Uveitis and Scleritis as a Risk Factor for Mortality.

PURPOSE: Uveitis and scleritis may be caused by local or systemic infection, or associated with noninfectious systemic inflammatory autoimmune disease. This study explored the all-cause mortality following an individual's first presentation with uveitis/scleritis. METHODS: A cross-sectional study was conducted on all uveitis/scleritis patients diagnosed by uveitis specialists and treated in a single tertiary referral center in New Zealand between 2006 and 2020(15y). Masquerade syndromes including intraocular lymphoma were excluded. Outcome measures: demographics, etiology of uveitis/scleritis, anatomical location and all-cause mortality. RESULTS: 2723 subjects were identified. Median age of onset of uveitis/scleritis was 44.9 years (Range:1.5-99.5 years). 49.6% were female. Median follow-up from diagnosis of uveitis/scleritis was 8.0 years (IQR 4.1-11.6 years) with a total follow-up of 24 443.3 subject-years. The most frequent diagnosis was idiopathic disease (30.9%), HLA-B27-positive uveitis (20.0%), and sarcoidosis (4.7%). Infectious etiologies (24.1%) were most commonly from herpes zoster virus (9.3%) and toxoplasmosis (4.3%). The age-adjusted mortality rate was higher in subjects with idiopathic disease, sarcoidosis, Fuchs' uveitis syndrome, granulomatosis with polyangiitis/ANCA-associated vasculitis, toxoplasmosis, and herpes zoster virus, when compared to HLA-B27-positive uveitis. Hazard of mortality peaked in the first seven years following diagnosis, then subsequently declined. Patients with uveitis/scleritis had a significantly higher rate of mortality compared to the general New Zealand population (IRR 1.656 p = 0.017). CONCLUSION: Infectious etiologies of uveitis/scleritis in this cohort were high when compared to other developed nations, attributable to data from a tertiary referral center treating inpatients. Potential shared inflammatory mechanisms in the eye and other organs can lead to concurrent non-ocular disease requiring systemic treatment, impacting an individual's longevity.

Etiology of hypopyon in patients presenting acutely to the emergency eye department and characteristics of hypopyon uveitis.

OBJECTIVE: To examine the etiology of undifferentiated hypopyon presenting acutely and to better characterize hypopyon uveitis. METHODS: Patients with hypopyon were retrospectively identified from presentations to the emergency eye department between January 2015 and 2022 and also from a uveitis database of 3,925 patients seen between January 2008 and January 2022. A total of 426 episodes of hypopyon occurred in 375 eyes in 359 patients, and medical records were reviewed for each patient. RESULTS: In all, 222 hypopyon episodes were due to uveitis, and 204 were due to nonuveitic causes. The most common cause of hypopyon was HLA-B27-associated uveitis in 146 patients (34.3%). The next most common causes were infectious keratitis in 125 patients (29.3%) and endophthalmitis in 63 patients (14.8%). Compared with those presenting with nonuveitic hypopyon, patients with uveitis tended to present younger (p < 0.001), were more likely to be male (p < 0.0001), had better initial and final visual acuities (p < 0.001), and had lower intraocular pressures (p = 0.030). CONCLUSION: About half of the cases of hypopyon were secondary to uveitis, most of them being associated with HLA-B27 conditions with a good prognosis, and the other half were secondary to infectious keratitis and endophthalmitis with a poor prognosis.

Acute posterior multifocal placoid pigment epitheliopathy: clinical presentation and risk of stroke and transient ischaemic attack.

