OVERLAP OF IGG4 POSITIVE PRIMARY SCLEROSING CHOLANGITIS WITH AUTOIMMUNE HEPATITIS MAY BE ASSOCIATED WITH SCHISTOSOMAL PROCTITIS AND MICROSCOPIC COLITIS: A CASE PRESENTATION

Aims To diagnose a case of cholestatic Liver Disease .

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.

Results Bone Marrow Biopsy showed high normocelluar bone marrow in fragments and trails.Liver biopsy showed marked bridging fibrosis with occasional nodules completely surrounded by fibrosis.Moderate dense round cell infiltrate mainly lymphocytes with scattered plasma cells & neutrophils in the fibrous septa and the expanded portal areas.There was moderate bile duct injury and bile ductular proliferation. Intreface hepatitis (piece meal necrosis) was seen continuous around < 50% of portal areas. There was moderate cellular and canalecular cholestasis with feathery degeneration of the hepatocytes.Upper &lower GI endoscopies with distal dudenal & colonic biopsies showed mild non specific dudenitis.The rectosigmoid biopsies showed Bilharzial colitis.The cecum & transvers colon biopsies showed intact villous pattern with superficial erosions & infiltration of the lamina propria by mild chronic inflammatory infiltrate,formed mainly of lymphocytes and plasma cells (microscopic colitis).

Conclusions Overlap of IgG4 positive PSC with AIH may be associated with Schistosomal proctitis and microscopic colitis.