Background:Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and skin as well as organ fibrosis. The lungs, skin, and gastrointestinal care frequently affected. Primary biliary cholangitis (PBC) is an autoimmune disease of the liver associated with potential progression to liver fibrosis. More recently, these two disorders have been described as an overlapping entity, especially in patients with limited, anti-centromere (CENP-B) positive SSc (1). Here, we report the first results of a pilot study of shear wave elastography (SWE) in patients with SSc and SSc/PBC compared to PBC patients.Objectives:To assess liver stiffness in patients with SSc, SSC/PBC overlap, and PBC with shear wave elastography.Methods:We analyzed a sample of 10 SSc to 11 PBC patients. In all patients, a baseline US examination of the liver and liver stiffness measurement by 2D SWE were performed using a GE logiq E10 ultrasound machine. Normal values for SWE in healthy people have recently been published (2). Liver stiffness measurement was performed according to the recommendations of the manufacturer and the recommendations of the current “EFSUMB Guideline and Recommendation on the Clinical Use of Liver Elastography” (3). In addition, age, body mass index (BMI), and antibody profiles were assessed.Results:Of 8 SSc patients without PBC, 6 were anti-CENPB pos., 1 had Scl70, and 1 Pm/Scl antibody. Median age was 59.5 (47-71). Median BMI was 23.1 (19.6-25). 1 patient had SSc/PBC overlap, 1 had hepatic steatosis. 1 was positive for CENP-B/AMA-M2 antibodies, the other patient was Scl70 positive. Median age of these 2 patients was 55.5 (55-56), BMI was 23.86 (20.9-26.8). 11 patients with PBC were positive for AMA-M2 antibodies, median age was 58 (41-78) years, BMI was 27.8 (15.8-50.3). The differences were not statistically different. Liver stiffness is expressed in kPa. Measurements had an interquartile range/median ratio <30%, indicating sufficient quality of the measurement. Liver stiffness was higher in patients with SSc/PBC overlap/hepatic steatosis (9.4±0.18, **p=0.0133) and PBC alone (7.359±2.51, **p=0.0163) compared to SSc alone (4.526±0.89 kPa). The results indicate that PBC is the main driver of liver stiffness in patients with SSc, and SSc alone may not necessarily lead to an increased liver stiffness (figure 1).Figure 1.Conclusion:Our results indicate that SWE is a useful tool in for the non-invasive assessment of liver stiffness in SSc and SSc/PBC overlap. We will further increase the sample size, especially of patients with SSc/PBC overlap. Of note, other liver diseases, such as hepatic steatosis, have to be kept in mind when SWE is performed as they may contribute to liver stiffness.References:[1]Lepri G, Randone SB, Cerinic MM, Allanore Y. Systemic sclerosis and primary biliary cholangitis: An overlapping entity?: Journal of Scleroderma and Related Disorders [Internet]. 2018 Oct 25 [cited 2020 Jun 17]; Available from: https://journals.sagepub.com/doi/10.1177/2397198318802763[2]Petzold G, Hofer J, Ellenrieder V, Neesse A, Kunsch S. Liver Stiffness Measured by 2-Dimensional Shear Wave Elastography: Prospective Evaluation of Healthy Volunteers and Patients With Liver Cirrhosis. J Ultrasound Med. 2019 Jul;38(7):1769–77.[3]Dietrich CF, Bamber J, Berzigotti A, Bota S, Cantisani V, Castera L, et al. EFSUMB Guidelines and Recommendations on the Clinical Use of Liver Ultrasound Elastography, Update 2017 (Long Version). Ultraschall Med. 2017 Aug;38(4):e48.Disclosure of Interests:Viktor Korendovych: None declared, Jan-Gerd Rademacher: None declared, Golo Petzold: None declared, PETER KORSTEN Speakers bureau: Abbvie, Pfizer, Boehringer-Ingelheim, Novartis, Chuigai, Sanofi, GSK, all unrelated., Consultant of: Abbvie, Pfizer, Boehringer-Ingelheim, Novartis, Chuigai, Sanofi, GSK, Gilead, Lilly, all unrelated., Grant/research support from: GSK, unrelated to this study.
Two-dimensional shear wave elastography and ultrasound-guided attenuation parameter for progressive non-alcoholic steatohepatitis
