Continuing Education Activity

Posner Schlossman syndrome, also known as glaucomatocyclitic crisis, is an infrequently encountered cause of inflammatory ocular hypertension. It is characterized by recurrent episodes of uniocular rise in intraocular pressure, out of proportion with minimal intraocular inflammation. Cytomegalovirus infection of the anterior chamber may be associated with this disease. Once thought to be benign, it is now known to be associated with significant ocular morbidity and loss of vision. This activity outlines the evaluation and management of Posner Schlossman syndrome and highlights the role of the interprofessional team in evaluating and treating this condition.

Objectives:

• Summarize the etiology of Posner Schlossman syndrome.
• Outline the pathophysiology/histopathology of Posner Schlossman syndrome.
• Explain the common physical exam and investigation findings associated with Posner Schlossman syndrome.
• Review the interprofessional management and care of patients with Posner Schlossman syndrome.

Introduction

Posner and Schlossman first published a series of nine patients with the characteristic presentation of unique glaucoma, with open anterior angles of the anterior chamber, associated with unilateral acute elevations in intraocular pressure (IOP) in 1948.[1][2] This disease later became known as the Posner Schlossman syndrome (PSS). PSS is also called the glaucomatocyclitic crisis.[3] It is a unilateral ocular disease characterized by recurrent episodes of acute non-granulomatous anterior uveitis and increased intraocular pressure (IOP).[3][4] 

PSS is therefore classified as inflammatory glaucoma. These episodes or attacks could eventually result in secondary chronic glaucoma.[5] It is a rare disease, and initial attacks may go undetected because of the mild nature of the uveitis. PSS could be misdiagnosed as an acute angle-closure glaucoma episode early in the disease because of the acute marked elevation in intraocular pressure and unnoticed inflammatory reaction in the anterior chamber.[6] 

The clinical course of the disease tends to exhibit a variable pattern, and a patient could have frequent attacks with shorter symptom-free intervals or less frequent attacks with more prolonged symptom-free intervals. In-between episodes, the patient has a normal IOP. Whatever the case, the symptoms are self-limiting, with spontaneous resolution within days or weeks, even without treatment.[3][7] Management during an episode is aimed at controlling the IOP and minimizing intraocular inflammation.[3] The purpose of this review is to summarize information on the diagnosis and treatment of PSS.

Etiology

Though the exact cause of PSS is subject to debate as different etiologies of PSS, have been proposed.[8] Theories on etiology include autoimmune, and allergy as proposed by Theodore and Kraupa (who described this entity before Posner and Schlossman).[9] Posner and Schlossman proposed autonomic dysregulation as the mechanism of the disease, while Skolic, Raitta, and Vannas proposed abnormalities of the vasculature.[10][11] The infectious theory appears to have gained widespread acceptance because of well-documented evidence. The more likely infectious theory of PSS etiology implicates the following viruses, cytomegalovirus (CMV), varicella-zoster virus (VZV), and herpes simplex virus (HSV).[12][13] Another possible cause is Helicobacter pylori.[14]

The most robust evidence for a causal association between PSS and CMV is the report by some researchers of the successful treatment of PSS using antiviral agents targeting CMV. Bloch-Michel et al. first proposed CMV as the infective agent, and other researchers have corroborated this finding.[15] The evidence for this is detecting CMV antibodies and CMV DNA in the aqueous humor using polymerase chain reaction (PCR). The CMV DNA has been detected in the aqueous humor during periods of acute flares of PSS and weeks after an attack.[13]  

It has been reported that anterior uveitic patients who respond suboptimally to conventional topical steroid therapy have a higher probability of having a viral infection.[16][17] In a study, 67% of anterior uveitis patients who underwent aqueous sampling tested positive for CMV, HSV, VSV, or rubella.[16] It was also observed that patients with elevated intraocular pressures were more likely to test positive for CMV (75%).[18] Furthermore, oral ganciclovir and valganciclovir targeting the infecting virus have been used with good effect.[19] Intravitreal ganciclovir has also been used with a favorable response, confirming the viral etiology of PSS. Most of these patients experienced a relapse after cessation of antiviral therapy.[20][17]

