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Middle East African Journal of Ophthalmology, Volume 21, Number 3, July - September - 2014 251

INTRODUCTION

Uveitis is a sight-threatening inflammatory eye condition that involves the uveal tissue. It may occur as an isolated

idiopathic condition, as a complication of infection, or in association with systemic inflammatory disease. The limited experience of general ophthalmologists in handling uveitis cases may delay initiating proper treatment leading to devastating visual consequences.

The aim of this review is to help ophthalmologists develop a systematic clinical approach to diagnosing emergent uveitis based on medical history, clinical findings and investigations. Detailed management of the acute phase of each disorder is also discussed. Long-term therapy of diseases or their complications is beyond the scope of this review.

EVALUATION OF UVEITIC EMERGENCY

To recognize and diagnose the most important causes of an emergency related to uveitis, one should opt for a viable, well-established systematic approach that is tailored to cover all potential conditions in a simple manner. The majority of uveitis

cases present with sufficient time to complete investigations before initiation of therapy. However, uveitis emergencies are an exception, as delay in treatment can lead to the optic disc or macular damage causing permanent visual impairment. Conditions that require immediate treatment include acute retinal necrosis (ARN), progressive outer retinal necrosis (PORN), cytomegalovirus (CMV) retinitis, ocular toxoplasmosis, Behçet’s disease (BD), Vogt-Koyanagi-Harada (VKH) disease and sympathetic ophthalmia (SO). The different causes of decreased vision of each condition is summarized in Table 1.

The essential signs and symptoms of uveitis include: Pain, redness and photophobia; floaters and blurry vision and; scotoma. The classic sign of circumcorneal redness is associated with acute uveitis. Circumcorneal injection is due to inflammation of the deeper iris or ciliary body inflammation. Acute referred pain radiating over a large area of the trigeminal nerve is common. Pain usually results from acute inflammation in the region of the iris or from secondary glaucoma. Photophobia is present in the affected eye if the light is shined in the unaffected eye (true photophobia). This sign is helpful to differentiate uveitis from conjunctivitis. Redness, pain and photophobia usually indicate an episode of anterior uveitis.

A Systematic Approach to Emergencies in Uveitis

Hassan A. Al‑Dhibi1, Ammar M. Al‑Mahmood2, J. Fernando Arevalo1,3

1Division of Vitreoretinal Surgery and Uveitis, 2Division of Anterior Segment and Uveitis, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia, 3Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA

Corresponding Author: Dr. Hassan A. Al‑Dhibi, Division of Vitreoretinal Surgery and Uveitis, King Khaled Eye Specialist Hospital, P. O. Box 7191, Riyadh 11462, Kingdom of Saudi Arabia. E‑mail: [email protected]

ABSTRACT

Uveitis is a common cause of preventable blindness although it is consider a sight‑threatening condition particularly in cases with posterior segment inflammation. To deal with emergency conditions in uveitis, we must aware of the essential signs and symptoms that reflect a true uveitic emergency. Failure to recognize these essential signs and symptoms of a true uveitic emergency may result in a devastating visual outcome. This review provides general ophthalmologists and residents, clinical guidelines for the main uveitic entities that require immediate recognition and urgent intervention in the emergency room to prevent severe permanent visual loss.

Key words: Acute Retinal Necrosis, Behçet’s Disease, Cytomegalovirus Retinitis, Emergency, Ocular Toxoplasmosis, Progressive Outer Retinal Necrosis, Sympathetic Ophthalmia, Uveitis, Uveitis, Vogt‑Koyanagi‑Harada Disease

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Al-Dhibi, et al.: A systemic Approach to Emergencies in Uveitis

252 Middle East African Journal of Ophthalmology, Volume 21, Number 3, July - September - 2014

Floaters and blurry vision are usually presenting symptoms of posterior uveitis. In acute uveitis, a decrease in vision might be caused by a refractive error secondary to macular edema, hypotony or a change in lens position. Retinitis or retinochoroiditis directly affecting the macular function may be another possibility. Floaters indicate vitreous involvement due to inflammatory cells, posterior vitreous detachment or vitreous condensation. Scotoma indicates focal retinal damage or optic nerve involvement which is commonly caused by uveitis associated to ocular toxoplasmosis.

