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<p>Pseudoxanthoma elasticum (PXE) is an inherited mineralization disorder that leads to calcification of elastic fibers. The ocular characteristics arise due to the calcification of Bruch’s membrane, which is located between the retinal pigment epithelium and the choriocapillaris. Despite major scientific progress in understanding the underlying pathological mechanism in recent years, no causal treatment has so far been established. Therefore, the management of patients is currently limited to the treatment of secondary complications, such as intravitreal anti-vascular endothelial growth factor (VEGF) injections in cases of exudative neovascularization. This article discusses upcoming studies that aim to reduce ectopic mineralization. To confirm the diagnosis and possible inclusion in such studies a&#xa0;second assessment should therefore be carried out in a&#xa0;specialized center for PXE.</p>

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Pseudoxanthoma elasticum – Neue Therapieansätze am Horizont?

  • 期刊论文

  • 发表日期：

    2025年5月28日

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  • Jonathan Meinke,
  • Kristin Raming,
  • Maximilian Pfau,
  • Frank G. Holz,
  • Kristina Pfau

摘要

Pseudoxanthoma elasticum (PXE) is an inherited mineralization disorder that leads to calcification of elastic fibers. The ocular characteristics arise due to the calcification of Bruch’s membrane, which is located between the retinal pigment epithelium and the choriocapillaris. Despite major scientific progress in understanding the underlying pathological mechanism in recent years, no causal treatment has so far been established. Therefore, the management of patients is currently limited to the treatment of secondary complications, such as intravitreal anti-vascular endothelial growth factor (VEGF) injections in cases of exudative neovascularization. This article discusses upcoming studies that aim to reduce ectopic mineralization. To confirm the diagnosis and possible inclusion in such studies a second assessment should therefore be carried out in a specialized center for PXE.