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Exfoliation syndrome (ES) is an age-related disorder in which greyish-white flakes accumulate in different tissues in the anterior eye. Its pathogenesis is not completely known, but it results in electron-dense microfibrils. The finding that these can be seen outside the eye in many visceral organs inspired the theory that ES might be a part of a generalized disorder. It was postulated that ES might contribute to increased morbidity, mainly of systemic vascular diseases. This review is a summary of the existing knowledge. The prevalence of arterial hypertension (AHT) in elderly populations is > 30%. No differences have been found in the frequency of AHT among patients with ES or exfoliative glaucoma (EG) compared with those with primary open-angle glaucoma (POAG) or no ES. There are conflicting reports of frequencies of ischaemic heart disease (IHD). A recent registry-based study that used uniform criteria for IHD found no difference in the rate of IHD between patients with EG and those with POAG. However, findings of elevated homocysteine levels in the plasma and aqueous humour of patients with ES or EG suggest an increased vascular risk. No studies have yet been conducted to assess possible links between ES and systemic vascular diseases. In a single-blind study, ES was associated with abdominal aortic aneurysm, but this was not found in a large, cross-sectional investigation. The frequency of ES in patients with diabetes mellitus (DM) is only about half of that when compared in patients with no ES or with POAG. This finding warrants further studies. Molecular genetics research has found no common denominator for ES and the vascular diseases. There is no evidence that ES or EG are related to increased mortality for cardiovascular diseases. Further large-scale, randomized clinical studies are required. At present, there are no known medical indications that infer an increased systemic vascular risk or imply a need for the complete internal medical examination of a symptom-free patient with newly diagnosed ES in the eye.
Dr. A. Tarkkanen, Helsinki University Eye Hospital, Helsinki, Finland. ahti.tarkkanen@kolumbus.fi