A targeted medical history and clinical examination are helpful in categorizing patients with uveitic emergencies especially when approaching a group of disorders, which are quite similar. Pertinent information on history taking includes sex, age, general health status, past medical history and previous history of surgery or penetrating trauma [Diagram 1].

Differentiation of infectious or noninfectious etiology is fundamental for appropriate treatment. For example, immunosuppression for an infectious uveitis may result in permanent visual loss. A history of organ transplant, immunosuppressive therapy or immune status in general may be essential in certain disorders to make the diagnosis. Diagnosis of uveitic emergencies is made clinically and is confirmed with fundus fluorescein angiography (FFA) and laboratory evaluation. Anterior segment examination and a dilated fundus examination are essential for diagnosis. Important findings include the presence or absence of vitreous opacity, retinitis, exudative retinal detachment, retinal scarring and location of retinitis [Diagram 2]. The basic uveitis work-up should include complete blood count, differential, liver function test, renal profile, rapid plasma reagin, tuberculin skin test, chest X-ray and urine analysis. These laboratory evaluations are mandatory for all patients prior to treating any of the conditions discussed below.

DIAGNOSIS AND MANAGEMENT OF SPECIFIC UVEITIC EMERGENCIES

A list of all uveitic emergencies is beyond the scope of this article. Here, we present the most common emergencies in our clinical experience.

ARNARN is an uncommon but devastating, potentially blinding disorder that occurs most commonly in immunocompetent individuals of either gender at any age. Varicella zoster virus, herpes simplex virus, CMV and Epstein-Barr virus have been implicated in the pathogenesis of ARN.1-7 Patients may complain of red eye and decreased vision. ARN is characterized by focal, well-demarcated areas of retinal infiltrate and necrosis located in the peripheral retina, rapid, circumferential progression of necrosis, evidence of occlusive vasculopathy and prominent inflammatory reaction in the vitreous and anterior chamber [Figure 1].8 Retinitis progresses rapidly in the absence of treatment and in classic cases spread to the posterior pole. Diagnosis of ARN is primarily based on typical clinical features. Polymerase chain reaction (PCR) analysis of intraocular fluids is helpful in detecting virus type and establishing the diagnosis of ARN in atypical cases.6-9

The goal of therapy is to speed the resolution in the affected eye and to prevent spread of the disease to the contralateral eye. However, treatment does not reduce the rate of retinal detachment in the affected eye. The treatment protocol is intravenous (IV) acyclovir 10 mg/kg every 8 h for 10 days followed by oral valacyclovir 1 g TID or acyclovir (800 mg 5 times/day), for up to 6 weeks from the onset of infection. This duration of therapy is important as involvement of the second eye typically starts within 6 weeks of initial infection.

The lesions may progress during the first 48 h of treatment. Stabilization and early regression of retinitis is usually seen within 4 days. 10-12 Although the use of systemic steroids is controversial, we recommend initiating oral prednisone 48 h following the initiation of anti-viral therapy at a dose of 1 mg/kg/day for 1-2 weeks followed by a taper over 2-6 weeks. Our limited experience (few cases) with intravitreal injection of ganciclovir (0.2-2.0 mg/0.1 mL) or foscarnet (1.2 mg/0.1 mL) to treat ARN has resulted in successful outcomes to date. Similar results have been reported.13

Prophylactic barrier laser photocoagulation posterior to active retinitis to wall off or prevent subsequent rhegmatogenous retinal detachment (RRD) prior to discharging a patient is recommended [Figure 2]. Patients are seen daily and are examined every few weeks to months for the following year. A careful fundus evaluation with scleral depression is performed at each visit to rule out retinal holes that may lead

Table 1: Causes of decreased vision in different uveitic emergencies

Disorder Causes of decreased visionAcute retinal necrosis Optic neuropathy, vascular occlusion,

retinal detachmentProgressive outer retinal necrosis

Vascular occlusion, retinal detachment

Cytomegalovirus retinitis Maculopathy, optic neuropathy, retinal detachment

Ocular toxoplasmosis Macular retinochoroidal scaring, glaucoma, macular edema, epiretinal membrane, choroidal neovascularization, retinal detachment

