Pseudoexfoliative fibrillopathy in visceral organs of a patient with pseudoexfoliation syndrome

Abstract

Evidence is increasing that pseudoexfoliative material develops in widespread areas of skin and parabulbar tissues as well as intraocularly. To determine whether this process is even more diffusely distributed, ultrastructural examination was performed on visceral and ocular tissues of a patient with long-standing glaucoma found to have bilateral ocular pseudoexfoliation at autopsy. Aggregates consistent with pseudoexfoliative material were present in the lung, heart, liver, and gallbladder, in addition to the classic intraocular sites. The aggregates were in the fibrovascular septa and stroma of these organs, most frequently adjacent to elastic and oxytalan fibers. They stained positively for elastin and human amyloid P protein, like the ocular sites, in preliminary immunologic testing. Rare atypical aggregates were seen in one of the four control patients. These findings suggest that pseudoexfoliation is a systemic process involving abnormal matrix synthesis, particularly as related to elastic tissue components.