BACKGROUND/AIMS: Acute posterior multifocal placoid pigment epitheliopathy is a rare but important disease that can be associated with life-threatening complications due to cerebral vasculitis. The primary objective was to determine the incidence of neurological complications and risk factors for stroke and transient ischaemic attack (TIA) associated with acute posterior multifocal placoid pigment epitheliopathy. Secondary objectives included the clinical presentation, visual outcomes and recurrence rates. METHODS: This was a multicentre retrospective case series including 111 eyes from 60 subjects presenting from January 2009 to June 2020. RESULTS: Median age at presentation was 29 years (IQR 24.7-35.1) and 36 subjects (60.0%) were male. 20 subjects (33.3%) reported a viral prodrome. Stroke and TIA were observed in seven subjects (11.7%). Older age was the only significant risk factor for stroke/TIA (p=0.042). Vision loss occurred in seven eyes, with four eyes (3.6%) having final visual acuity 6/15-6/60 and three eyes (2.7%) having visual acuity of 6/60 or worse. Recurrence occurred in 10 subjects (16.7%). CONCLUSIONS: The presence of headache cannot reliably predict those at risk of stroke/TIA. Individuals presenting with acute posterior multifocal pigment epitheliopathy should therefore undergo a clinical neurological review and work-up for cerebral vasculitis as deemed appropriate by the treating ophthalmologist and collaborating neurologist.

Epiretinal membrane in uveitis: Rate, visual prognosis, complications and surgical outcomes.

BACKGROUND: Epiretinal membrane (ERM) is a common finding in patients with uveitis that contributes to visual impairment. We describe the long-term visual acuity (VA) and morphometric progression in patients with uveitis and epiretinal membrane (ERM). METHODS: Retrospective cohort study of patients with uveitic ERM from a tertiary centre database. Multivariate analysis of risk factors for ERM progression was calculated using a marginal Cox regression model to estimate hazard ratios (HR). RESULTS: Two hundred and sixteen eyes (4%) of a total 5450 eyes with uveitis were identified to have an ERM. The most common diagnosis was idiopathic uveitis in 45 patients (28.7%), followed by sarcoidosis in 21 (13.4%), HLAB27-related uveitis in 15 (9.6%) and toxoplasmosis in 15 (9.6%). Risk factors for ERM development include age (HR 1.03), intermediate uveitis (HR 2.33), posterior uveitis (HR 1.53) and ERM fellow eye (HR 18.28). Anterior uveitis (HR 0.53) and alternating disease (HR 0.53) were protective. Median VA was 20/40 at diagnosis of ERM and 20/40 at final follow up. Progression of ERM grade occurred in 17 eyes (7.9%) during the study period. ERM peel was performed in 44 eyes (20.4%). Median VA was 20/60 and 20/40 at baseline and 12 months after surgery, respectively. Improvement in visual acuity occurred in 23 eyes (60.5%) following surgery. CONCLUSIONS: In addition to intermediate and posterior uveitis, fellow eye involvement is a strong risk factor for ERM development. In treated uveitis, the majority maintain their long-term vision and rates of ERM progression are low.

The spectrum of paediatric uveitis in New Zealand.

AIMS: To describe the aetiology, complications, treatment and outcomes of paediatric uveitis. METHODS: This was a retrospective chart review including all paediatric participants presenting with uveitis to a tertiary referral hospital in Auckland, New Zealand between January 1997 and March 2020. RESULTS: Two hundred and twenty-four eyes of 143 participants were included. One hundred and three (46.0%) eyes were found to have uveitis without the child reporting any symptoms. Non-infectious uveitis occurred in 97 (67.8%) participants and infectious aetiology occurred in 46 (32.2%) participants. One hundred and twenty-six (56.3%) eyes developed complications by final follow-up, including ocular hypertension (60 eyes, 26.8%), cataract (55 eyes, 24.6%) and glaucoma (21 eyes, 9.4%). Conventional disease modifying anti-rheumatic drugs (DMARDs) were required in 58 (59.8%) participants, and biologic disease modifying anti-rheumatic drugs in 31 (32.0%) participants with non-infectious uveitis. Participants who were younger at presentation were more likely to require a DMARD (OR 0.896 p=0.032). Vision loss of 6/15 or worse occurred in 38 (17.0%) eyes. CONCLUSIONS: Infections are an important cause of uveitis in this age group. Asymptomatic presentation and complications commonly occur. A large proportion of children with non-infectious uveitis will require steroid sparing immunosuppression.