Behçet’s disease Maculopathy, optic disc atrophy, cataract, glaucoma, retinal ischemia, retinal detachment, phthisis bulbi

Vogt‑Koyanagi‑Harada disease

Exudative retinal detachment, maculopathy, sub‑retinal neovascular membrane and fibrosis

Sympathetic ophthalmia Exudative retinal detachment, cataract, glaucoma, maculopathy





Diagram 1: Pertinent medical history in cases of uveitic emergency. PORN = Progressive outer retinal necrosis, ARN = Acute retinal necrosis, CMV = Cytomegalovirus, VKH = Vogt‑Koyanagi‑Harada syndrome, SO = Sympathetic ophthalmia

Diagram 2: Clinical diagnostic approach to uveitic emergencies based on ocular manifestations. ARN = Acute retinal necrosis, VKH = Vogt‑Koyanagi‑Harada syndrome, CMV = Cytomegalovirus, SO = Sympathetic ophthalmia, PORN = Progressive outer retinal necrosis





to a detachment. If the retinitis crosses the margin of the laser demarcation, consider additional laser therapy.14

Currently, there are no established treatment guidelines for ARN. Current treatment trends vary widely, including single agents or combinations of oral, IV and intravitreal agents.12,15

The prognosis of untreated ARN is associated with poor visual outcome due to retinal detachment or optic atrophy.12

PORNPORN usually occurs in immunocompromised patients. A sudden decrease in vision with visual field restriction are the main complaints. PORN is characterized by a minimal non-granulomatous anterior uveitis without vitritis. It is associated with a necrotizing retinitis starting at the posterior pole and spreading toward the peripheral retina [Figure 3]. Unlike ARN, retinal vasculitis and optic neuritis are uncommon. Retinitis is usually bilateral. 16-18 Similar to ARN, PORN is a clinical diagnosis. PCR analysis of intraocular fluids is helpful in detecting virus type and establishing the diagnosis of PORN in atypical cases.6,7,9 This disease frequently leads to rapid bilateral blindness due to infection or secondary retinal detachment. Treatment is similar to ARN. Acyclovir alone appears to be relatively ineffective. The current recommendation is to combine acyclovir with ganciclovir and foscarnet.19,20 Recently, some success has been noted with intravitreal gangciclovir compared with systemic therapy alone.21

CMV retinitisCMV retinitis can present in a fulminant form or an indolent variant. Fulminant CMV retinitis is the classic appearance of

the disease. Symptoms depend on the location of the retinitis and most patients present with complaints of decreased vision and floaters. CMV retinitis is characterized by dense, white confluent opacification of the retina along the vessels with retinal hemorrhage and vascular sheathing, but without any central atrophic lesions [Figure 4]. The indolent form of CMV retinitis is associated with granular foci of retinal necrosis with a central atrophic zone, fewer hemorrhages and less vascular sheathing.18,22,23 The diagnosis of CMV retinitis typically is based on the clinical findings seen on a dilated fundus examination, Goldmann-Witmer co-efficient and Viral PCR from ocular fluid or tissue may be useful in unclear cases.6,7,9

IV ganciclovir and foscarnet are used individually or in combination. Responsive cases may be switched to oral therapy (ganciclovir 1000 mg PO TID or valganciclovir 900 mg PO QD as maintenance therapy). However, currently, most patients are treated with oral therapy with valganciclovir 900 mg PO BID for 21 days then (if retinitis is resolving) 900 mg PO QD as maintenance therapy. Patients with progressive retinitis not responding to the treatment above may benefit from intravitreal antiviral injections and implants. Macula sparing RRD may be treated with laser demarcation. Pars plana vitrectomy with injection of silicone oil is indicated for detachments involving the macula.14

Figure 4: Cytomegalovirus retinitis. (a) Fundus photograph showing retinal whitening (retinitis), hemorrhages and vasculitis

Figure 1: Acute retinal necrosis, (a and b) retinal vasculitis with confluent and stellate peripheral areas of cream‑colored retinal necrosis

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Figure 3: Progressive outer retinal necrosis. (a) Multifocal areas of retinitis (b) homogeneous confluent areas of deep retinal opacification involving the macula with cherry‑red spot and absence of vitreous inflammation (Courtesy of Andre Cury, MD)

