Symptoms of Wilson disease (WD) vary and additional factors such as autoimmunity may play an important role in WD pathogenesis. The presence of antinuclear antibodies (ANA), anti‐neutrophil cytoplasmic antibodies, neuronal surface antibodies, and onconeural antibodies in WD was investigated using standardized indirect immunofluorescence assays and Western Blot analysis. The presence of all studied autoantibodies was higher in WD patients in comparison to healthy subjects, but there was no statistically significant difference in autoantibodies frequency according to disease manifestation. D‐penicillamine treatment was associated with a higher presence of ANA than zinc sulfate but without an increase in autoimmune diseases rate.
Background Wilson's disease (WD) is a rare, autosomal disorder of copper metabolism, in which antibodies mediate and modulate the clinical manifestation of the CNS disease. The aim of this study was to investigate the presence and impact of autoantibodies in the clinical course of WD. Method Study involved 88 WD diagnosed and already treated patients with 7-years follow-up. Indirect immunofluorescence assays and Western Blot analysis was used. Results Patients who presented neurological manifestations had significantly more frequently ANA, ANCA and ANNA presence than in healthy individuals (p=0.029, p=0.009, p=0.025, respectively). In patients treated with d-penicillamine positive ANA antibodies were observed more frequently (p=0.005), whereas in zinc sulphate treated patients PP, ANCA and ANNA were significantly more frequently positive (p=0.038, p=0.024, p=0.002, respectively). Within the group of patients who died, positive ANA antibodies were significantly more frequent in comparison to patients who survived (p=0.020). Patients presenting positive cANCA had significantly higher UWDRS II, III and total scores compared to patients without ANCA antibody (p=0.033, p=0.022, p=0.022, respectively). Conclusion Drug-induced ANCAs may have a predictive value, as it is positively correlated with UWDRS II, III and total score in patients with WD. Only patients with neurological manifestations had significantly higher ANA, ANCA and ANNA presence. Increased positivity of those antibodies may predict the neurological manifestation of the severe cases and may be helpful for the neurological assessment of WD. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): Medical University of Warsaw
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