Diabetes Mellitus Related Anterior Uveitis - An Overlooked Clinical Entity.

BACKGROUND: This study aimed to evaluate the characteristics of anterior uveitis in patients presenting with poorly controlled diabetes mellitus and with no other identifiable cause for their uveitis. METHODS: A retrospective study of 121 eyes in 89 patients who presented at Auckland District Health Board with idiopathic acute anterior uveitis and uncontrolled diabetes between September 2009 and January 2022. RESULTS: The diagnosis of diabetes mellitus was known prior to presentation in 80 subjects (89.9%) and was discovered as a result of screening tests in the remainder. Mean HbA1c at presentation was 117.3 mmol/mol. Most uveitis was severe with 3+ (30 eyes, 25.4%) or 4+ cells (30 eyes, 25.4%) in the anterior chamber. Recurrence occurred in 22 eyes (18.2%) and was associated with elevated HbA1c. The visual prognosis was good with median visual acuity at 12 months of 6/7.5. CONCLUSION: Poorly controlled diabetes can be associated with acute anterior uveitis.

Rhegmatogenous retinal detachment presentation and surgery in uveitic eyes.

BACKGROUND/AIMS: To explore the occurrence, uveitis activity, features, rate of proliferative vitreoretinopathy (PVR) and outcomes following rhegmatogenous retinal detachment (RRD) in a large tertiary referral uveitis service. METHODS: Retrospective analysis of subjects attending between 2008 and 2019. Multivariate analysis of risk factors for RRD was calculated. Nelson-Aalen plots were used to demonstrate cumulative risk of RRD. Outcomes of RRD surgery and prognostic indicators were analysed. RESULTS: Two thousand four hundred and forty-seven (2447) subjects (3516 eyes) with uveitis included. The mean follow-up was 5.7 years (19 767 eye-years); 56 eyes developed a RRD (1.6%). Thirty-two eyes had surgery in our unit. Risk factors for RRD were posterior uveitis or panuveitis (HR 3.386, p<0.001), male gender (HR 2.045, p=0.029) and infectious aetiology (HR 1.942, p=0.044). PVR was present in six (18.8%) eyes at presentation, and a further four (12.5%) developed it after the primary surgery. Final follow-up data showed 16 (50%) moderate or severe visual loss, although 29 (90.6%) had anatomical reattachment without oil in situ. CONCLUSIONS: There is a high rate of RRD in uveitis eyes. This is accompanied by high rates of PVR and redetachment. Anatomical success was high, but visual outcomes remain unpredictable.

Long term complications and vision loss in HLA-B27 uveitis.

OBJECTIVES: To evaluate the long term complications and vision loss in HLA-B27 uveitis. METHODS: Retrospective review of subjects with HLA-B27 uveitis in a public tertiary centre between January 2008 and 2020. RESULTS: 562 HLA-B27-positive subjects (834 eyes) had mean follow-up of 9.8 years (8173.2 eye-years). Median visual acuity at ten years was 0.1 logMAR (IQR 0.0-0.1). Complications occurred in 404 eyes (48.4%): posterior synechiae (39.7%), cataract (22.1%), elevated intraocular pressure (15.5%), cystoid macular oedema (6.0%). Permanent moderate vision loss ( ≤ 0.4 logMAR) due to uveitis occurred in 14 eyes (1.7%) and severe vision loss (≤ 1.0 logMAR) in 7 eyes (0.8%). Complications were more common with older age (OR 1.017 p = 0.016), chronic inflammation (OR 5.272 p < 0.001) and intermediate uveitis (OR 5.982 p < 0.001). CONCLUSIONS: Complications are frequent in HLA-B27 uveitis, especially in older subjects, chronic inflammation and intermediate uveitis. Despite this, the majority of subjects maintain good visual prognosis.

