Methods Thirty Six years old male presented by 4 weeks of jaundice,fever, rigors, epistaxis. There was associated generalized fatigue, pruritus, dark urine .Bowel habits were normal.There was no history of drug intake or viral hepatitis. Lap.cholecystectomy was done 2 months earlier. Total bilirubin: 7.9 mg/dl,direct bilirubin: 4.5 mg/dl,Albumin:3.4 gm/dl, INR:1.5, ALT: 76 IU/L,AST:134 IU/L, Alkaline phosphatase:123 KA, GGT:123 IU/L.ANA, Anti-ds DNA,AMA,ASMA,ALKM,P-ANCA,total IgA level and IgA tTG,all were normal.Total IgG:4122 mg/dl (700–1800 mg/dl),IgG4:207 mg/dl (8–140 mg/dl),Total IgM: 400 mg/dl(< 240 mg/dl). Serum iron and cupper studies were normal.CBC showed thrombocytopenia (142 × 10ˆ9/L).Multiphasic abdomenal CT showed enlarged liver with multiple cirrhotic nodules. Moderately enlarged spleen with no focal lesions.Mild pelvic ascites. MRCP showed normal CBD .IHBRs showed stenotic segments with distal dilatation denoting inflammatory process (cholangitis).No pancreatic duct dilatation.