Background Systemic sclerosis (SSc) is a complex and rare autoimmune disease characterized by vascular damage as well as skin and internal organs sclerosis. The occurrence and extent of primary liver damage in SSc patients has not been adequately studied. In a review of 727 patients with scleroderma only 8 (1.1%) had hepatic involvement. If this very uncommon complication occurs, it usually happens 10 to 15 years after the onset of scleroderma. Transient elastography (Fibroscan) has been validated for the diagnosis of hepatic fibrosis.
Objectives Our aim was to study the frequency of liver stiffness detected with Fibroscan in patients with SSc and the association of liver stiffness with liver disease severity measured by the AST platelet ratio index (APRI).
Methods All consecutive patients with a diagnosis of SSc according to the American College of Rheumatology attending a single clinic during a one-year period were included. The APRI was calculated in all patients as follows: AST/upper limit of normal × 100/platelet count (109/l). An APRI≥0.5 has acceptable accuracy for excluding significant fibrosis. Transient elastography (TE) was performed with the Fibroscan Echosens 502 model (XL). Liver stiffness scores were expressed as kilopascals (Kpa). For the diagnosis of fibrosis, a cut-off value of TE >7.5 kpa has been proposed. Prevalence was obtained with 95% confidence intervals (CI). Correlation was tested with Spearman’s rho.
Results The study included 40 patients (M/F 6/34) with a median age of 59 years (range: 34-79), HBV, HVC, and anti-mitochondrial antibodies were all negative, alcohol consumption was less than 20 g/day in all cases. Median liver stiffness score was 5.32 Kpa (range: 3.1-9.4). One patient had a TE ≥7.5 Kpa (prevalence 5%; 95% CI 0.1 – 24.8). This patient had a value of 9.4 Kpa with an ultrasound pattern compatible with fatty liver and an APRI value of 0.18. The median APRI value was 0.29 (range: 0.16-0.58). The patient with the highest APRI value had a liver stiffness score of 0.31 Kpa. There was no correlation between the APRI score and liver stiffness by Fibroscan (rho =0.11).
Conclusions The prevalence of significant liver fibrosis is moderate to low in SSc patients with no clinical evidence of hepatic disease, as liver could be less prone to SSc-specific pathophysiologic events. Fibroscan, a non-invasive method, detected a prevalence of liver stiffness similar to expected. Liver enzymes levels do not correlate well with liver stiffness.
Disclosure of Interest None Declared