Rate of recurrence of toxoplasmosis retinochoroiditis at a tertiary eye centre in Auckland.

AIM: Our aim was to examine rate of recurrence of toxoplasmosis retinochoroiditis and risk factors for recurrence. No New Zealand epidemiological data on recurrence rates of toxoplasmosis retinochoroiditis have been previously published. METHODS: Retrospective chart review of all patients with toxoplasmosis retinochoroiditis presented to Auckland District Health Board Department of Ophthalmology between 2006-2019. RESULTS: One hundred and twenty-six eyes of 115 patients were included with a median age at initial diagnosis of 36.7 years (IQR 23.7-53.8). Fifty-nine patients were female (51.3%), and 16 patients (13.9%) were immunosuppressed. Twenty-six of the 86 patients tested (30.2%) were IgM positive at presentation. Mean follow-up was 6.1 years and 73 recurrences occurred during the follow-up period in 36 patients (31.3%). Treatment was initiated in 87.4% of cases, with oral cotrimoxazole or clindamycin the most common options. Recurrence occurred in 14.8% in the first year (95% CI 10.3%-21.0%), and the risk of recurrence was increased 2x for every previously documented recurrence (HR 2.00; p<0.001). There was no statistically significant increased risk of recurrence with age, IgM positivity, immunosuppression or macular involvement. CONCLUSIONS: Toxoplasmosis retinochoroiditis had a 14.8% risk of recurrence in the first year, with each previous recurrence increasing the risk by two-times.

Infectious Uveitis after Local Steroid and/or Methotrexate Injections at A Tertiary Referral Center in New Zealand: A Case Series.

AIM: Infectious uveitis is one of the most visually devastating causes of uveitis worldwide and accounts for 19.6% of all cases of uveitis in New Zealand. With the burgeoning use of intravitreal injections, there has been a commensurate increase in the number of injection-related complications and reports of infectious uveitis following their administration in recent years. We present a case series of four patients with infectious uveitis after local injections. METHOD: We retrospectively reviewed the data of four patients (mean age, 67.25 ± 7.58 years) who presented to the department of ophthalmology at Auckland District Health Board with infectious uveitis which occurred or worsened after local triamcinolone acetonide (TA) and/or methotrexate (MTX) injections. RESULTS: Three patients received local TA and one patient received intravitreal MTX. All patients were immunosuppressed prior to treatment. Two patients had toxoplasma chorioretinitis which worsened with local TA and one patient developed cytomegalovirus (CMV) retinitis after intravitreal TA. The last patient had syphilis retinopathy which worsened with intravitreal MTX. There were atypical presentations, as demonstrated by a case of presumed birdshot chorioretinopathy flare which tested positive for toxoplasma chorioretinitis with polymerase chain reaction (PCR). CONCLUSION: Uveitis due to infectious etiologies needs to be carefully excluded prior to the use of local steroid and/or methotrexate injections. Disease presentations may be atypical in the presence of marked immunosuppression following local therapy. Polymerase chain reaction (PCR) can play an important role in the diagnosis in this setting.

High rate of conversion from ocular hypertension to glaucoma in subjects with uveitis.