Epidemiology

Males are more at risk of developing PSS. Though a study reported more females (56.6%), most studies have shown a male predilection.[21][22] PSS has been reported in a 13-year-old, but it is a disease of adulthood.[23] Most patients are diagnosed within the age range of 20 to 50 years.[24][25] However, affected age groups from various studies show significant regional variation. For instance, a European study reported that the incidence of PSS was highest in the 20 to 29 year age group.[26] Reports from Asia suggest a later onset, i.e., third to the fifth decade.[27][13]

Rates of PSS in Japan and Singapore have been documented to be within 1.7% to 4.3% of all uveitis diagnoses.[2][28][29] According to a Japanese study, the rate of glaucoma varies from 6.5% to 11.4% of all uveitic glaucoma.[30] Another study on PSS from Finland reported an incidence of 0.4 and a prevalence of 1.9 in 100 000 population.[26] A hospital-based study from Wenzhou, China, reported a ten-year cumulative incidence of 39.5 in 100 000 population and a mean annual incidence of 3.91 in 100 000 population. This study reported a male predominance and age range 20 to 59 years to be most affected. The same study reported a significantly higher rate of new cases in spring, suggesting a possible seasonal variation of PSS.[31]

Pathophysiology

The pathophysiology of PSS remains unclear. PSS appears to fall into the spectrum of acute uveitic conditions with possible involvement of the trabecular meshwork. With CMV infection of the anterior chamber, possible mechanisms of elevated IOP include

• Trabeculitis,
• Trabecular outflow obstruction by chronic inflammatory cells and pigments, and
• Eventual peripheral anterior synechiae (PAS) with evolution to secondary angle closure.[2] 

Around 26% to 52% of PSS patients may show the presence of CMV DNA in the aqueous.[32][33][16] The location of the initial infection is unknown. Possible locations include the iris, trabecular meshwork, and corneal endothelial cells. Genetics, vascular endothelial dysfunction, and aqueous humor cytokine profile may also contribute to the pathophysiology of PSS.[21] Furthermore, HLA polymorphism is associated with PSS amongst a Chinese population.[34] Polymorphism of both classical and nonclassical HLA types has been observed with PSS.[34][35] 

Hirose, Ohno, and Matsuda noted the presence of HLA-Bw54 in 9 (41%) of 22 Japanese patients with PSS.[36] This association with HLA suggests a genetic basis for PSS. However, the contribution of HLA to the entire spectrum of PSS is not known.

Histopathology

Mononuclear cells having long pseudopods have been identified in the trabecular meshwork using electron microscopy.[37] These mononuclear cells were seen in the intraoperative specimen of PSS patients who underwent trabeculectomy due to uncontrolled IOP. The significance and origin of these mononuclear cells are unknown, but they may increase resistance to aqueous outflow.

History and Physical

The history given by the patient may vary from being asymptomatic to having unilateral ocular pain or discomfort and dimness of vision. If symptomatic, recurrent episodes may be experienced. Haloes that may be seen around a light reflect the presence of corneal epithelial edema, which can be associated with a severe increase in intraocular pressure (IOP). It is usual for the patient to have repeated episodes in the same eye, but bilateral presentations have also been reported.[38] The frequency of episodes could vary considerably. Some patients have more frequent episodes within a month, while others have fewer episodes, e.g., one to two attacks within a year. In-between attacks, the patient is symptom-free.

