Background Hepatobiliary involvement (HBI) has been associated to systemic sclerosis (SSc). However, it is not considered as characteristic clinical manifestation of that autoimmune disease

Objectives To assess the prevalence and causes of HBI in SSc and to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non HBI).

Methods Up to January 2015, 1572 SSc patients were collected in the Registro de ESCLErodermia (RESCLE) and causes of hepatobiliary disturbances were recorded. We investigated the HBI related characteristics and survival from the entire cohort and according to the following cutaneous subsets: dcSSc, lcSSc, and SSc sine scleroderma (ssSSc).

Results Out of 1572, 118 (7.5%) patients had HBI, and primary biliary cholangitis (PBC) was largely the main cause (n=67, 4.3%), followed by autoimmune hepatitis (n=19, 1.2%), and anti-mitochondrial negative PBC (n=6, 0.4%). Other causes of HBI were: secondary liver diseases (n=11, 0.7%), SSc-related HBI (n=7, 0.4%), nodular regenerative hyperplasia (n=3, 0.2%), liver cirrhosis (n=3, 0.2%), and unknown origin (n=2, 0.1%). In multivariate analysis, HBI was independently associated to lesser risk of dcSSc (5.1% vs 24.4%, OR: 0.18, p=0.005%), and higher frequency of calcinosis (26% vs 18%, OR: 1.80; p=0.028), left ventricular diastolic dysfunction (46% vs 27%, OR: 1.73; p=0.027), sicca syndrome (51% vs 29%, OR: 2.03; p=0.003), and anti-centromere antibodies (ACA, 73% vs 44%, OR: 1.86; p=0.023). According to the cutaneous subsets, HBI was associated: (1) in lcSSc subset, to longer time from SSc onset to diagnosis (10.8±12.5 vs 7.2±9.3, OR: 1.03; p=0.012), sicca syndrome (54% vs 33%, OR: 1.96; p<0.001), and ACA (80% vs 56%, OR: 2.64; p<0.001); and (2) in ssSSc subset, to sicca syndrome (44% vs 19%, OR: 3.43; p=0.018). No associations were found in dcSSc subset. HBI was the cause of death in 2.3% patients. Kaplan–Meier cumulative survival for the SSc cohort and the cutaneous subsets, according to the presence or absence of HBI showed no differences.

Conclusions The prevalence of HBI in SSc was 7.5%. Primary autoimmune liver diseases accounted for 77% of all conditions. PBC was the main cause of HBI reaching 4.6% of the overall SSc cohort. Patients with HBI were rarely classified as dcSSc subset and the elapsed time from the first SSc symptom to SSc diagnosis was longer. Calcinosis cutis, diastolic dysfunction, sicca syndrome, and the presence of ACA were all independently associated to HBI. In lcSSc subset, HBI was associated to sicca syndrome and ACA but in ssSSc only sicca syndrome was more prevalent. HBI was the cause of death in 2.3%

References

1. Assassi S., Fritzler MJ, Arnett FC, et al., Primary biliary cirrhosis (PBC), PBC autoantibodies, and hepatic parameter abnormalities in a large population of systemic sclerosis patients. J Rheum 2009; 36: 2250–2256.

2. Rigamonti C, Shand LM, Feudjo M, et al. Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut 2006; 55; 388–394.

References

Disclosure of Interest None declared