Concomitant immune‐related events in Wilson disease: implications for monitoring chelator therapy

Seeßle, Jessica; Gotthardt, Daniel; Schafer, Mark A.; Gohdes, Annina; Pfeiffenberger, Jan; Ferenci, Péter; Stremmel, Wolfgang; Weiss, Karl Heinz

doi:10.1007/s10545-015-9866-0

Cited by 18 publications

(23 citation statements)

References 24 publications

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“…One study found more patients treated with DPen (6/91, 6%) to develop autoimmune diseases as compared to Zn (0/58) or trientine (0/58) . One study detected no difference between DPen‐ and Zn‐treated patients for the 15‐years probability of survival (78 ± 6% vs 67 ± 17%) .…”

Section: Resultssupporting

confidence: 77%

“…A total of 174 records were selected for full‐text screening to assess eligibility. Of these, 26 publications reporting on 23 studies met our inclusion criteria (Figure ) . Reasons for exclusion of the 148 studies are shown in Table .…”

Section: Resultsmentioning

confidence: 99%

“…One study found more patients treated with DPen (6/91, 6%) to develop autoimmune diseases as compared to Zn (0/58) or trientine (0/58). 73 One study detected no difference between DPen-and Zntreated patients for the 15-years probability of survival (78 ± 6% vs 67 ± 17%). 67 Focusing on progression of liver fibrosis, one study found a higher rate of progression in the DPen group (1/14, 7%) compared to Zn (0/3).…”

Section: D-penicillamine Vs Zinc Saltsmentioning

confidence: 95%

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Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta‐analysis of controlled studies

Appenzeller-Herzog

Mathes

Heeres

et al. 2019

Liver International

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Background & aims Wilson disease (WD) is a rare disorder of copper metabolism. The objective of this systematic review was to determine the comparative effectiveness and safety of common treatments of WD. Methods We included WD patients of any age or stage and the study drugs D‐penicillamine, zinc salts, trientine and tetrathiomolybdate. The control could be placebo, no treatment or any other treatment. We included prospective, retrospective, randomized and non‐randomized studies. We searched Medline and Embase via Ovid, the Cochrane Central Register of Controlled Trials, and screened reference lists of included articles. Where possible, we applied random‐effects meta‐analyses. Results The 23 included studies reported on 2055 patients and mostly compared D‐penicillamine to no treatment, zinc, trientine or succimer. One study compared tetrathiomolybdate and trientine. Post‐decoppering maintenance therapy was addressed in one study only. Eleven of 23 studies were of low quality. When compared to no treatment, D‐penicillamine was associated with a lower mortality (odds ratio 0.013; 95% CI 0.0010 to 0.17). When compared to zinc, there was no association with mortality (odds ratio 0.73; 95% CI 0.16 to 3.40) and prevention or amelioration of clinical symptoms (odds ratio 0.84; 95% CI 0.48 to 1.48). Conversely, D‐penicillamine may have a greater impact on side effects and treatment discontinuations than zinc. Conclusions There are some indications that zinc is safer than D‐penicillamine therapy while being similarly effective in preventing or reducing hepatic or neurological WD symptoms. Study quality was low warranting cautious interpretation of our findings.

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Section: Resultssupporting

confidence: 77%

Section: Resultsmentioning

confidence: 99%

Section: D-penicillamine Vs Zinc Saltsmentioning

confidence: 95%

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Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta‐analysis of controlled studies

Appenzeller-Herzog

Mathes

Heeres

et al. 2019

Liver International

Self Cite

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“…In a study, elevated serum antinuclear antibodies did not help predict longterm penicillamine associated immunological reactions. 67 Overall, most of the penicillamine-induced immunological adverse effects can be managed by concomitant steroids. 43,46 On prolonged penicillamine use, patients may also develop various skin lesions related to interference of penicillamine with collagen and elastin linking.…”

Section: Treatment Optionsmentioning

confidence: 99%

Advances in Treatment of Wilson Disease

Aggarwal

Bhatt

2018

Tremor and Other Hyperkinetic Movements

101

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Background: Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal. Though treatments for WD have been available since the 1950s, the disease continues to be associated with considerable morbidity and mortality because of missed diagnosis, and delayed or inadequate treatment. In this paper we survey WD-related literature in order to review recent advances in WD treatment. Methods: We performed a literature search using the PubMed database for articles relating to WD and its medical treatment. We reviewed the articles, and crossreferences of relevant articles, to summarize the current practices for treatment of WD. Results: The survey shows that if WD is properly treated, in most patients the liver can be stabilized, even severe neurological disability reversed, and patients can resume normal lives. Discussion: Medical treatment for WD includes use of copper chelators (penicillamine, trientine, dimercaprol, dimercaptopropane sulfonate, and ammonium tetrathiomolybdate) and drugs that decrease gastrointestinal copper absorption. Our knowledge of the treatment approaches has benefited from the large systematic clinical studies that have been conducted over the last decade. For each drug used to treat WD, we surveyed its development, indication for use, dosing, efficacy, and adverse effects.

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“…In a study, elevated serum antinuclear antibodies did not help predict long-term penicillamine associated immunological reactions. 67 Overall, most of the penicillamine-induced immunological adverse effects can be managed by concomitant steroids. 43 , 46 On prolonged penicillamine use, patients may also develop various skin lesions related to interference of penicillamine with collagen and elastin linking.…”

Section: Resultsmentioning

confidence: 99%

Advances in Treatment of Wilson Disease

Aggarwal,

Bhatt

2018

Tremor and Other Hyperkinetic Movements

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Concomitant immune‐related events in Wilson disease: implications for monitoring chelator therapy

Cited by 18 publications

References 24 publications

Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta‐analysis of controlled studies

Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta‐analysis of controlled studies

Advances in Treatment of Wilson Disease

Advances in Treatment of Wilson Disease

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