An ocular examination is critical to making the diagnosis of PSS. Findings during an attack vary considerably. In some cases, vision may be normal, whereas extreme poor vision to counting finger or light perception may be the presentation if IOP significantly increases with corneal epithelial edema. The IOP will be elevated to as high as 40 mm of Hg or above during an attack.[39] Gonioscopy should be done and will show an open angle. However, Liu et al. reported a series of patients with narrow or closed angles diagnosed as PSS. This clinical scenario may be more commonly observed in patients of Asian descent.[40] 

Peripheral anterior synechiae are not seen despite the intraocular inflammation. There are reports of angle abnormalities such as anteriorly displaced Schwalbe's line and prominent iris process.[41][42] PSS has been reported in a 20-year-old patient with Axenfeld anomaly.[43] However, these are not taken as typical findings in PSS or as diagnostic criteria.

Evidence of intraocular inflammation usually appears after the IOP has been elevated for days to weeks. If examined using a slit-lamp biomicroscope, one will notice anterior chamber reaction with the occurrence of fine, non-granulomatous keratic precipitates (KPs). In cases with CMV infection, there could be associated endotheliitis. Endotheliitis is seen as a coin-like opacity appreciated on the cornea.[17] This area of endotheliitis consists of circumferential KPs with stromal edema.[37] This clinical presentation is highly predictive of aqueous humor infection with CMV, which can be confirmed by PCR testing of aqueous humor tap.[17] Features suggestive of a diagnosis of PSS include a history of recurrent acute attacks, high IOP with disproportionately few anterior chamber cells, and one or 2 'sentinel' keratic precipitates over the inferior endothelium.

Optic disc findings are significant in PSS. In several cases, the optic disc appears normal during an acute attack. Also, the optic disc may show decreased perfusion during the episode of elevated intraocular pressure.[44][45] Over an extended period and with the protracted rise in IOP, permanent glaucomatous damage may occur. In some cases, open-angle glaucoma eventually ensues. Another clinical sign observed after recurrent or prolonged episodes of PSS is iris atrophy.[11][32] Posterior synechiae are, however, not a clinical feature of PSS. A uveitic cataract may occur after prolonged intraocular inflammation.

Evaluation

Essential investigation in characterizing PSS is PCR analysis of aqueous humor tap to assess viral DNA.[46][27] The common viruses assessed are CMV, HSV, and VZV.[47][37] Also, laboratory serum titers for the same viruses can be done. Confirmation of the presence of the virus may play a vital role in treatment planning.

It is essential to perform central visual field testing (Humphrey visual field, 24-2 or 30-2) to assess a glaucomatous pattern of visual field loss, suggestive of optic nerve damage. Also, stereoscopic optic disc photography is helpful for glaucoma assessment and monitoring. The finding of progressive optic disc cupping and disc hemorrhage after an acute attack of severely elevated IOP may suggest permanent damage to the optic disc.

Iris angiography during an acute episode may demonstrate areas of segmental iris ischemia, vascular congestion, and leakage.[11] Scanning laser Doppler flowmetry of the optic nerve also demonstrates decreased optic nerve perfusion during an acute attack.[45]

Optical coherence tomography (OCT) and OCT angiography (OCTA) of the optic disc provide useful information about optic nerve structure, retinal nerve fiber layer, and peripapillary vessel density. A decrease in these parameters may suggest permanent glaucomatous damage. 

The images shown represent the optical coherence tomogram (OCT) image of both eyes of a male patient who was 45-year-old at the time of diagnosis of left eye Posner Schlossman syndrome. His clinical presentation was characterized by recurrent episodes of left eye anterior uveitis (iritis), elevated intraocular pressure, and open anterior chamber angle. His vision was normal in both eyes. He was normal in between attacks. This OCT was obtained nine years after presentation; Figure 1 shows significant loss of ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) in the left eye. There is evidence of progression to glaucomatous damage, characterized by optic nerve cupping of the left eye. The optic nerve is normal in the right eye. Figure 2 examines the change analysis over the nine-year follow-up period and reveals a rate of GCC loss of -0.97microns/year and RNFL of -1.14microns/year, both having significant P values. He has been managed with topical steroid and non-steroid (topical Nepafenac) during periods of acute inflammatory flair and maintained on topical hypotensive to manage ocular hypertension.

