Clinical Relevance of Liver Involvement in the Clinical Course of Systemic Sclerosis.

Maria Lorena,Maia Lepore,Pier Paolo Sainaghi,Manuela Rizzi,Daniele Sola,Giulia Francesca Manfredi,Mattia Bellan,Rosalba Minisini,Roberta Pedrazzoli,Carla De Benedittis,Stelvio Tonello,Mario Pirisi,Cristina Rigamonti,Antonio Acquaviva

doi:10.3390/jcm11040966

Abstract

Liver involvement in systemic sclerosis (SSc) is rare. We evaluated the prevalence of liver fibrosis and hepatic autoimmunity in SSc patients in a retrospective observational cohort (97 SSc or mixed connective tissue disease with sclerodermic manifestations patients undergoing transient elastography, evaluating liver stiffness (LS) and controlled attenuation parameter (CAP), due to clinical indications along with biochemistry assessments and major antibodies associated to liver autoimmunity). Among them, 11 had LS ≥ 7.5 kPa and 5 showed an LS compatible with cirrhosis (LS ≥ 12.5 kPa). Predictors of LS ≥ 7.5 fibrosis were alcohol consumption (>14 or >7 alcoholic units/week for men and women, respectively), waist circumference (>102 or >88 cm for men and women, respectively), elevated alkaline phosphatase, and anti-La and anti-mitochondrial antibody (AMA) positivity. Six patients had CAP values compatible with severe steatosis (≥280 dB/m). Waist circumference, body mass index and diabetes mellitus were significant predictors of steatosis. Out of 97 patients, 19 were positive for AMA, 4 for anti-Sp100, 1 for anti-Gp210 and 7 were diagnosed with primary biliary cholangitis. Among SSc patients, hepatic fibrosis biomarkers and AMA prevalence are relatively high, suggesting the opportunity of performing a transient elastography and a screening for hepatic autoimmunity at diagnosis and/or during disease progression.

Highlights

Systemic sclerosis (SSc) is an autoimmune connective tissue disease (CTD) characterized by fibrosis of the skin and internal organs [1]
Age > 18 years; systemic sclerosis (SSc) diagnosis according to ACR/EULAR 2013 criteria [13]; mixed connective tissue disease (MCTD) with sclerodermic features according to Kasukawa criteria [14]; patients who underwent hepatic elastography in the previous two years
We show that among SSc patients, the proportion of those with significant liver fibrosis is not negligible, but can be predicted by a history of excessive alcohol consumption, large Waist circumference (WC) and high alkaline phosphatase (ALP) levels

Summary

Introduction

Systemic sclerosis (SSc) is an autoimmune connective tissue disease (CTD) characterized by fibrosis of the skin and internal organs [1]. The pathophysiology of SSc is complex and still under investigation: vascular damage in genetically susceptible individuals, elicited by environmental factors, induces the activation of endothelial cells. These cells release cytokines and adhesion molecules, triggering the immune system. Fibrotic changes of the internal organs, such as lungs, heart, kidneys, and gastrointestinal tract, characterize the clinical course of both limited (lcSSc) and diffuse cutaneous. A CTD may overlap with a liver autoimmune disease; the association with primary biliary cholangitis (PBC)

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