Spectrum of Autoimmune Liver Disease and Real-World Treatment Experience from a Tertiary Care Hospital

Abstract

Background and aims: Autoimmune liver disease (AILD) comprises of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) with a spectrum of overlap amongst the three. We analyzed the spectrum and treatment outcomes of patients with AILD presenting to a tertiary care center in India.

Methods: A retrospective analysis of AILD patients from June 2008 to April 2021 was performed. The diagnosis was based on clinical, biochemical, imaging, serological, and histological characteristics. Eligible patients received treatment depending on the disease stage. Biochemical response to treatment was defined as normalization of AST, ALT, bilirubin, and immunoglobulin G levels at 6 months in AIH, normalization of total bilirubin and/or albumin at 1 year in PBC and decrease in alkaline phosphatase (ALP) levels by 40% in PSC.

Results: Two hundred seventy-five patients were analyzed. AIH (58.54%) was most common, followed by an overlap of AIH-PBC (24%) and AIH-PSC (6.54%), PSC (6.18%), and PBC (4.72%). Most patients presented in 3rd or 4th decade, except PBC which occurred predominantly in 5th decade. The majority of patients were females (72.72%). Jaundice was the most common presentation seen in 60% of patients. Cirrhosis was present in 57.47% of patients. Patients with overlap had more pruritus (54.76 vs 6.83%), fatigue (63.1% vs 49.7%), hepatomegaly (52.4% vs 25.5%), and higher ALP (80.9% vs 37.7%) than patients with AIH alone. Acute presentation was seen in 33 patients (13.5%) with most having AIH flare. Five patients had acute liver failure (ALF) and 9 had acute-on-chronic liver failure (ACLF). ALF was associated with 80% mortality while 55.56% of patients with ACLF had a complete biochemical response to immunosuppression. Among patients with AIH and/or overlap who received immunosuppression, a complete biochemical response to immunosuppression was seen in 60.69% of patients. High ALT (OR 1.001 [1.000-1.003], P = 0.034), high albumin (OR 1.91 [1.05-3.48], P = 0.034) and low fibrosis on biopsy (OR 0.54 [0.33-0.91], P = 0.020) predicted complete response.

Conclusion: AIH is the most common AILD followed by overlap syndromes, PSC and PBC in our cohort. Biochemical response to immunosuppression is seen in 60% of patients with AIH & low fibrosis score on histopathology predicts a complete response.

Keywords: ACLF, acute-on-chronic liver failure; AIH, autoimmune hepatitis; AILD, Autoimmune liver diseases; ALF, acute liver failure; ALP, alkaline phosphatase; ALT, alanine aminotransferase; AMA, anti-mitochondrial antibody; ASMA, anti-smooth muscle antibody; AST, aspartate aminotransferase; ELISA, enzyme-linked immunosorbent assay; IBD, inflammatory bowel disease; INR, international normalized ratio; IgG, immunoglobulin G; LC-1, liver cytosol 1; LKM-1, liver kidney microsomal 1; LSM, liver stiffness measurement; LT, liver transplant; MMF, mycophenolate mofetil; MRCP, magnetic resonance cholangiopancreatography; PBC, primary biliary cholangitis; PSC, primary sclerosing cholangitis; SLA, soluble liver antigen; UDCA, ursodeoxycholic acid; ULN, upper limit of normal; autoimmune hepatitis; cirrhosis; overlap syndromes; primary biliary cholangitis; primary sclerosing cholangitis.