Furthermore, anterior segment OCT of the cornea in eyes with PSS associated with an endotheliitis depicts this as an irregular and highly reflective endothelial cell layer.[48] Also, confocal scanning microscopy of the cornea endothelial layer in eyes with endotheliitis shows large endothelial cells containing nuclei with a high reflection area and surrounded by a halo of low reflection, like the eye of an owl.[49] These "owl eye" cells are deemed to be CMV infected corneal endothelial cells with an intranuclear inclusion body.

Treatment / Management

Treatment of PSS is aimed at IOP reduction and reducing intraocular inflammation. More recently, therapy targeting the infecting virus using antiviral treatment has been used. Treatment goals for PSS can be achieved using medical and surgical methods.

Medical Therapy

First-line treatment for reduction of IOP during acute attacks includes topical beta-blockers, alpha agonists, and carbonic anhydrase inhibitors. Any of these classes of topical medications or a combination of drugs can be used with a good response. In some cases, systemic medications such as oral carbonic anhydrase inhibitors are warranted to control the acute rise in IOP. It is best to avoid topical prostaglandin analogs because of the risk of exacerbation of inflammation. If the IOP is reduced to normal, such topical ocular hypotensive could be withdrawn between attacks, especially if the patient is at low risk of developing GON or other ocular complications. Use of topical pilocarpine should be discouraged as this is thought to exacerbate trabeculitis.[50]

Intraocular inflammation, often mild, can be controlled using topical steroids such as prednisolone or dexamethasone. Topical fluorometholone has also been used with good effect to control low-level intraocular inflammation. Topical and oral non-steroidal anti-inflammatory drugs (NSAIDs) can be used for the suppression of inflammation. NSAIDs will avoid possible steroid-induced glaucoma and has a desirable anti-prostaglandin effect since aqueous prostaglandin level increase has been associated with attacks.

Patients should be followed up daily to monitor the IOP during an acute episode to ensure lowering of IOP to a safe level. Less frequent visits (weekly or monthly as appropriate) can be adopted when steroid and ocular hypotensive are tapered. Instructions to consult with an ophthalmologist as soon as an attack is noted should be given. In general, at the time of initiation of treatment, documentation of baseline optic nerve parameters (as listed earlier) is required in the absence of apparently significant corneal edema. Topical ocular hypotensive and steroid should be commenced appropriately and tapered if the response is favorable. If the response to maximum therapy is unfavorable (i.e., the patient shows no signs of improvement) or if multiple recurrences occur, a search should be done for the presence of anterior chamber CMV infection using an aqueous tap. Treatment with an antiviral agent should be considered if evidence of CMV DNA is found.

There is evidence of benefit for treating CMV infection of the anterior chamber.[51][47] Treatment of this CMV infection has been shown to improve PSS control. Valganciclovir and ganciclovir have been used for the treatment of CMV infection. Different formulations and combinations have been researched, and good effects have been reported. Also, various routes of administration of antiviral agents have been studied, including topical, oral, and intravitreal routes for the treatment of CMV hypertensive uveitis.

A combination of topical and oral ganciclovir has been used in previous research. The use of topical ganciclovir has resulted in the resolution of inflammation with cessation of topical steroids and reduction in pressure-lowering drops in several cases. However, this treatment did not completely stop the recurrence of future episodes.[47][52] Different concentrations of topical ganciclovir have been used (2% and 0.15%). It appears that the 2% preparation of ganciclovir is associated with a higher concentration of the drug in the anterior chamber and results in less recurrence, less endothelial cell loss, and better control of IOP.[51][25] Topical ganciclovir has been used as a 2% gel with good effect.[51] The 0.15% ganciclovir gel, which had more recurrence in an earlier study, showed a good effect but with a longer duration of use in a more recent study.[53]

