Liver Fibrosis in Primary Sjögren’s Syndrome

Abstract

BackgroundPrimary Sjögren syndrome (pSS) is a systemic autoimmune epithelitis, potentially affecting salivary epithelium, biliary epithelium, and hepatocytes. Common immunological mechanisms might cause clinically silent liver inflammation, and combined with non-alcoholic fatty liver disease (NAFLD), liver fibrosis (LF) may occur. No studies have explored the occurrence of LF in the context of NAFLD among pSS patients.MethodsConsecutive pSS patients from the rheumatology outpatient clinic of the Department of Pathophysiology and individuals evaluated in the hepatology outpatient clinic for possible NAFLD serving as comparators underwent transient elastography (TE) to assess LF and liver steatosis (LS). All participants had no overt chronic liver disease. Clinical, demographic, and laboratory data were collected from all participants at the time of TE.ResultsFifty-two pSS patients and 198 comparators were included in the study. The median age (range) of pSS and comparators was 62.5 (30–81) and 55 (19–86) years, respectively. Both groups had similar prevalence regarding type 2 diabetes mellitus, hyperlipidemia, and similar body mass index (BMI). Patients with pSS had less frequently high LS (S2, S3) (27% vs. 62%, p < 0.001) and significant LF (F2–4) [2 (3.8%) vs. 34 (17.2%), p = 0.014] than comparators. Univariable analysis showed that advanced LF was significantly associated with older age, higher LS, greater BMI, and disease status (comparators than pSS); of these, only age was identified as an independent LF risk factor in the multivariable logistic regression analysis.ConclusionLiver fibrosis among pSS patients is most likely not attributed to the disease per se.

Atypical Presentation of Macrophage Activation Syndrome Associated with Primary Sjogren\'s Syndrome

“…It is generally considered that liver injury results from either infiltration of activated hemophagocytic histiocytes or overproduction of cytokines. 7 Our patient did not have infiltration; hence, we attributed this to high cytokine levels. But surprisingly, her bilirubin and enzymes were high when ferritin was low and vice versa.…”

“…Primary (familial) occurs in the pediatric age-group and is attributed to genetic mutations, while secondary occurs in adults and is usually triggered by malignancies *Human immunodeficiency virus or receiving long-term immunosuppressive therapy like glucocorticoids, azathioprine, and cyclosporine; #defined as hemoglobin <9.2 gm/dL and leukocyte count <5000/mm 3 , platelet count <110000/mm 3 ; a total score of 224 with a probability of 96-98% for HLH; our patient had a fever, mild hepatomegaly, high ferritin, triglycerides and normal fibrinogen levels, and elevated AST and positive bone marrow biopsy cirrhosis, autoimmune hepatitis, and sclerosing cholangitis. 7 Both hepatocellular and cholestatic patterns are seen in Sjogren's syndrome.…”

Case report: Unusual development of hepatocellular carcinoma during immunosuppressive treatments against rheumatoid arthritis overlapping Sjögren’s syndrome; cirrhotic steatohepatitis with liver inflammation and fibrosis lurks in autoimmune disorders

“…NAFLD, and type 2 diabetes mellitus lead to the release of multiple pro-inflammatory cytokines, including tumor necrosis factor-alpha and interleukin-6, which favor the development of hepatic steatosis and inflammation within the liver, preceding HCC development ( 29 – 31 ). The estimated prevalence of liver fibrosis was shown to be approximately 5%, with NAFLD reaching 30% in SS patients and being positively associated with body mass index ( 32 ). Despite the absence of extraglandular manifestations of SS, these combined risk factors may have contributed to the development of steatohepatitis in this case.…”

Acute intestinal pseudo-obstruction secondary to Sjogren’s syndrome in pregnancy: a case report and literature review

“…SjS induced primary biliary cirrhosis (PBC) or sclerosing cholangitis were also considered but eventually excluded after a meticulous evaluation. Hepatic fibrosis may be induced by SjS and lead to abnormal liver function [ 14 ], but due to the unavailability of liver biopsy during pregnancy to confirm the occurrence of fibrosis, SjS induced liver fibrosis is not the primary cause of consideration in this case. Given the fact that liver enzymes normalized after pregnancy termination, pregnancy itself was more likely to be the cause of hepatic injury, rather than Sjögren’s syndrome.…”