Relevance of the pseudoexfoliation syndrome for the glaucomas
Relevance of the pseudoexfoliation syndrome for the glaucomas
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DOI：
10.1007/s00347-002-0702-1
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发表时间：
2002-09-01
期刊：
影响因子：
--
通讯作者：
Naumann, GOH
中科院分区：
文献类型：
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作者：
Schlötzer-Schrehardt, U;Küchle, M;Naumann, GOH
继发性慢性开角型青光眼伴假性剥脱（PEX）综合征约占所有青光眼的25%，是青光眼最常见的可识别病因。潜在的疾病PEX综合征是细胞外基质的一种全身性过程，其特征在于在许多眼内和眼外组织中产生和进行性积累异常细胞外物质。最近的数据支持PEX综合征的发病概念，作为一种弹性组织变性，特别是弹性微纤维。小梁网在这种特征性基质过程中的积极参与可能导致40-60%的患者发生青光眼。此外，PEX综合征也是广泛的自发性或术中和术后眼部并发症以及全身性心血管疾病的重要风险因素。PEX相关的开角型青光眼代表了一种相对严重和进行性类型的青光眼，由于高眼内压水平和昼夜压力曲线的波动，其预后通常较差。慢性压力升高的主要原因似乎是小梁网细胞和施累姆氏管细胞局部产生PEX材料，随后发生施累姆氏管和小管组织的退行性变化。导致压力增加的其他致病因素包括明显的黑色素分散、房水蛋白浓度增加、血管因素和筛板结缔组织改变。其他类型的青光眼，如急性开角型青光眼，在诊断性瞳孔散大期间由黑色素簇射引起，或由于瞳孔或睫状体阻滞引起的继发性闭角型青光眼，在PEX患者中也很常见。发病因素TGF-β 1和TIMP-1/2似乎是因果关系参与这一纤维化过程，因此可能代表特定的，合理的治疗方法的潜在目标。
Secondary chronic open-angle glaucoma associated with pseudoexfoliation (PEX) syndrome accounts for approximately 25% of all glaucomas and represents the most common identifiable cause of glaucoma overall. The underlying disorder, PEX syndrome, is a generalized process of the extracellular matrix characterized by production and progressive accumulation of an abnormal extracellular material in many intra- and extraocular tissues. Recent data support the pathogenetic concept of PEX syndrome as a type of elastosis affecting particularly elastic microfibrils. Active involvement of the trabecular meshwork in this characteristic matrix process may lead to glaucoma development in 40-60% of the patients. In addition, PEX syndrome also represents an important risk factor for a broad spectrum of spontaneous or intra- and postoperative ocular complications as well as for systemic cardiovascular diseases. PEX-associated open-angle glaucoma represents a relatively severe and progressive type of glaucoma with a generally poor prognosis due to high intraocular pressure levels and fluctuations in the diurnal pressure curve. The primary cause of chronic pressure elevation appears to be local production of PEX material by trabecular meshwork cells and Schlemm's canal cells with subsequent degenerative changes of Schlemm's canal and juxtacanalicular tissues. Additional pathogenetic factors contributing to pressure increase include pronounced melanin dispersion, increased protein concentrations of the aqueous humor, vascular factors, and connective tissue alterations of the lamina cribrosa. Other types of glaucoma, such as acute open-angle glaucoma, provoked by melanin showers during diagnostic mydriasis, or secondary angle closure glaucoma due to pupillary or ciliary block, are also common in PEX patients. The pathogenetic factors TGF-beta1 and TIMP-1/2 appear to be causally involved in this fibrotic process and thus may represent potential targets for specific, rational therapeutic approaches.