While oral valganciclovir was found to be superior to ganciclovir gel in terms of failure rate, the recurrence rate was significantly higher, 80%, compared to 57%.[52] Oral valganciclovir has been administered at an oral loading dose of 900 mg twice daily for a minimum of 2 weeks, then followed by an additional 450 mg valganciclovir twice-daily maintenance therapy.[54] In this study, a good treatment effect was noted at this dose.[54] However, recurrence occurred after valganciclovir was stopped. Recurrence following cessation of valganciclovir has been reported to be high. This high recurrence rate can be attributed to the fact that valganciclovir is virostatic instead of virucidal. Also, the eye's immune privilege prevents the total eradication of the CMV infection.[52] 

Long-term valganciclovir treatment resulted in long-term continued disease control in a reasonable number of patients, in doses of valganciclovir, as indicated earlier. Intravitreal injection of ganciclovir 2.0 mg in 0.05 cc, with or without oral antiviral, has also been studied.[55] Though intravitreal valganciclovir given at a dose of 2mg/0.05ml resulted in a reduction of aqueous CMV to undetected levels,  the rationale for intravitreal injections in an infection limited to the anterior chamber has been questioned. Further, oral valganciclovir can achieve similar aqueous CMV reduction, though with a risk of leukopenia.[56] White cell count should therefore be monitored in those patients on oral valganciclovir.[17][20]

Surgical Therapy

The use of surgery in PSS patients is for lowering IOP after the failure of medical treatment.[57] Surgery may also be needed for the management of corneal endothelial insufficiency. A surgical technique that has been used for IOP control is trabeculectomy. However, due to pre-operative conjunctival inflammation, with mobilization of fibroblasts and macrophages, there is an increased tendency for conjunctival scarring and consequent failure of the conjunctival filtering bleb.[58] 

Therefore, a practical technique is the augmentation of the trabeculectomy with antimetabolite, such as mitomycin C (MMC). Trabeculectomy with MMC reduces IOP spikes, and a general improvement in the inflammation and PSS disease is noticed.[59][60] An explanation for this post-operative improvement in inflammation might be because of draining out of the aqueous chamber inflammatory cells through the filtering bleb, reducing anterior chamber activity and trabeculitis.[2]

Other IOP lowering surgical techniques used in PSS include trabectome surgery, trabeculectomy, and Ahmed glaucoma drainage device insertion.

Cataract surgery will be required in the setting of cataract formation. As a general theme, pre-operative control of inflammation is essential before any surgical procedure is undertaken.

As in post uveitic corneal endothelial insufficiency, penetrating keratoplasty (PK) or Descemet's stripping automated endothelial keratoplasty (DSAEK) can be employed to treat a similar complication in PSS. Also, after corneal surgery in PSS eyes, graft failure rates will mirror the situation in post uveitic cornea grafts (50%), which is higher than for other causes of corneal endothelial insufficiency (13%).[61]

Differential Diagnosis

It is essential to recognize the differential diagnoses of PSS since this can affect the therapy and prognosis. The most familiar differential diagnoses include commoner glaucomas. These include acute and chronic angle-closure glaucoma, ocular hypertension, primary open-angle glaucoma (POAG), the uveitic glaucomas, Fuchs heterochromic iridocyclitis, herpetic iridocyclitis, and other viral uveitis.[62][63]  

Efforts must be made to differentiate PSS from other commoner causes of raised IOP.

Acute angle-closure (ACG) ranks high on the list because of the acuteness of symptoms, unilateral nature, presence of marked rise in IOP, and the presence of ocular inflammation. A case report of missed diagnosis of PSS as ACG exists, and in this case, peripheral iridectomy was performed due to an error in diagnosis.[6] However, ACG can be differentiated from PSS by the following features: narrow or closed angles, more severe painful red eye, fixed dilated pupil, nausea and vomiting, and peripheral anterior synechiae (PAS). In chronic ACG, the occurrence of PAS will be a distinguishing factor.

