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Pseudoexfoliationssyndrom

Klinische Diagnose und systemische Manifestationen

PEX syndrome

Clinical diagnosis and systemic manifestations

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Zusammenfassung

Das Pseudoexfoliations (PEX)-Syndrom spielt aufgrund des demografischen Wandels eine zunehmend wichtigere Rolle im klinischen Alltag. Neben dem Risiko einer Katarakt- und Glaukomentwicklung prädisponiert das PEX-Syndrom durch Beteiligung des gesamten Vorderabschnitts auch zu einem breiten Spektrum klinischer und chirurgischer Komplikationen. Die exakte und frühe Diagnose eines PEX-Syndroms ist in Anbetracht des hohen Glaukomrisikos und der potenziellen Komplikationen im Rahmen der Kataraktchirurgie von klinischer Relevanz. Eine gesicherte Diagnose des Krankheitsbildes kann aufgrund des häufigen Fehlens der zentralen Linsenablagerungen nur nach Pupillenerweiterung erfolgen. Frühstadien sind anhand dezenter Linsenveränderungen in Assoziation mit Pigmentdispersion, Pupillarsaumatrophie und Mydriasisschwäche erkennbar. Bei Vorliegen asymmetrischer Befunde hinsichtlich Pupillenweite, Pigmentierungsgrad, Katarakt und Augeninnendruck sollte dem Verdacht auf ein PEX-Syndrom nachgegangen werden. Obwohl die Beschreibung des PEX-Syndroms als systemischer Prozess der extrazellulären Matrix, einhergehend mit PEX-Ablagerungen in Haut, Gefäßwänden und zahlreichen Organsystemen, schon Anfang der 1990er-Jahre erfolgte, ist der kausale Zusammenhang zwischen diesen Ablagerungen und dem Vorliegen einer Systemerkrankung nicht abschließend geklärt. Durch eine wachsende Anzahl von Einzelstudien gibt es Hinweise auf Assoziationen zwischen PEX-Syndrom und kardio- und zerebrovaskulären Erkrankungen. Die Beweislage ist aber nach wie vor nicht eindeutig, und es besteht Bedarf an weiterer Abklärung durch bevölkerungsbasierte, prospektive, randomisierte Studien.

Abstract

As a result of demographic changes pseudoexfoliation (PEX) syndrome, an age-related systemic disorder of the extracellular matrix, will become an increasingly important issue in clinical practice. Apart from its well-known association with cataract and glaucoma, PEX syndrome predisposes to a broad spectrum of spontaneous and surgical ocular complications due to characteristic alterations of all anterior segment tissues. In view of the high risk of glaucoma development and potential complications during cataract surgery, an accurate and early diagnosis of PEX syndrome is of considerable clinical relevance. Since the characteristic central PEX deposits are lacking in up to 50 % of patients, a reliable diagnosis requires pupillary dilation. Early stages of the disease may be recognized on the basis of subtle alterations of the lens surface in addition to poor pupillary dilation and pigment-related signs including pigment dispersion and peripupillary atrophy. Any asymmetric clinical signs, e.g., regarding pupil width, pigmentation, cataract and intraocular pressure, should alert the ophthalmologist to the potential presence of PEX syndrome. Although the description of PEX syndrome as a systemic disorder of the extracellular matrix associated with the deposition of PEX material in the skin, blood vessel walls and various organ systems dates back to the early 1990s, a causal relationship between the abnormal material deposits and systemic diseases has not yet been clearly established. A growing number of smaller studies have found suggestive evidence for associations between PEX syndrome and cardiovascular/cerebrovascular diseases. The current evidence, however, is ambiguous and requires further investigation through multicenter or population-based, prospective, randomized clinical studies.

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  1. Klinik und Poliklinik für Augenheilkunde, Universitätsklinikum Leipzig AöR, Liebigstr. 10-14, 04103, Leipzig, Deutschland

    E. Scharfenberg

  2. Augenklinik, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Deutschland

    U. Schlötzer-Schrehardt

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Correspondence to E. Scharfenberg.

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Scharfenberg, E., Schlötzer-Schrehardt, U. Pseudoexfoliationssyndrom. Ophthalmologe 109, 952–961 (2012). https://doi.org/10.1007/s00347-012-2534-y

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