Why is glaucoma associated with exfoliation syndrome?☆
Introduction
Exfoliation syndrome (XFS) is an age–related, generalized disorder of the extracellular matrix characterized by production and progressive accumulation of a fibrillar material, not only in ocular tissues, but in skin and connective tissue portions of various visceral organs. Its ocular manifestations affect all of the structures of the anterior segment as well as conjunctiva and orbital tissues. Overall, it is the most common identifiable cause of glaucoma, accounting for the majority of cases in some countries (Ritch, 1994b). Despite its wide prevalence and clinical importance, the pathogenesis of XFS and the exact composition of the exfoliation material (XFM) remain unknown. New and developing conceptual approaches have increased the importance of accurate diagnosis of what is a potentially curable disorder.
XFS was first described in 1917 by Lindberg (Lindberg (1917), Lindberg (1989)) who noted bluish-gray flecks at the pupillary margin of the iris in 50% of patients with chronic open-angle glaucoma. Angle-closure glaucoma (ACG) has only recently also been found to be common. The epidemiology of XFS in patients with and without has been extensively reviewed and is beyond the scope of this paper (Forsius, 1988; Ringvold, 1999; Ritch and Schlötzer-Schrehardt, 2001; Tarkkanen, 1962). We will examine the pathophysiologic and clinical features of the disease and describe the etiology of the development of glaucoma as it is presently understood.
Section snippets
Pathophysiology of XFS
XFS is a generalized abnormal process of the extracellular matrix characterized by the multifocal excessive production of a typical extracellular fibrillar material, which is not degraded but progressively accumulates in various intra- and extraocular tissues (Schlötzer-Schrehardt et al., 1992b; Streeten et al., 1992). The etiology of this systemic matrix process remains unknown.
Symptoms
Throughout its long and insidious course, XFS is usually clinically silent. Symptoms may arise only with the development of cataract or when glaucoma ensues. Open-angle glaucoma may remain asymptomatic and undetected until serious disability has occurred. However, in some cases it may give rise to subjective symptoms: dull eye ache, or “tension” due to high IOP; difficulty in reading, diminished peripheral or night vision, and ultimately failure of vision due to advanced glaucomatous
Natural history and prognosis
The prevalence of XFS in glaucoma cohorts is significantly higher than in age-matched non-glaucomatous populations. Elevated IOP with or without glaucomatous damage occurs in approximately 25% of persons with XFS, or about 6–10 times the rate in eyes without XFS (Aasved, 1971c; Kozart and Yanoff, 1982; Kozobolis et al., 1997; Ringvold et al., 1991). Glaucoma in XFS has a more serious clinical course and worse prognosis than POAG. At the time of diagnosis, IOP is higher than in POAG and there is
References (270)
- et al.
A histopathologic study of iris changes in pseudoexfoliation syndrome
Ophthalmology
(1995) - et al.
Iris modifications following extracapsular cataract extraction with posterior chamber lens implantation
J. Cat. Ref. Surg.
(1991) - et al.
The development of microneovascular changes in the iris in pseudoexfoliation of the lens capsule
Ophthalmology
(1987) - et al.
The presentation and prognosis of glaucoma in pseudoexfoliation of the lens capsule
Ophthalmology
(1988) Pupillary dilation in eyes with pseudoexfoliation syndrome
Am. J. Ophthalmol.
(1988)- et al.
Pseudoexfoliation syndrome masquerading as uveitis
Am. J. Ophthalmol.
(1994) Corticosteroid-induced ocular hypertension in pseudo-exfoliation of lens capsule
Am. J. Ophthalmol.
(1970)- et al.
Concerning the chamber angle, II. Exfoliation of the zonular lamella and glaucoma capsulare
Am. J. Ophthalmol.
(1940) - et al.
Glaucoma capsulare
Am. J. Ophthalmol.
(1947) - et al.
Hyphema associated with pupillary dilation in a patient with exfoliation glaucoma and warfarin therapy
Am. J. Ophthalmol.
(1999)
Increased prevalence of occludable angles and angle-closure glaucoma in patients with pseudoexfoliation
Am. J. Ophthalmol.
Optic disc hemorrhages in a population with and without signs of glaucoma
Ophthalmology
Dynamics of aqueous humor in the syndrome of exfoliation with glaucoma
Am. J. Ophthalmol.
