PerspectiveOcular and Systemic Pseudoexfoliation Syndrome
Section snippets
Ocular manifestations relevant to clinical management
The hallmark of PEX syndrome is the pathologic production and accumulation of an abnormal fibrillar extracellular material in anterior segment tissues. As suggested by ultrastructural indications, the characteristic PEX fibrils appear to be multifocally produced by various intraocular cell types including the preequatorial lens epithelium, nonpigmented ciliary epithelium, trabecular endothelium, corneal endothelium, vascular endothelial cells, and virtually all cell types of the iris.4 The
Open questions and future challenges
Despite its clinical significance and many new insights in recent years, much remains to be learned about the clinical ocular and systemic manifestations, the epidemiologic and genetic characteristics, and the cause and pathogenesis of PEX syndrome. Last but not least, it would be desirable to have a universally applied nomenclature for this disease reflecting its true underlying pathophysiology. From a pathologist’s viewpoint, the process does not represent a true exfoliation of the lens
Conclusions
PEX syndrome is a common age-related generalized fibrotic matrix process, which may not only cause severe chronic open-angle glaucoma and cataract, but also a spectrum of other serious, potentially disastrous ocular and surgical complications. In view of the multitude of clinical complications, this entity is of relevance to all ophthalmologists including subspecialists in glaucoma, cataract, cornea, retina, uveitis, and neuroophthalmology. In view of a rising mean age of populations, we shall
Ursula Schlötzer-Schrehardt, PhD, is a Professor of Experimental Ophthalmology and a Chief Scientist in the Department of Ophthalmology at the University of Erlangen-Nürnberg, Erlangen, Germany. Apart from being dedicated to the subject of pseudoexfoliation syndrome and glaucoma, her research interests relate to corneal epithelial stem cells and ophthalmic pathology.
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Ursula Schlötzer-Schrehardt, PhD, is a Professor of Experimental Ophthalmology and a Chief Scientist in the Department of Ophthalmology at the University of Erlangen-Nürnberg, Erlangen, Germany. Apart from being dedicated to the subject of pseudoexfoliation syndrome and glaucoma, her research interests relate to corneal epithelial stem cells and ophthalmic pathology.
Gottfried O. H. Naumann, MD, is an Emeritus of the Department of Ophthalmology at the University of Erlangen-Nürnberg, Erlangen, Germany, has been President of the International Council of Ophthalmology (ICO) from 1998 to 2006. His textbook “Pathology of the Eye” is available in German, English, and Japanese languages. Dr Nauman clinical and research interests focus on microsurgery of the anterior segment and on Ophthalmic Pathology.
Supported in part by grant SFB-539 from the German Research Foundation.