In POAG, the patient may be older; there may be a known family history, and intraocular inflammation is absent.

In uveitic glaucoma, the intraocular inflammation is often more fulminant. Fuchs heterochromic iridocyclitis can be differentiated from PSS by the presence of iris heterochromia (occurs late in the course of the disease), fine abnormal anterior chamber angle vessels, fine diffuse keratic precipitates, and posterior subcapsular cataract. The uveitic glaucomas are an essential group of conditions that are similar to PSS. This uveitic glaucoma could be viral in etiology, such as the herpetic iridocyclitis due to HSV and VZV. In order to differentiate this uveitic glaucoma from PSS, the following features may be observed, sectorial or diffuse iris atrophy, more severe anterior chamber reaction, and IOP may not be markedly elevated as seen in PSS.

Sectorial iris atrophy is suggestive of HSV or VZV. Also, vesicular rash and dendritic ulcers may be suggestive of HSV. Generally, in the clinical setting of high IOP and uveitis, one should consider other possible causes of ocular inflammation such as HLA B27 uveitis, sarcoidosis, HSV, Vogt Koyanagi Harada (VKH) disease, and human T lymphotropic virus type 1. The finding of a severely elevated IOP not in keeping with mild iridocyclitis, absence of PAS, and posterior synechiae, should raise the suspicion of PSS.

If the diagnosis is in doubt, performing aqueous humor sampling and qualitative PCR analysis may become necessary. More than one virus can co-exist in a patient.[64] There have been reports of concurrent occurrence of CMV positive PSS in one eye and HSV positive keratouveitis occurring in the fellow eye.

Other differentiating signs of HSV include keratitis with corneal stromal opacities and edema, resulting in scarring, neovascularization, and hypoaesthesia. VZV manifests with maculopapular rash and vesicles, which may occur at the nose's tip, side, or root and sometimes on the forehead. This presence of rashes at the tip of the nose is known as the "Hutchinson's sign" and is a strong predictor of intraocular involvement by the VZV. It is also seen in immunodeficiency states.

Pertinent Studies and Ongoing Trials

A study used enhanced depth imaging optical coherence tomography (EDI-OCT) to measure the macular choroidal thickness (CT) in PSS eyes during acute episodes and compared this with CT in remission and the fellow eyes and with controls.[65] This study reported a significantly thinner mean CT in affected eyes in acute attacks compared to fellow eyes. The mean CT was significantly thicker in healthy normal than the affected eye during an acute attack and similar to the contralateral unaffected eye. Also, mean CT was significantly increased in the affected eye in remission compared to during an acute attack.

Prognosis

Over the years, several patients have been noted to experience a progression to significant optic nerve damage with changes in the visual field and ganglion cell loss. The total duration of elevated IOP is responsible for this damage to the optic nerve, which assumes typical characteristics of a glaucomatous optic nerve head and therefore is assumed to be secondary glaucoma. This association with glaucomatous optic neuropathy (GON) has been explored extensively.[66]

In one study, as much as 45% of patients at the initial visit presented with concomitant GON.[5] This finding is because flares of inflammation occur with mild symptoms, and patients are unaware of the significant protracted rise in IOP and consequent damage to the optic nerve. For this reason, in eyes with evidence of significant OCT RNFL damage, it is rational to continue using topical ocular hypotensives in between acute attacks.[2] 

This category of patients should be treated prophylactically. In one study, up to 26.5% of PSS patients developed GON. Factors that could predict progression to GON have been researched. Among those factors examined, the total duration of disease was predictive of glaucoma progression; but the age of onset and number of attacks were not.[59]

Complications

Initially, PSS was considered to be a benign disease. However, this disease can result in significant ocular complications. On the list of ocular complications is progression to GON, as discussed in the preceding section. Patients at risk of developing subsequent optic nerve damage, such as patients with a large cup to disc ratio and RNFL damage and focal thinning of NRR (neuroretinal rim), should be treated prophylactically with topical hypotensive and can proceed to filtration surgery if IOP remains uncontrolled with evidence of progressive optic nerve damage.[2][67]

PSS can also cause non-arteritic anterior ischemic optic neuropathy (NAION) and optic atrophy.[68][69][70] Such eyes diagnosed with PSS with risk factors for NAION and ischemic optic atrophy, such as a small cup to disc ratio, should also receive prophylactic topical hypotensive.

