Ab1051 Autoimmune Hepatic Disease in Sle - A Cohort Study Over 40 Years

Background: Systemic lupus erythematosus (SLE) is a chronic and potentially severe disease, that may affect virtually every organ and system. The 2019 EULAR/ACR criteria exclude both liver and gastrointestinal involvement. However, abnormal liver function tests (LFT) are frequently noted during the course of the disease and it can overlap with autoimmune liver disease (AILD), which may not be as uncommon as previously thought. Objectives: To investigate the prevalence of AILD amongst SLE patients in a cohort of 850 SLE patients followed for up to 45 years and to determine the clinical, serological and outcome characteristics of this population. Methods: Retrospective observational study from January 1978 to December 2023, of SLE patients followed in the Lupus Clinic at a tertiary hospital. Clinical records were reviewed to obtain information about the patients' demographics and both SLE and AILD clinical and serological profiles and outcome. Results: Among 850 patients, 17 (2.0%) were diagnosed with AILD. 14 (82.3%) and 2 (11.8%) had clinical, serological and histological profiles compatible with autoimmune hepatitis (AIH) or primary biliary cholangitis (PBC), respectively, and one (5.9%) fulfilled criteria for AIH/PBC overlap. No cases of primary sclerosing cholangitis were identified. Most were female (n=16; 94.1%) and caucasian (n=9; 52.9%). The mean age of SLE onset was 24.2 ± 13.8 years old, whilst the mean age of AILD diagnosis was 29.9 ± 17.8 years old. SLE diagnosis mostly preceded AILD diagnosis (n=10; 58.8%), with an average time to develop AILD of 5.6 years. Regarding SLE manifestations, constitutional (n=14; 82.4%), mucocutaneous (n=14; 82.4%) and musculoskeletal (n=15; 88.2%) symptoms were the most prevalent, as expected in a general SLE population, but vasculitic manifestations were somewhat more prevalent (n=9; 52.9%). Regarding the serological profile, all patients were anti-nuclear antibody positive (ANA), 8 (47,1%) were anti-dsDNA positive, 2 (11.8%) were anti-smooth muscle antibody (ASMA) positive and 3 (17.6%) were anti-mitochondria antibody (AMA) positive. Eight (47.1%) patients developed cirrhosis, but only 2 (11.8%) developed hepatocellular carcinoma (HCC; both had a Barcelona Clínic Liver Cancer stage of B or less). Three (17.6%) patients underwent liver transplantation and each had an AIH diagnosis. Five (29.4%) died during follow-up, but only in 2 (11.8%) was the cause of death directly related with their liver disease (1 due to liver failure, 1 due to HCC). Four additional patients were identified as having clinical and serological profiles suggestive of AIH, but no biopsy was performed, thus a definitive diagnosis could not be reached. However, as no alternative causes were evident, a working diagnosis of lupus hepatitis was assumed. Conclusion: Abnormal LFT are common during the course of SLE and might have several causes. Although not as common as drug-induced liver injury or hepatic steatosis, overlapping AILD must be considered, as AILD can progress to cirrhosis, HCC, liver failure and even death. Our study confirmed that overlap was more frequent in females, all the overlap patients were ANA positive and SLE typically precedes AILD. However, our study revealed some new findings that need to be confirmed in other cohorts eg higher prevalence of vasculitic SLE manifestations and cirrhosis in overlap patients. Our study emphasised the need to investigate markers that help to distinguish patients with AILD from those with lupus hepatitis, as this may have important therapeutic and prognostic implications. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.