Abstract
D-penicillamine remains a cornerstone of copper chelation therapy in Wilson’s disease, an inherited autosomal recessive disorder, which results in impaired biliary copper excretion and accumulation of copper in plasma and tissues, preferentially in the central nervous system and liver. Safety of the drug is under debate because many case reports have described severe adverse events, leading to the discontinuation of therapy [ [1] Weiss K.H. Thurik F. Gotthardt D.N. Schäfer M. Teufel U. Wiegand F. et al. Efficacy and safety of oral chelators in treatment of patients with Wilson disease. Clin Gastroenterol Hepatol. 2013; 11: 1028-1035 Abstract Full Text Full Text PDF PubMed Scopus (140) Google Scholar ]. D-penicillamine can induce variable immune-related disorders, including antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis. The exact mechanisms of D-penicillamine-induced autoimmunity are still unknown. Of note, pre-existing autoimmune diseases can be present prior to the initiation of treatment in approximately 5% of patients with Wilson’s disease [ [2] Seessle J. Gotthardt D.N. Schäfer M. Gohdes A. Pfeiffenberger J. Ferenci P. et al. Concomitant immune-related events in Wilson disease: implications for monitoring chelator therapy. J Inherit Metab Dis. 2016; 39: 125-130 Crossref PubMed Scopus (11) Google Scholar ].
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