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Figure 2: (a and b) Fundus photographs of prophylactic barrier laser for acute retinal necrosis

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Prior to highly active antiretroviral therapy (HAART), the prognosis for patients with CMV retinitis was guarded and many individuals lost vision. However, any damage to the retina caused by CMV retinitis is permanent regardless of HAART; so early detection of the disease remains critical to preserve the vision.22

Ocular toxoplasmosisA recent survey of uveitis presenting at a university center in Saudi Arabia found 8.2% of cases were due to ocular toxoplasmosis.24 The typical lesion is necrotizing retinitis. In primary acquired retinal disease, the retinitis is present in the absence of a previous scar. However, in reactivations the lesion will occur adjacent to a previous scar with variable pigmentation [Figure 5a and b]. The usual presentation is sudden onset of floaters, visual loss, pain, or photophobia. Vitritis is commonly seen overlying the active lesion and might be severe enough to prevent proper visualization of the lesion (“headlights in the fog”).25,26 FFA in the early phase shows the focus of inflammation as a hypofluorescent area followed by progressive leakage from the surrounding retinal vessels and the edge of the main lesion, resulting in a hyperfluorescent area [Figure 5c and d]. The diagnosis of ocular toxoplasmosis is made mainly by clinical ocular examination, In atypical cases, serum and aqueous humor or vitreous anti-Toxoplasma titers of IgM and IgG may be helpful to support the diagnosis. PCR of aqueous and vitreous samples is another tool with high sensitivity and specificity.27,28

In cases of mild peripheral retinochoroiditis occurring in immunocompetent patients, the condition is usually self-limited and requires observation only. Treatment should be considered for cases with lesions in the macula, within 2-3 mm of the disc, threatening a large retinal vessel, vitritis severe enough to cause a two-line decrease in vision, or disease in an immunocompromised patient. Classic first-line therapy includes clindamycin 300 mg PO QID, trimethoprim/sulfamethoxazole (160 mg/800 mg) one

tablet PO BID with or without clindamycin and prednisone. We recommend the use of oral prednisone 48 h following the initiation of anti-toxoplasma therapy at a dose of 1 mg/kg/day. Prednisone should be tapered prior to stopping anti-toxoplasma therapy. The duration of therapy is generally 4-6 weeks. Consider extended therapy in immunocompromised patients.14 The treatment regimen has changed little since it was originally introduced and there is a lack of consensus in treatment. For example, ophthalmologists in developing countries (e.g., India and our preference) prefer using trimethoprim/sulfamethoxazole antibiotic therapy whereas ophthalmologists in developed countries (e.g.,Germany and the United States) prefer pyrimethamine/sulfadiazine.29

BDBD is more prevalent in regions along the silk Road, from the Mediterranean to the far East.30 Approximately 12.5% of uveitis cases from a Saudi Arabian university center were due to BD.24 Uveitis in BD is always non-granulomatous and eventually involves both the anterior and posterior segment of the eye bilaterally. Ocular symptoms may include periorbital pain, redness, photophobia and sudden loss of vision. The typical anterior uveitis in BD presents with endothelial dusting, minimal fibrinous reaction despite the severity of inflammation and a hypopyon that shifts freely with head position. The hypopyon may be “hot” or “cold,” (i.e., with or without ciliary injection; [Figure 6a]). Diffuse vitritis is a sign of posterior segment involvement which has a prognostic implication. Retinal veins are predominately affected. Perivenous cuffing or sheathing is a sign of acute periphlebitis. Hemorrhagic periphlebitis is also present when there is profound damage to the vascular wall or in association with occlusive retinal periphlebitis.31 Retinitis is another posterior segment finding that appears with rapid onset as yellow-white retinal infiltrates in varying number and distribution that disappear in several days without visible scaring and may be associated with retinal hemorrhages [Figure 6b].32

BD is a clinical diagnosis that based on systemic and ocular findings, Initial management may require hospitalization. The treatment includes IV methylprednisolone 1 g administered daily for 3 days followed by prednisone PO 1 mg/kg/day. We also recommend administration of two immunosuppressants as initial therapy such as cyclosporine 2-5 mg/kg/day PO on two