AIMS: To examine presentation, management and long-term sequelae of ocular hypertension and uveitic glaucoma. METHODS: Retrospective observational study of all subjects with uveitic glaucoma or ocular hypertension seen in Auckland uveitis clinics over the last 10 years. RESULTS: A total of 188 eyes of 139 subjects with uveitic glaucoma or ocular hypertension were included for analysis. Total follow-up was 1854.5 eye years (mean 9.9 years). The mean age at uveitis diagnosis was 49.3 years. 52.5% of subjects were male. The most common diagnoses were idiopathic uveitis (29.3%), sarcoidosis (13.3%), herpes zoster (6.9%), HLA-B27 uveitis (6.9%), tuberculosis (5.9%) and Posner-Schlossmann or cytomegalovirus (CMV) uveitis (5.3%). Median intraocular pressure (IOP) at diagnosis was 35 mm Hg (IQR 29-45). 144 eyes (77.0%) developed glaucoma during the follow-up period, of whom 41 lost some central vision due to glaucoma. Oral acetazolamide was required for IOP control in 64.5%, 50 eyes underwent trabeculectomy, 18 eyes required a tube and 6 underwent minimally invasive glaucoma surgery. CONCLUSION: Rapid progression was observed from ocular hypertension to uveitic glaucoma. Uveitic glaucoma is aggressive, with high likelihood of requiring surgical management and high risk of central vision loss. Close collaboration between uveitis and glaucoma specialists is required to maximise outcomes for these patients.

Systemic Associations of Sarcoid Uveitis: Correlation With Uveitis Phenotype and Ethnicity.

PURPOSE: To examine systemic associations of sarcoid uveitis and association with uveitis clinical phenotype and ethnicity. DESIGN: Retrospective cross-sectional study. SUBJECTS: A total of 362 subjects with definite or presumed sarcoid uveitis from Moorfields Eye Hospital, Royal Victorian Eye and Ear, and Auckland District Health Board. METHODS: Data were collected from the review of clinical notes, imaging, and investigations. Sarcoidosis was diagnosed in accordance with the International Workshop on Ocular Sarcoidosis guidelines. MAIN OUTCOME MEASURE: Diagnosis of associated systemic disease secondary to sarcoidosis. RESULTS: A total of 362 subjects with sarcoid uveitis were identified. Median age was 46 years, and 226 (62.4%) were female. Granulomatous anterior uveitis (47.8%), intermediate uveitis with snowballs (46.4%), and multifocal choroiditis (43.1%) were the most frequent clinical presentations, and disease was bilateral in 313 (86.5%). Periphlebitis was observed in 21.0%, and solitary optic nerve or choroidal granuloma in 11.3%. Lung parenchymal disease was diagnosed in 200 subjects (55.2%), cutaneous sarcoid in 98 (27.1%), sarcoid arthritis in 57 (15.7%), liver involvement in 21 (5.8%), neurosarcoid in 49 (13.5%), and cardiac sarcoid in 16 subjects (4.4%). Subjects with cardiac sarcoid were less likely to have granulomatous anterior uveitis (P = .017). Caucasian subjects were older at presentation (48 vs 41 years; P = .009), had less granulomatous anterior uveitis (26.4% vs 51.7%; P < .001), and were less likely to present with cutaneous involvement (23.1% vs 35.4%; P = .040). CONCLUSIONS: Ophthalmologists need to be aware of the systemic associations of sarcoid uveitis, in particular potentially life-threatening complications such as cardiac sarcoidosis. Differences observed in uveitis phenotype and between ethnicities require further investigation.

Rates of spondyloarthropathies vary with age and ethnicity in HLAB27 uveitis.

