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Supplementation essential for vitamin D deficiency in EPP: research

Vitamin D supplementation significantly increases vitamin D levels and reduces its deficiency, which is common in adults with erythropoietic protoporphyria (EPP), regardless of whether they are treated with Scenesse (afamelanotide), a recent study reports.

However, treatment with Scenesse did not significantly affect vitamin D levels in these patients, except in patients over 50 years of age or after prolonged treatment periods (2.5 to five years).

“Based on these findings, we recommend that future guidelines include prescription of (vitamin D) supplementation and regular monitoring of vitamin D levels in EPP patients, including those receiving (Scenesse),” researchers wrote.

The study, “The effects of cholecalciferol and afamelanotide on vitamin D levels in erythropoietic protoporphyria; a multicenter cohort study”, was published in the British Journal of Dermatology.

EPP is a form of porphyria characterized by hypersensitivity of the skin to sunlight and certain forms of artificial light, which in a significant proportion of patients results in a vitamin D deficiency and low bone density, or osteoporosis.

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Scenesse is a skin implant to increase pain-free exposure to light

Developed by Clinuvel, Scenesse is a skin implant approved in the US and Europe to increase pain-free light exposure and prevent phototoxic reactions, which refers to damage to the skin caused by exposure to sunlight. Each implant contains 16 mg afamelanotide and is placed under the skin once every two months in spring and summer.

However, “studies of vitamin D levels in EPP have only examined patients who had not been treated with (Scenesse).”

To investigate the prevalence of vitamin D deficiency in EPP patients receiving afamelanotide and/or vitamin D supplementation (cholecalciferol), the researchers conducted an observational study among adults with EPP who were followed at two medical centers in the Netherlands and Germany .

A total of 230 patients (138 from the Netherlands and 92 from Germany), mainly men (53%) with an average age of 39.1 years, were followed for an average of eight months.

In total, the researchers performed 1,774 vitamin D measurements, which corresponds to an average of 7.7 per patient with a median interval of two months.

Vitamin D measurements were performed in four groups of patients. A total of 135 measurements were taken in patients who received no treatment, 331 in those who received Scenesse, 104 in those who received vitamin D supplementation and 1,104 in those who received both treatments.

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79% of EPP patients have a vitamin D deficiency

The prevalence of vitamin D deficiency (vitamin D levels equal to or less than 50 nmol/l) in EPP patients was 79%, with more than half (55%) of patients having severe deficiency (levels equal to or lower than 30 nmol/l). L).

Vitamin D supplementation significantly increased median levels of vitamin D to 60 nmol/l when given alone, or to 62 nmol/l when combined with Scenesse, reducing the prevalence of vitamin D deficiency to 46% and that of severe vitamin D deficiency up to 62 nmol/l. 19%.

Treatment with Scenesse alone did not significantly increase mean vitamin D levels nor reduce the prevalence of vitamin D deficiency, which was 71%.

After adjusting the data for sex, age, season and treatment group, the researchers found that both vitamin D supplementation and the combined treatment significantly increased vitamin D levels over time. These effects were observed immediately after initiation of treatment.

When Scenesse was given alone, it only significantly increased vitamin D levels after 2.5 to five years of starting treatment. A significant increase in vitamin D levels was also observed in patients taking Scenesse who were over 50 years old.

Subgroup analysis also revealed that women with EPP had higher vitamin D levels than men, and that treatment effects were generally more beneficial in women. Vitamin D supplementation was more effective during the winter months and all treatments had a more pronounced effect in older patients overall.

“Our study highlights the continued importance of cholecalciferol as part of supportive care for EPP patients, even in the availability of (Scenesse),” the researchers wrote, adding: “future research should focus on EPP patients who receive afamelanotide for extended periods of time to assess its impact. at vitamin D levels.”