弹性假黄瘤中布鲁赫膜钙化的自然史
The Natural History of Bruch's Membrane Calcification in Pseudoxanthoma Elasticum.
作者信息
Risseeuw Sara, van Leeuwen Redmer, Imhof Saskia M, Spiering Wilko, Norel Jeannette Ossewaarde-van
机构信息
Department of Ophthalmology, University Medical Center Utrecht, Utrecht University, The Netherlands.
Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, The Netherlands.
出版信息
Ophthalmol Sci. 2021 Jan 11;1(1):100001. doi: 10.1016/j.xops.2020.100001. eCollection 2021 Mar.
PURPOSE
To describe the natural history of Bruch's membrane (BM) calcification in patients with pseudoxanthoma elasticum (PXE).
DESIGN
Retrospective cohort study.
PARTICIPANTS
Both eyes of 120 PXE patients younger than 50 years, 78 of whom had follow-up imaging after more than 1 year.
METHODS
All patients underwent multimodal imaging, including color fundus photography, near-infrared reflectance (NIR) imaging, and late phase indocyanine green angiography (ICGA). We determined the distance from the optic disc to the central and temporal border of peau d'orange on NIR, expressed in horizontal optic disc diameter (ODD). The length of the longest angioid streak was classified into 5 zones.
MAIN OUTCOME MEASURES
Age-specific changes of peau d'orange, angioid streaks, and ICGA hypofluorescence as surrogate markers for the extent of BM calcification.
RESULTS
In cross-sectional analysis, longer angioid streaks were associated with increasing age ( < 0.001 for trend). The temporal border of peau d'orange showed a weak association with increasing age (β = 0.02; 95% confidence interval [CI], 0.00-0.04), whereas the central border showed a strong association (β = 0.12; 95% CI, 0.09-0.15). Longitudinal analysis revealed a median shift of the central border to the periphery of 0.08 ODD per year (interquartile range [IQR], 0.00-0.17; < 0.001). This shift was more pronounced in patients younger than 20 years (0.12 ODD per year [IQR, 0.08-0.28]) than in patients older than 40 years (0.07 ODD per year [IQR, -0.05 to 0.15]). The temporal border did not shift during follow-up ( = 0.69). New or growing angioid streaks were detected in 39 of 156 eyes (25%). The hypofluorescent area on ICGA was visible only in the fourth or fifth decade and correlated with longer angioid streaks.
CONCLUSIONS
In PXE patients, the speckled BM calcification slowly confluences during life. The location of the temporal border of peau d'orange remains rather constant, whereas the central border shifts to the periphery. This suggests the presence of a predetermined area for BM calcification. A larger ICGA hypofluorescent area correlates with older age and longer angioid streaks, which implies that it depends on the degree of BM calcification.
目的
描述弹性假黄瘤（PXE）患者 Bruch 膜（BM）钙化的自然病程。
设计
回顾性队列研究。
研究对象
120 例年龄小于 50 岁的 PXE 患者的双眼，其中 78 例在 1 年多后进行了随访成像。
方法
所有患者均接受多模态成像，包括彩色眼底照相、近红外反射（NIR）成像和晚期吲哚菁绿血管造影（ICGA）。我们测定了 NIR 上从视盘到橘皮样改变中心和颞侧边界的距离，以水平视盘直径（ODD）表示。最长的血管样条纹长度分为 5 个区域。
主要观察指标
橘皮样改变、血管样条纹和 ICGA 低荧光的年龄特异性变化，作为 BM 钙化程度的替代标志物。
结果
在横断面分析中，较长的血管样条纹与年龄增加相关（趋势 P<0.001）。橘皮样改变的颞侧边界与年龄增加呈弱相关（β = 0.02；95%置信区间[CI]，0.00 - 0.04），而中心边界呈强相关（β = 0.12；95%CI，0.09 - 0.15）。纵向分析显示，中心边界每年向周边移动的中位数为 0.08 ODD（四分位间距[IQR]，0.00 - 0.17；P<0.001）。这种移动在 20 岁以下的患者中更明显（每年 0.12 ODD[IQR，0.08 - 0.28]），而在 40 岁以上的患者中为每年 0.07 ODD（IQR，-0.05 至 0.15）。颞侧边界在随访期间未发生移动（P = 0.69）。在 156 只眼中的 39 只（25%）检测到新的或增长的血管样条纹。ICGA 上的低荧光区域仅在第四个或第五个十年可见，且与较长的血管样条纹相关。
结论
在 PXE 患者中，散在的 BM 钙化在生命过程中缓慢融合。橘皮样改变颞侧边界的位置保持相对恒定，而中心边界向周边移动。这提示存在一个预定的 BM 钙化区域。较大的 ICGA 低荧光区域与年龄较大和较长的血管样条纹相关，这意味着它取决于 BM 钙化的程度。
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