Background:studies have shown that anticentromere antibody (ACA) positivity in primary Sjogren’s syndrome (pSS) is associated with autoimmune liver diseases, most often primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) [1, 2, 3], but detailed characteristics of the frequency and severity of liver disease in these patients is not presented in the literature.Objectives:to identify the frequency, structure and characterize the course of autoimmune liver diseases in pSS+ACA.Methods:we observe 82 patients with pSS+ACA. The diagnosis of pSS was established on the basis of Russian 2001 criteria, SSc was excluded based on the ACR/EULAR 2013 criteria [4]. 18 of 82 patients (22%) had a persistent increase in alkaline phosphatase, 11 of them were positive for antimitochondrial antibodies (AMA) and, according to the recommendations of the American Association for the Study of Liver Diseases [5], they were diagnosed with PBC. 7 of 18 patients were AMA-negative, 2 of them had a liver biopsy and the diagnosis of AMA-negative PBC was confirmed, 4 patients who did not have a liver biopsy and 1 patient with hepatitis B were excluded from the study. Also, in 6 of 64 patients without signs of liver damage, an increase in AMA was detected, in 1 of them a liver biopsy was performed and the diagnosis of PBC was confirmed. Thus, the group of patients with pSS+ACA and autoimmune liver diseases included 19 patients: 12 patients with AMA-positive PBC, 2 patients with AMA-negative PBC, and 5 patients with asymptomatic AMA positivity.Results:The median follow-up for 19 patients with pSS+ACA and autoimmune liver diseases was 4 years. AMA were detected in 89.5% of patients, an increase in IgM - in 42.1%, an increase in ALT / AST - 63.2%, a decrease in albumin, prothrombin index and cytopenia - 15.8% (were associated with the development of liver cirrhosis). In most cases, the clinical course of liver disease was characterized by an asymptomatic, slowly progressing course, with no signs of progression during observation. Cirrhosis and portal hypertension were detected in 15.8% of patients, hepatic encephalopathy - in 10.5%. Liver biopsy was performed in 9 patients, PBC was diagnosed in all cases (overlap syndrome with AIH was established in 3 cases). Assessment of PBC histological stages showed signs of stage 1 in 5 patients, stage 2 in 1 patient, stage 3 in 3 patients. Observation of 5 patients with stage 1 PBC and 5 AMA-positive patients without signs of liver damage (median follow-up was 2 years), showed the absence of clinical, laboratory and instrumental progression of liver disease, which is why we believe that these patients have epithelitis of the biliary ducts as manifestation of glandular lesions in pSS, but not PBC.Conclusion:autoimmune liver diseases in pSS+ACA are detected in 23.2% of patients, most of whom develop PBC and epitheliitis of the biliary ducts with the same frequency, less often overlap syndrome of PBC and AIH, and characterized by a mild, slowly progressing course and rarely lead to liver cirrhosis.References:[1]Masako Kita et al. Abnormal Liver Function in Patients with Sjogren’s Syndrome. Acta Med. Nagasaki 41: 31-37.[2]Baldini, Chiara et al. “Overlap of ACA-positive systemic sclerosis and Sjögren’s syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma.” Clinical and experimental rheumatology vol. 31,2 (2013): 272-80.[3]Bournia, Vasiliki-Kalliopi K et al. “Anticentromere antibody positive Sjögren’s Syndrome: a retrospective descriptive analysis.” Arthritis research & therapy vol. 12,2 (2010): R47. doi:10.1186/ar2958.[4]van den Hoogen, Frank et al. “2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.” Arthritis and rheumatism vol. 65,11 (2013): 2737-47. doi:10.1002/art.38098.[5]Lindor, Keith D et al. “Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases.” Hepatology (Baltimore, Md.) vol. 69,1 (2019): 394-419. doi:10.1002/hep.30145.Disclosure of Interests:None declared
Liver Biopsy as a Useful Tool in the Management of Autoimmune Liver Diseases in Childhood