BACKGROUND/AIMS: To determine associations between HLAB27-positive uveitis, ethnicity and seronegative spondyloarthropathies (SpAs) in a New Zealand population. METHODS: Retrospective observational cohort study. Medical records of all subjects with uveitis at Auckland District Health Board from 2008 to 2018 were reviewed for HLAB27 status, age of presentation, ethnicity and SpA. RESULTS: In 10 years, 2567 subjects with uveitis were seen and 492 (19.2%) were HLAB27-positive. Of the HLAB27-positive subjects, 301 were male (60.3%) and median age was 37.8 years (IQR 29.7-50.0). Ethnicities were Caucasian (n=298, 60.6%), Asian (n=111, 22.6%), Maori (n=41, 8.2%) and Pacific Islander (n=38, 7.7%). Uveitis classification was anterior (n=478, 97.2%), intermediate (n=40, 8.1%), panuveitis (n=9, 1.8%) and scleritis (n=2, 0.4%). Maori or Pacific Islander ethnicity was associated with intermediate or panuveitis (p=0.003). Ankylosing spondylitis occurred in 163 subjects (33.1%); 29 (17.8%) were Maori or Pacific Islander. Subjects were younger (OR 0.982, p=0.009) and male (OR 1.980, p=0.001). There was no association with ethnicity or uveitis classification. Psoriatic arthritis (PsA) occurred in 11 subjects (2.2%). Chronic anterior uveitis was more common with PsA (27.3% vs 7.1%, p=0.023). There was no association with gender or ethnicity. Inflammatory bowel disease occurred in 19 subjects (3.8%) and reactive arthritis occurred in 14 subjects (2.8%). None developed chronic anterior uveitis (p=0.246 and p=0.227, respectively). There was no association with age at presentation, gender, ethnicity or uveitis classification. CONCLUSION: This cohort of New Zealand-based subjects with HLAB27-positive uveitis showed a difference in age and ethnicity in uveitis subtypes and SpAs.

Vision loss in anterior uveitis.

AIMS: To evaluate the long-term risk of permanent vision loss in subjects with anterior uveitis. METHODS: Retrospective study of subjects attending uveitis clinic at Auckland District Health Board and Moorfields Eye Hospital between 2008 and 2018. Main outcome measures were: best corrected visual acuity (BCVA); moderate vision loss (MVL ≤20/50); and severe vision loss (SVL ≤20/200). RESULTS: 2526 eyes of 1814 subjects were included with a mean follow-up of 6.8 years (17 235.4 eye-years of follow-up). MVL occurred in 240 eyes (9.5%) during the follow-up period, of which 97 (3.8%) had permanent MVL due to uveitis. The incidence of permanent MVL due to uveitis was 0.006 per eye-year with a cumulative risk at 10 years of 6.6% (5.2%-8.4%). The most common cause of permanent MVL due to uveitis was uveitic glaucoma (31.3%), followed by cystoid macular oedema (27.1%) and corneal scar (21.9%). SVL occurred in 80 eyes (3.2%) during the follow-up period, of which 39 (1.5%) had permanent SVL due to uveitis. The incidence of permanent SVL due to uveitis was 0.002 per eye-year with a cumulative risk at 10 years of 2.6% (1.8%-3.7%). Multivariate analysis showed older age at presentation, chronic anterior uveitis (CAU), infectious aetiology and poor presenting BCVA were all risk factors for permanent MVL due to uveitis. CONCLUSIONS: Although vision loss is an uncommon complication in anterior uveitis, the risk is greatest in those with CAU, infectious aetiology and poor presenting BCVA. Uveitic glaucoma is the most common cause of vision loss.

Ocular syphilis in Pacific peoples-are we making misdiagnoses secondary to yaws?

AIMS: To determine the demographic and clinical features of patients with ocular disease consistent with syphilis and positive treponemal serology in Auckland, and to compare patients who lived in a Pacific nation before 1960 with all other patients with regard to these features, considering a possible history of yaws infection. METHODS: Retrospective review of subjects seen in uveitis and neuroophthalmology clinics at Auckland District Health Board between January 2006 and June 2019. RESULTS: Two thousand four hundred and ninety-three subjects were reviewed in uveitis clinics during the timeframe, of whom 45 were diagnosed with syphilitic uveitis (1.8%). Mean age was 56.2±14.8 years and 34 (75.5%) were male. Ethnicity was Caucasian in 16 (35.5%), Pacific peoples in 16 (35.5%), Maori in two (4.4%), Asian in six (13.3%) and other in five (11.1%). Pacific peoples were older at presentation (p=0.001) and 75.0% were aged >60 compared to 24.1% of non-Pacific peoples (p=0.002). Comparing Pacific people born prior to 1960 (aged >60) to the rest of the cohort, older Pacific subjects had lower RPR titres (median 3 vs 32 p=0.004), less optic nerve swelling (0% vs 28.0% eyes p=0.014) and less posterior uveitis (6.25% vs 32.0% eyes p = 0.033). No difference was observed in anterior and intermediate uveitis between the groups. No difference was observed in the resolution or recurrence of inflammation between the groups. CONCLUSION: Syphilitic uveitis is common in New Zealand, occurring in 1 in 55 patients seen in consultant uveitis clinics. Clinicians should consider a history of yaws in Pacific peoples presenting with ocular inflammation and positive treponemal serology. In these cases alternative causes of ocular pathology should be included as differentials. In cases of diagnostic uncertainty, the risk of treatment versus the potentially severe sequelae of untreated syphilis need to be considered.

