difficult to differentiate IgG4-SC and more likely to lead to misdiagnosis.Clinicians should strengthen the understanding of the clinical characteristics,manifestations,diagnosis and differential diagnosis of IgG4-SC,expand their diagnostic thinking,comprehensively analyze the condition,and promptly conduct serum IgG4 detection,experimental treatment with Glucocorticoid,and histopathological examination if necessary,so as to reduce the occurrence of clinical misdiagnosis and misdiagnosis.