Corneal complications can occur in PSS patients. Reduced endothelial cell count has been demonstrated in eyes with CMV-associated PSS.[2] Corneal endothelial insufficiency ensues with significant quantities of endothelial cell loss following recurrent disease. Also, persistent elevation in IOP results in cornea epithelial edema and predisposes to corneal endothelial cell loss. Endotheliitis is another finding in CMV-associated PSS. Endotheliitis is a clinical presentation of CMV infection of the anterior chamber in the immunocompetent patient. Other complications of PSS include iris atrophy and cataract.[32]

Deterrence and Patient Education

A high index of suspicion is required to make a prompt diagnosis of PSS. The finding of a unilateral markedly elevated IOP out of proportion with the level of intraocular inflammation with an open anterior chamber angle should raise the suspicion of PSS. Patient education is critical to prevent long periods of elevated IOP during attacks, resulting in earlier complications. Patients should be informed of the recurrent nature of the disease and advised to seek ophthalmologic consultation if symptoms of relapse such as mild ocular discomfort, pain, or blurry vision occur between periods of remission. Because some acute episodes may go unnoticed due to the mild nature of the uveitis, clinic appointments should be kept, and delays should be avoided in seeing PSS patients, especially those at risk of developing complications such as GON, NAION, and ischemic optic atrophy. Prophylactic treatment should be discussed with at-risk patients and escalation to surgical treatment provided if needed.

Enhancing Healthcare Team Outcomes

PSS can result in significant ocular hypertension. With recurrent episodes of rising intraocular pressure over a protracted period, significant permanent optic nerve damage occurs. Other complications of this disease have been described earlier on. Patients suffering from PSS may be misdiagnosed, and this poses a challenge. In some cases, the patient does not present to the clinic because of the absence of distressing symptoms. The patient may be first seen at the primary care service center. Therefore, for prompt diagnosis of PSS, the optometrist, often the primary eye care provider, could be the first member of the healthcare team to see the patient and, as such, should be aware of the diagnosis of PSS. The optometrist should appropriately refer to the ophthalmologist to control IOP and intraocular inflammation. During disease-free intervals, the optometrist may monitor the patient, with IOP being a regularly monitored parameter.

The patient will require careful monitoring to detect optic nerve damage, and imaging of the optic nerve and other ancillary investigations may need to be performed by the ophthalmic technician. Cases of frequent recurrence and inflammation not responsive to topical steroids require aqueous biopsy and PCR analysis to screen for CMV and other viral DNA. Performing this essential investigation will require the help of a laboratory scientist. Since PSS can result in known complications, the services of different ophthalmic subspecialists will be necessary, such as uveitis, glaucoma, cataract, and cornea specialists.

This team must collaborate effectively for the improved outcome of the PSS patient. Furthermore, compliance with medications is essential to achieve control of the IOP and inflammation. The pharmacist should emphasize this need for compliance to the patient at the point of dispensing medications. The patient should also be counseled on the appropriate technique for instillation of topical medications by the pharmacist and ophthalmic nurse and informed of the consequence of non-use of the eye drops.

Lastly, as some PSS patients have more frequent acute attacks than others, this group of patients will require closer monitoring. Eyedrops for such patients may be tapered off slowly. Therefore communication to such patients on when to return to the clinic should be unambiguous. The counselor should ensure that the PSS patient can be seen between appointments if they experience an attack before the next appointment.