Outcome of peripheral iridotomy in subjects with uveitis.

BACKGROUND/AIMS: Peripheral iridotomy (PI) may be required in subjects with uveitis to manage iris bombe, seclusio pupillae and primary angle closure glaucoma. The aim of this study was to identify risk factors for failure of both laser and surgical PIs in patients with uveitis and determine survival durations. METHODS: Retrospective study of subjects with a history of uveitis undergoing yttrium-aluminium-garnet (YAG) laser or surgical PI at Auckland District Health Board over an 11-year period. Failure of PI was defined as loss of patency or recurrence of iris bombe. A mixed effects shared frailty model was constructed with PI nested within eyes nested within patients, to examine time to failure. RESULTS: 131 PIs were performed in 52 eyes of 39 subjects during the study period (111 YAG PIs and 20 surgical PIs). Median age at time of PI was 46.6 years and 60.5% of subjects were female. HLAB27 positive uveitis was the most common diagnosis (25.6% of subjects). Median survival time was 70 days for YAG PI and 11.0 years for surgical PI. On multivariate analysis, younger age at time of PI (HR 0.933, p<0.001) and iris bombe (HR 2.180, p=0.046) were associated with risk of failure. Surgical PI was associated with a lower risk of failure (HR 0.151, p<0.001) compared with YAG PI. Glaucoma developed in 19 eyes (36.5%), of which 13 required glaucoma surgery. CONCLUSION: Surgical PI had longer survival than YAG PI, and should be considered in subjects presenting with iris bombe and in young subjects with uveitis.

Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression.

IMPORTANCE: Inflammatory ocular diseases are associated with systemic disease, ocular morbidity and mortality. BACKGROUND: To examine clinical characteristics of subjects with peripheral ulcerative keratitis (PUK) and necrotising scleritis. DESIGN: Retrospective case series in a single tertiary centre over 10 years. PARTICIPANTS: All patients presenting with necrotising scleritis, PUK or scleritis with corneal infiltrates. Fifty-two eyes (41 subjects) were identified. METHODS: Demographic and clinical data analysis. MAIN OUTCOME MEASURES: Visual loss, ocular complications and mortality. RESULTS: Mean follow up was 7.0 ± 5.3 years, presentation age 51.3 ± 18.6 years, and 23 subjects (56.1%) were female. Overall, 27 eyes presented with PUK, 26 with necrotising scleritis, and 22 with corneal infiltrates associated with active scleritis. Associated systemic diagnosis was observed in 12 subjects at presentation, and in 21 subjects (51.2%) by final follow up. Recurrence occurred in 23 eyes (44.2%): 10 eyes experiencing PUK or necrotising scleritis; 13 eyes with non-necrotising scleritis. Perforation occurred in six eyes (11.5%). Moderate vision loss occurred in 10 eyes (19.2%) and severe loss in six eyes (11.5%). Five subjects (12.2%) were deceased by study conclusion, of whom four were not on immunomodulatory therapy (IMT). A marked difference in estimated survival was observed with IMT, with mean estimated survival of 10.7 years without treatment, compared to 24.7 years with IMT (P = .045). CONCLUSIONS AND RELEVANCE: PUK and necrotising scleritis represent a severe inflammatory disease with high rates of perforation, visual loss and mortality. IMT was associated with a lower rate of mortality and longer estimated survival in this group.

