Plasma α-L-fucosidase-1 in patients with Sjögren's syndrome and other rheumatic disorders
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Ildikó Endreffy1, Geir Bjørklund2, Attila Bartha3
1Department of Pediatrics, Jósa András County Hospital, Nyíregyháza, Hungary.
Insights
Plasma L-alpha-fucosidase-1 (α-L-FUCA-1) levels were significantly associated with Sjögren's syndrome (SS) in both children and adults. This finding suggests α-L-FUCA-1 may serve as a potential serological marker for SS.
Area of Science:
- Biochemistry
- Immunology
- Pediatrics
Background:
- Human alpha-fucosidase (EC 3.2.1.51) is an increasingly important hydrolase.
- Limited data exist on plasma levels in pediatric autoimmune disorders, especially Sjögren's syndrome (SS).
Purpose of the Study:
- To assay plasma L-alpha-fucosidase-1 (α-L-FUCA-1) activity in hospitalized children and adults.
- To evaluate the association between α-L-FUCA-1 plasma levels and SS and other rheumatic disorders.
Main Methods:
- Plasma α-L-FUCA1 activity was measured in 73 Hungarian patients (32 children, 41 adults).
- Statistical analyses included linear regression, Durbin-Watson (DW), and Pearson tests to assess relationships between α-L-FUCA-1 levels and autoimmune manifestations.
Main Results:
- α-L-FUCA-1 showed a significant correlation with SS in both pediatric (P=0.0023) and adult (P=0.00035) cohorts.
- While α-L-FUCA1 distribution was not differential in other rheumatic disorders (r=0.2042, P=0.1531), it was significantly associated with SS (r=0.1462, P=0.0032).
Conclusions:
- Plasma α-L-FUCA-1 levels are significantly altered in patients with SS.
- Further research into α-L-FUCA-1 is warranted to explore its potential as a differential serological marker for SS.
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.