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Anti-copper therapy safe for pregnant patients with Wilson’s disease

Women with Wilson’s disease who received anti-copper therapy during pregnancy had fewer spontaneous abortions compared with those who did not receive therapy, according to recently published data.

“Pregnancy in [Wilson’s disease] patients is safe and most patients have successful pregnancies. Our data confirm that patients with [Wilson’s disease] with adequate copper control have better chances of successful pregnancies than untreated [Wilson’s disease] patients,” the researchers wrote. “The risk of birth defects in [Wilson’s disease] is generally low, which also applies for patients treated with chelating agents.”

The retrospective study included data from 1965 to 2015 on 136 women with Wilson’s Disease with 282 pregnancies. The initial manifestations of Wilson’s disease were hepatic in 73 patients, neurologic in 44, mixed in 12 and asymptomatic in 7. Wilson’s disease was not diagnosed at the time of conception in 86 of the pregnancies.

Compared with the other patients in the study, those who presented with initial neurologic manifestations experienced significantly more spontaneous abortions (OR = 2.335; 95% CI, 1.323-4.118).

Liver cirrhosis and portal hypertension at time of conception were not significantly correlated with Wilson’s disease.

The researchers observed alterations of maternal hepatic function, such as hyperbilirubinemia and elevated transaminases, in 16 pregnancies among 14 women. All the cases of hepatic complication resolved after delivery; however, there were four cases of spontaneous abortion.

Newly observed neurologic symptoms or deterioration of existing symptoms were present in three pregnancies among three women.

Compared with therapy-naive patients, treatment with D-penicillamine and zinc during pregnancy resulted in significantly fewer spontaneous abortions (P = .003). Similarly, those who received treatment during pregnancy had significantly fewer spontaneous abortions compared with those who did not (OR = 3.01; 95% CI, 1.737-5.216).

There were evident birth defects in seven pregnancies.

“Prevention of symptomatic deterioration of the mother should be the primary concern, achievable by continuation of anti-copper therapy during pregnancy. This includes more frequent monitoring of liver function tests and close monitoring for neurologic symptoms,” the researchers concluded. “On the other hand, prevention of overchelation — or more precise over-decoppering — might be achievable by calculated dose adjustments in terms of dose reduction of anti-copper drugs during pregnancy.” – by Talitha Bennett

Disclosure: The researchers report no relevant financial disclosures.