What causes Posner-Schlossman syndrome (PSS) (glaucomatocyclitic crisis)?

Updated: May 18, 2020
  • Author: Leonard K Seibold, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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The etiology of glaucomatocyclitic crisis has been a topic of debate. Several factors have been postulated as contributors to the development of glaucomatocyclitic crisis, to include the following:

  • Abnormal vascular process
  • Autonomic defect
  • Allergic condition
  • Variation of developmental glaucoma
  • Infection [14, 15] - Cytomegalovirus (CMV), [16, 17, 18, 19] herpes simplex virus (HSV), [19, 20] , and Helicobacter pylori [21]

Description of a final common pathway usually includes a reference to changes in the trabecular meshwork leading to a reduction of outflow facility. However, some authors describe an increase in aqueous production.

Transfer coefficients of fluorescein in aqueous in the anterior chamber, by flow and by diffusion, are elevated during attacks of glaucomatocyclitic crisis. Between attacks, both coefficients return to normal. [22]

Elevations in IOP are postulated to be secondary to inflammation of the trabecular meshwork, which may be mediated by prostaglandins or cytokines. [23]

Prostaglandins, especially prostaglandin E, have been found in higher concentration in the aqueous humor of patients during acute attacks. These levels return to normal between episodes. [24, 25]

In a study using rabbit eyes, prostaglandin E was shown to contribute to a breakdown of the blood-aqueous barrier. The vascular effects of prostaglandins may contribute to the tortuosity seen in iris vessels and the leakage demonstrated with fluorescein angiography of the iris. To confuse matters, in another animal study, elevated prostaglandins increased outflow facility, which would contribute to a lower IOP and, thus, not be consistent with the reduced outflow facility seen in patients with glaucomatocyclitic crisis during an acute episode. [26] Another theory purports an increased aqueous production resulting from elevated levels of aqueous prostaglandins.

In summary, the exact mechanism by which prostaglandins regulate IOP has not been described, but a direct correlation between elevated levels of prostaglandins in the aqueous humor and the level of IOP has been found during acute attacks of glaucomatocyclitic crisis.

Cytokines have also been found to be elevated in patients with uveitic glaucoma. Interleukin (IL)–6, IL-8, monocyte chemoattractant protein-1, tumor necrosis factor-a, and vascular endothelial growth factor were found to be elevated in 143 patients with uveitis glaucoma undergoing trabeculectomy. These elevations were found to be significant compared to healthy controls. [27] The significance of these levels remains unknown.

Most recently, CMV has become the accepted infectious precursor to uveitis in PSS. CMV PCR was performed in 73 cases of refractory anterior segment inflammation. CMV DNA was identified in 24 cases, and a higher number of DNA copies was found to be a risk factor for significant IOP elevation. [28]

In the setting of PSS, CMV PCR testing of aqueous biopsy samples has been positive in varying percentages. The percentages vary by location but typically range from 38% to as high as 75% in one study. [6, 29, 30] A causal relationship of CMV, confirmed via PCR testing of aqueous taps, has prompted the evaluation of topical and oral antiviral agents in immunocompetent patients. [31, 32, 33, 34]

Evidence also shows that glaucomatocyclitic crisis may be associated with POAG. Patients with a 10-year or longer history of PSS are 3 times more likely to develop visual field changes and optic disc changes. [35] These patients may have a higher than normal incidence of corticosteroid responsiveness, leading to an elevated IOP. This must be kept in mind during the treatment of this disorder with corticosteroids.

Associations with certain allergic conditions and gastrointestinal diseases, most notably peptic ulcer disease, have been described. [36]

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