Clinical and Pathological Characteristics of Autoimmune Hepatitis with Acute Presentation

Abstract

Aim. To study the differences between acute presentation-autoimmune hepatitis (A-AIH) and chronic autoimmune hepatitis (C-AIH). Methods. Through long-term follow-up, 80 patients were included in our study by using the revised international autoimmune hepatitis group (IAIHG) score and were divided into acute and chronic groups for comparison. Results. No significant difference was found in the gender, age, IAIHG score (pretreatment/posttreatment), definite diagnosis rate, extrahepatic autoimmune disease, onset time, or treatment before biopsy between the acute and chronic groups. In terms of clinical symptoms, A-AIH patients were more prone to jaundice, anorexia, yellow urine, and detesting oil than C-AIH patients, but melena only occurred in chronic group (P < 0.05). The acute group exhibited more severe injury upon histological evaluation, with lobular inflammation and bile duct injury, especially central necrosis of the lobule, more pronounced in this group (P < 0.05). Conclusion. A-AIH had manifestations of acute hepatitis and presented cholestasis. Serum indicators could preliminarily distinguish A-AIH and C-AIH. Histologically, the primary manifestation of A-AIH was lobular inflammation, which was usually accompanied by lobular central necrosis. For the diagnosis of A-AIH, more attention should be paid to long-term follow-up. This study was registered at ClinicalTrials.gov (identifier: NCT02994537).

Figures

Figure 1

Flow chart. The figure shows the entire process and the contents of the study. IAIHG: international autoimmune hepatitis group; AIH: autoimmune hepatitis; A-AIH: acute presentation-autoimmune hepatitis; TB: total bilirubin; ALT: alanine aminotransferase; AST: aspartate transaminase; C-AIH: chronic autoimmune hepatitis; HGB: haemoglobin; PLT: platelet; WBC: white blood cell; DB: direct bilirubin; ALB: albumin; GLB: globulin; ALP: alkaline phosphatase; GGT: gamma-glutamyl transferase; PT: prothrombin time; INR: international normalized ratio; IgG: immunoglobulin G; ANA: anti-nuclear antibody; AMA: anti-mitochondrial antibody; LKM: liver kidney microsomal; LC: liver cytosol; SLA: soluble liver antigen; ANCA: anti-neutrophil cytoplasmic antibody; Coombs: Coombs test; G: inflammation grade; S: fibrosis stage.

Figure 2

Histological features of A-AIH and C-AIH. A-AIH (A1–4) and C-AIH (C1–4): A1, C1: A1 shows multiacinar and centrilobular necrosis in A-AIH, which is uncommon in C-AIH (H&E, ×200, small pictures emphasized the typical areas in the big pictures); A2, C2: the main manifestation of A-AIH is cellular swelling, ballooning change, and lobular inflammation accompanied by mild interface inflammation; however, C-AIH often has moderate-to-severe interface inflammation caused by lymphoplasmacytic infiltration (H&E ×200); A3, C3: CK7 staining showing regeneration of cholangiocytes due to the multiacinar necrosis of hepatocyte in A-AIH. However, bile duct regeneration was less likely to be found in C-AIH (CK7, ×200); A4, C4: blue staining shows fibrosis in the portal area; fibrous septa were found in the chronic group but not the acute group (Masson trichrome staining, ×200).

Figure 3

ROC curve of ALB. The area under the receiver operating characteristic (ROC) curve was 0.765 for ALB, 95% confidence interval (CI) was 0.662–0.869, and rates for sensitivity and specificity were 72.9% and 68.7%, respectively.