High rate of recurrence of herpes zoster-related ocular disease after phacoemulsification cataract surgery.

PURPOSE: To examine the outcomes of phacoemulsification cataract surgery in eyes with herpes zoster-related keratitis and/or uveitis and evaluate the risks for recurrent disease. SETTING: Public ophthalmology service, Auckland, New Zealand. DESIGN: Retrospective case series. METHODS: Patients with herpes zoster-related keratitis and/or uveitis who had cataract surgery in the ipsilateral eye were reviewed. Outcome measures were intraoperative and postoperative complications, postoperative visual acuity, and recurrent disease. RESULTS: Fifty-seven eyes of 57 patients were included. Thirty-eight patients (66.7%) had recurrent disease before cataract surgery. Intraoperative complications occurred in 8 patients (14.0%) and included posterior capsule tear in 2 patients (3.5%). Postoperative complications included intraocular pressure of 30 mm Hg or higher in 2 patients (3.5%) and central corneal edema in 8 patients (14.0%); all resolved by 1 month. Cystoid macular edema occurred in 2 patients (3.5%). The median corrected distance visual acuity at 12 months was 20/40 (interquartile range, 20/30-20/50). Corneal scarring was associated with poorer vision (P = .003). Herpes zoster recurred in 23 patients (40.4%) after surgery. An increased risk for recurrence was associated with shorter periods of quiescence (P = .029) and greater number of recurrences before surgery (P = .039). One eye was eviscerated because of the severity of the disease. CONCLUSIONS: Phacoemulsification in eyes with previous herpes zoster-related keratitis or uveitis posed a mildly increased risk for intraoperative and postoperative complications; however, herpes zoster disease recurrence after surgery was common and was severe in some cases. Consideration should be given to maximizing the period of quiescence before surgery and the potential role of antiviral prophylaxis.

Utility of Screening Investigations for Systemic Sarcoidosis in Undifferentiated Uveitis.

PURPOSE: To establish the utility of screening investigations for systemic sarcoidosis in a large cohort of subjects presenting with undifferentiated uveitis. DESIGN: Evaluation of a diagnostic test. METHODS: Retrospective review of consecutive subjects presenting to Auckland District Health Board with undifferentiated uveitis who underwent screening for sarcoidosis. RESULTS: Seven hundred nine subjects were included in the study. Systemic sarcoidosis was identified in 10.7%, and was biopsy proven in 43.4%. Sensitivity and specificity were highest for computed tomography (CT) of the chest (98.0% and 100%) and for chest radiograph (CXR; 57.6% and 100%). Serum ACE was elevated in 43 subjects, of whom 29 (67.4%) had systemic sarcoidosis. Sensitivity of serum ACE was 38.2% and specificity 97.8%, with an area under the curve (AUC) of 0.801. Lymphopenia was observed in 40 subjects, of whom 18 (45.0%) had systemic sarcoidosis. Sensitivity of lymphopenia was 23.7%, with specificity 96.5% and AUC 0.761. All subjects with elevated ACE and lymphopenia had evidence of systemic sarcoidosis. Biopsy was performed in 50 subjects, positive in 33 subjects (66.0%). Mediastinal biopsy was the most frequent (26 subjects). Skin biopsy was performed in 11 subjects (positive in 8). Only 1 subject with a positive skin biopsy had elevated ACE, lymphopenia, and bihilar lymphadenopathy on CXR. CONCLUSIONS: Sensitivity and specificity were highest for chest CT. Although CXR had excellent specificity, CXR screening alone would still miss many cases of sarcoidosis. Combined elevated ACE and lymphopenia were strongly suggestive of systemic sarcoidosis, and biopsy of skin lesions may detect patients otherwise missed by routine screening tests.