Figure 5: Toxoplasma retinochoroiditis. (a) Focal area of retinochoroiditis (b) recurrent retinochoroiditis around an old scar associated with perivasculitis (c and d) large focal retinochoroiditis and corresponding fluorescein angiography revealing central area of hypofluorescence surrounded by a ring of hyperfluorescence

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Figure 6: Behcet’s disease. (a) Hypopyon (b) obliterative necrotizing retinal vasculitis with multifocal chalky white retinitis

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divided doses per day and either azathioprine (1-2 mg/kg/day) or mycophenolate mofetil 500 mg BID. In resistant cases, we recommend starting patients on infliximab (5 mg/kg IV) on week 0, 2, 6, 8 from initiation of the treatment. Therapy should be continued every 8 weeks. A pilot study of intravitreal infliximab has reported successful resolution of most symptoms in patients whom systemic administration of infliximab is not feasible or contraindicated.33 Visual impairment in BD occurs in 25% of involved eyes. Accordingly early detection of ocular disease and treatment is vital.34,35

VKH diseaseVKH disease is a bilateral inflammatory disease, although it might present in one eye prior to the fellow eye. In a survey of uveitis presentation patterns in Saudi Arabia, 19.4% of uveitis patients were diagnosed with VKH.24 The most common symptom is an acute bilateral decrease in vision. It presents as a granulomatous inflammation, with mutton fat keratic precipitates (KPs) and iris nodules. Posterior pole involvement presents with hyperemia and swelling of the optic disc, vitritis and multifocal exudative retinal detachments [Figure 7a-b]. FFA shows multiple hyperfluorescent pinpoint areas (“starry night” appearance) with late leakage and pooling under a detached retina with a hot disc [Figure 7c-e]. As the disease progresses, depigmentation of the fundus becomes apparent (“Sunset glow” fundus).18,36

We recommend hospitalization in the initial management of this disease. The treatment includes IV methylprednisolone 1 g administered daily for 3 days followed by prednisone 1-2 mg/kg PO QD to be tapered slowly over 9-12 months to a dose of 10 mg PO QD. We also recommend administration of immunosuppressant at initial therapy in the form of either azathioprine (1-2 mg/kg/day) or mycophenolate mofetil 500 mg BID. If two medications are not sufficient to control inflammation

add cyclosporine 2-5 mg/kg/day PO as two divided doses. A topical steroid such as prednisolone acetate 1% is recommended for anterior uveitis. A prospective study of mycophenolate mofetil combined with systemic corticosteroids for acute uveitis reported statistically significantly lower recurrent inflammation and a 35% lower incidence of complications with combined therapy versus corticosteroid monotherapy.37 The authors of this study concluded that combined therapy as initial therapy reduced the recurrence of uveitis and significantly improved visual outcome.37

SOSO is a bilateral, diffuse granulomatous panuveitis that occurs following penetrating trauma or surgery in one or both eyes. The disease can occur as early as a week and as late 6 decades following ocular trauma or surgery.38,39 Approximately 90% of patients develop the disease within 1 year of injury.34 The true incidence and prevalence of SO are hard to establish.40 SO represents up to 2% of all uveitis cases and data from a British/Irish study reported that SO affects 0.03/100,000 persons/year.40,41 Patients may present with symptoms ranging from mild to significant visual loss with associated pain, watering and photophobia in the sympathizing eye. Anterior segment examination shows a granulomatous reaction with mutton fat KPs in the corneal endothelium. Posterior or peripheral anterior synechiae may occur. The posterior segment generally reveals moderate to severe vitritis, papillitis and mid-equatorial yellowish-white choroidal lesions (Dalen-Fuchs nodules) and exudative retinal detachment may also develop [Figure 8a].42 FFA shows multiple hyperfluorescent areas in the venous phase with late leakage and pooling beneath areas of the exudative retinal detachment [Figure 8b].