Optic disk appearance in pseudoexfoliation syndrome
Am. J. Ophthalmol.
The frequency of fibrillopathia epitheliocapsularis (so-called senile exfoliation or pseudoexfoliation) in patients with open-angle glaucoma
Acta Ophthalmol.
The frequency of optic nerve damage and surgical treatment in chronic simple glaucoma and capsular glaucoma
Acta Ophthalmol.
Intraocular pressure in eyes with and without fibrillopathia epitheliocapsularis
Acta Ophthalmol.
Mass screening for fibrillopathia epitheliocapsularis
Acta Ophthalmol.
Incidence of defects in the pigmented pupillary ruff in eyes with and without fibrillopathia epitheliocapsularis
Acta Ophthalmol.
Pilocarpineeffect on the anterior chamber and lens thickness
Arch. Ophthalmol.
Pilocarpine in the presbyopedemonstration of an effect on the anterior chamber and lens thickness
Arch. Ophthalmol.
Pilocarpine-induced lens changesan ultrasonic biometric evaluation of dose response
Arch. Ophthalmol.
Pilocarpine therapy in glaucomaeffects on anterior chamber depth and lens thickness in patients receiving long term therapy
Arch. Ophthalmol.
Pseudoexfoliation syndrome in Icelandic families
Br. J. Ophthalmol.
Trabecular meshwork cellularity in primary open-angle glaucoma and non-glaucomatous normals
Ophthalmology
Early diagnosis and heredity of pseudocapsular exfoliation
Bull. Soc. Ophthalmol. Fr.
Lectin electron microscopic histochemistry of the pseudoexfoliation material in the skin
Invest. Ophthalmol. Visual Sci.
Acute angle-closure glaucoma in pseudoexfoliation syndrome
Glaucoma
Lens displacement associated with pseudocapsular exfoliation
Br. J. Ophthalmol.
Phakodonesisa sign of incipient lens displacement
Br. J. Ophthalmol.
Pseudocapsular exfoliation in the Bantu of South Africa, I. Early or pregranular clinical stage
Br. J. Ophthalmol.
Pseudocapsular exfoliation in the Bantu of South Africa, II. Occurrence and prevalence
Br. J. Ophthalmol.
Incidence of pseudoexfoliation in South African Negroes and Scots
Trans. Ophthalmol. Soc. UK
Anterior chamber depths in eyes with pseudoexfoliation
Br. J. Ophthalmol.
Pseudoexfoliation and angle-closure glaucoma
Glaucoma
The trabecular meshwork of a non-glaucomatous eye with the exfoliation syndrome
Virchows Arch.
Fibrillopathia epitheliocapsularis. The so-called senile exfoliation or pseudo-exfoliation of the anterior lens capsule
Acta Ophthalmol.
The fluoroangiographic picture of the iris in pseudoexfoliative glaucoma
Cesk. Oftalmol.
Fluorescein angiography and fluorophotometry of the iris in pseudoexfoliation of the lens capsule
Br. J. Ophthalmol.
Progressive corneal endothelial cell changes in anterior segment disease
Aust. N. Z. J. Ophthalmol.
[Morphology of the optic papilla in glaucoma. II. Secondary chronic open angle glaucoma]
Klin. Monatsbl. Augenheilkd.
Degeneration of zonular fibrils in a case of exfoliation glaucoma
Ophthalmologica
Exfoliation syndrome in the southern United States, II. Characteristics of patient population and clinical course
Acta Ophthalmol.
Pseudoexfoliation syndrome in patients with branch and central retinal vein thrombosis
Klin. Monatsbl. Augenheilkd.
Cataract extraction complicated by capsular glaucoma
Br. J. Ophthalmol.
Precapsular film on the aging human lens—precursor of pseudoexfoliation
Br. J. Ophthalmol.
Pseudoexfoliative disease of the lensa study in electron microscopy and histochemistry
Br. J. Ophthalmol.
On the molecular composition and physicochemical properties of the pseudoexfoliation material
Acta Ophthalmol.
The frequency distribution of the glaucoma tolerance limit
Acta Ophthalmol.
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Supported in part by the Joseph and Barbara Cohen Research Fund of the New York Glaucoma Research Institute, New York City, the Deutsche Forschungsgemeinschaft (SFB 539) and KESY, Greece.