The significance of cytoplasmic antinuclear antibody patterns in autoimmune liver disease.

Lucy Eunju Lee,Gianfranco D Alpini,Jimin Hwang,Younhee Park,Hyun Jin Cha,Jason Jungsik Song

doi:10.1371/journal.pone.0244950

Lucy Eunju Lee, Gianfranco D Alpini + Show 4 more

Open Access

https://doi.org/10.1371/journal.pone.0244950

Copy DOI

Journal: PloS one	Publication Date: Jan 7, 2021
Citations: 6	License type: CC BY 4.0

Affiliation: Yonsei University, Johns Hopkins University

Abstract

We aimed to determine the significance of cytoplasmic antinuclear antibody (ANA) patterns using computer-aided immunofluorescence microscopy in patients with autoimmune liver diseases (AILD). ANA staining pattern was identified by treating cultured human epithelial type 2 (HEp-2) cells with the sera of the patients. Medical records of patients with suspected AILD who had positive cytoplasmic ANA patterns between February 2017 and November 2019 were retrospectively reviewed for clinical, laboratory, and immunological data. Cytoplasmic ANA patterns of AILD and non-AILD groups were compared. Further subgroup analysis of patients with AILD who had reticular or speckled cytoplasmic ANA patterns was conducted. We found that among the 196 patients with positive cytoplasmic ANA patterns, 113 (57.6%) were diagnosed with AILD. The percentage of reticular cytoplasmic pattern was higher in the AILD group than that in the non-AILD group (64.0% vs. 21.9%, p < 0.001). Furthermore, patients with AILD who exhibited a reticular ANA pattern demonstrated a higher positive rate for anti-mitochondrial antibodies (66.7% vs. 2.6%, p < 0.001) than those who exhibited the speckled ANA pattern. Moreover, AILD patients with the reticular ANA pattern displayed a lower positive rate for anti-smooth muscle antibodies (0% vs. 45%, p < 0.001) and nuclear ANA pattern (73.2% vs. 97.5%, p = 0.003) than those with the speckled ANA pattern. Therefore, cytoplasmic ANA patterns could be used to guide AILD characterization in suspected AILD cases, especially as the reticular ANA pattern is strongly associated with AILD. Thus, it is important to check cytoplasmic ANA patterns for AILD evaluation, even when nuclear ANA patterns are negative.

Highlights

Autoimmune liver disease (AILD) is a rare immune-mediated chronic liver disease with heterogeneous clinical characteristics
autoimmune hepatitis (AIH) is subclassified into types 1 and 2 (AIH-1 and AIH-2) based on the serological profile; AIH-1 involves the presence of antinuclear antibody (ANA) and smooth muscle antibody (SMA), while AIH-2 involves the Cytoplasmic pattern Reticular Speckled
primary biliary cholangitis (PBC) is associated with multiple nuclear dots or rim-like membranous nuclear pattern of ANA, which are very specific findings (>95%)

Summary

Introduction

Autoimmune liver disease (AILD) is a rare immune-mediated chronic liver disease with heterogeneous clinical characteristics. AILD includes three major disease entities, autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) [1]. AIH is characterized by liver inflammation of unknown origin, presence of antinuclear antibody (ANA) and anti-smooth muscle antibody (SMA), increased IgG levels, and response to corticosteroids [2]. PBC is characterized by destructive lymphocytic cholangitis and the presence of anti-mitochondrial antibody (AMA) [3]. PSC is an autoimmune cholestatic liver disorder that exhibits a characteristic beaded appearance of the intra- and extra-hepatic bile ducts and is strongly associated with inflammatory bowel disease and cholangiocarcinoma [4]. Some patients exhibit characteristics of both AIH and PBC, thereby comprising a distinct population with the AIH-PBC overlap syndrome [5]

Objectives

Methods

Results

Conclusion