Treatment of SO starts with prevention. Enucleation of a (inciting) blind, traumatized eye before a sympathetic reaction can develop (usually considered within 14 days of the trauma). The main treatment for established cases is steroids. Topical steroids, preferably prednisolone acetate 1%, administered every 1-2 h is recommended. We also recommend periocular or intravitreal steroids (e.g., subconjunctival triamcinolone acetonide 40 mg in 1 mL) and starting systemic steroids

Figure 7: Acute bilateral Vogt‑Koyanagi‑Harada. (a and b) Fundus photographs showing hyperemic discs, multiple serous retinal detachments of the posterior pole of both eyes; (c and d) corresponding fluorescein angiogram of B showing multiple pinpoint areas of hyperfluorescence in the venous phase (c) and pooling of fluorescein beneath areas of serous retinal detachments in late phase (d)

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Figure 8: Sympathetic ophthalmia. (a) Fundus photograph showing hyperemic disc, exudative retinal detachment, multifocal choroditis. (b) Relevant fluorescein angiogram with multiple areas of hyperfluorescence that leak under a neursensory retinal detachment with blocked fluoresce at the level of the retinal pigment epithelium

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(e.g., prednisone 1 mg/kg PO QD). Steroids are tapered slowly as the condition improves and maintained for 3-6 months after all signs of inflammation have resolved. Systemic immunosuppression might be considered if the condition does not respond to steroids.14

ETIOLOGY OF EMERGENT UVEITIS IN SAUDI ARABIA

The prevalence of uveitis seems to differ in different regions of the world.37 This difference is partially due to the pathogens in the region.43 Studies on the prevalence of uveitis in Saudi Arabia have been conducted in Riyadh and the surrounding region.24,44 From the available publications and our retrospective survey of uveitis (Dhibi et al. KKESH Uveitis Survey Study Group. International Ocular Inflammation Society, Uveitis Society of India and International Assembly of Ocular Inflammation Societies. Goa, India. November 2011. Unpublished data), we present the chronologic prevalence of the types of uveitis discussed above. There were no reported cases of ARN from a 2002 study of the causes of uveitis.44 However a study24 published in 2010 reported 2% of eyes (out of 600 eyes) with uveitis associated to ARN and our survey found 1.35% cases of uveitis (out of 445 cases of panuveitis) due to ARN. Data on uveitis due to PORN are unavailable. A study published in 200244 indicated 6.5% (of 200 patients) of patients with uveitis were due to toxoplasmosis, in a study in 201023 8.5% of eyes with uveitis (out of 600 eyes) were due to toxoplasmosis. Our retrospective study of 888 uveitis cases that presented to the King Khaled Eye Specialist Hospital from 2001 to 2010 (unpublished data), found 3.3% of cases presenting with uveitis were due to toxoplasmosis. Uveitis associated to CMV was present in 0.5% of cases in the 2002 survey44 and in 1.6% of 63 cases of posterior uveitis in our study. The 200244 survey indicated 6.5% of uveitis cases were due to BD, the 201024 survey reported 12.5% of eyes with uveitis (of 600 eyes) were due to BD. In our survey, we found 14.6% of eyes (of 888 eyes) presenting with uveitis had underlying BD. Uveitis due to VKH occurred in 2.5% of cases in the 200244 survey, 19.4% of eyes in the 201024 survey and 22% in our survey. Uveitis due to SO occurred in 0.5% of eyes in the 200238 survey, in 0.9% of eyes in the 2010 survey24 and in 1.12% of 445 panuveitis cases in our survey.

CONCLUSION

Uveitic emergencies are sight-threatening disorders. Cases of emergent uveitis require immediate intervention to prevent visual loss. A high index of suspicion with prompt recognition of specific diseases and timely initiation of proper therapy are imperative. Clinically, this can be achieved with use of various IV, intravitreal and systemic. Other cases may require surgical intervention. There is a lack of consensus in treating some types of uveitis. The appropriate medical therapy in each case will be dependent on the type of uveitis and the associated systemic

disease. Knowledge of the etiology of the common uveitic emergencies and the incidence in different regions may aid in timely diagnosis and therapy.

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Cite this article as: Al-Dhibi HA, Al-Mahmood AM, Arevalo JF. A systematic approach to emergencies in uveitis. Middle East Afr J Ophthalmol 2014;21:251-8.

Source of Support: Nil, Conflict of Interest: None declared.



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Review Article A Systematic Approach to Emergencies in Uveitis · 2014-11-25 · VKH = Vogt‑Koyanagi‑Harada syndrome, SO = Sympathetic ophthalmia 9LWUHRXV 2SDFLW\ 3UHVHQW 3HULSKHUDO