Plasma α‐L‐fucosidase‐1 in patients with Sjögren's syndrome and other rheumatic disorders

Abstract

Background Human α‐fucosidase (EC 3.2.1.51) is a hydrolase the importance of which has been increasing in the latest years. However, data about its plasma level in children with autoimmune disorders, particularly Sjögren's syndrome (SS), are lacking. In this study, the plasma activity of L‐α‐fucosidase‐1 (α‐L‐FUCA‐1) was assayed in hospitalized children and adults and its association with SS and other rheumatic disorders further evaluated. Methods In total 73 Hungarian hospitalized patients, 32 children (2.5‐10 years) and 41 adults (32‐68 years), were enrolled in the study and underwent plasma assay of α‐L‐FUCA1 activity. Linear regression, Durbin‐Watson (DW), and Pearson tests were evaluated to investigate the relationship between α‐L‐FUCA‐1 plasma levels and autoimmune manifestations. Results α‐L‐FUCA‐1 correlated with SS both in children (2‐sided t test, P = 0.0023) and in adults (2‐sided t test, P = 0.00035). Linear regressions showed that in other rheumatic disorders, α‐L‐FUCA1 did not show any differential distribution related to the particular pathology (r = 0.2042, P = 0.1531, DW test = 2.2139 positive), while this trend was radically opposite for patients with SS (r = 0.1462, P = 0.0032, DW test = 1.3664, negative). Conclusions Alterations in plasma level of α‐L‐FUCA‐1 were significantly associated with SS. This preliminary result should encourage further research on α‐L‐FUCA‐1 as a possible differential serological marker of SS.

Changes of peripheral blood α-L-fucosidase activity in patients with rheumatoid arthritis: a cross-sectional study

“…Clinically, AFU is often served as a tumor marker in various cancers and its importance in the occurrence and progression of inflammatory and autoimmune diseases has been reviewed early ( 27 ). A research found that levels of AFU were significantly correlated to rheumatic diseases and the immune function of AFU may be a marker of rheumatism or chronic inflammatory manifestations ( 14 ). This research also pointed out that AFU activity in peripheral blood can be detected to further emphasize the relationship between glucose metabolism and immune response in RA patients.…”

“…The relationship between AFU and autoimmune rheumatic disease was also discussed. For example, Endreffy et al reported that AFU was associated with chronic inflammatory pathologies in a pediatric cohort, suggesting that AFU is a biomarker of chronic inflammation and autoimmunity ( 13 ); moreover, alterations in plasma level of AFU were significantly associated with the development of Sjögren’s syndrome, and the preliminary result should encourage further research on AFU as a possible differential biomarker for Sjögren’s syndrome ( 14 ). A prior study proposed that the immune function of AFU may be a diagnostic marker of rheumatic disorders.…”

The dual role of fucosidases: tool or target

“…This leads to a neurovisceral condition known as fucosidosis, which is a rare lysosomal storage disorder inherited in an autosomal recessive pattern (Michalski and Klein 1999 ; Saleh-Gohari et al 2018 ). Recent works suggest that changes in the plasma level of FUCA1 can be associated with Sjögren’s syndrome, an autoimmune, chronic, and systemic disorder characterized by lymphocytic infiltration of the exocrine glands and a remarkable B-cell hyperactivity (Mavragani and Moutsopoulos 2014 ; Endreffy et al 2019 ). The presence of certain FUC, such as human plasma α-L-fucosidase (FUCA2), was found to be necessary for adhesion of Helicobacter pylori , particularly to gastric cancer and duodenal ulcer specific strains (Liu et al 2009 ; Miura et al 2019 ).…”