Posner-Schlossman Syndrome European Study Group: study protocol and baseline patients characteristics of a multicentre study.

BACKGROUND: The aim of the Posner-Schlossman Syndrome European Study Group (PSS-ESG) is to acquire a comprehensive dataset of European patients with PSS. Here, we present the first report on the study protocol and the clinical findings of the patients at baseline. METHODS: The PSS-ESG is a retrospective, multicentre study designed to evaluate patients with PSS. The study, designed and driven by a European Expert Committee includes three datasets: (1) the baseline, (2) the follow-up and (3) the intraocular pressure (IOP)/glaucoma dataset. RESULTS: A total of 11 centres adhered to the PSS-ESG and 107 patients were included (68 males, 39 females) mostly Caucasian (93.4%). At uveitis onset, the patient's age ranged between 11 and 76 years, (mean age: 42±15 years).Best-corrected visual acuity was >0.5 in 80.3% of the eyes, IOP was >40 mm Hg in 44% of the eyes. Keratic precipitates were found in 78.5% of the eyes. No flare or cells in anterior chamber were detected in 56% and 53% of the cases, respectively. PCR analysis on aqueous sample was positive for cytomegalovirus-DNA in 50.6% out of the 81 tested patients. CONCLUSIONS: The PSS-ESG is the first multicentre study aimed to collect a comprehensive dataset of patients with PSS in non-Asian countries. A middlde-aged Caucasian male with a low-grade anterior chamber inflammation, keratic precipitates, preserved visual acuity and marked increased in IOP seemed to be the standard PSS patient across the 11 uveitis and glaucoma centres participating in the PSS-ESG.

PREDICTIVE ROLE OF SWEPT SOURCE OCT ANGIOGRAPHY IN RELAPSING VOGT-KOYANAGI-HARADA DISEASE.

PURPOSE: The purpose of this study was to describe the optical coherence tomography (OCT) angiography findings as a predictive role in the chronic relapsing stage of Vogt-Koyanagi-Harada disease and its comparison with other imaging modalities such as fluorescein angiography, indocyanine-green angiography, and spectral domain OCT. METHODS: A 37-year-old woman from Bangladesh was diagnosed with Vogt-Koyanagi-Harada disease. She was evaluated 8 months before for a routine examination when she was in clinical remission. Full ophthalmic evaluation with multimodal imaging and OCT angiography was performed. RESULTS: Ophthalmic evaluation was unremarkable. Spectral domain OCT disclosed increased choroidal thickness in both eyes while swept source OCT angiography imaging showed choroidal flow voids well-matching hypofluorescent round lesions found by indocyanine-green angiography. A week later the disease reactivated. CONCLUSION: Optical coherence tomography angiography may provide novel insights into inflammatory activity of the choroid and potentially have a predictive role in relapsing Vogt-Koyanagi-Harada disease.

Intraocular Tuberculosis: A Challenging Case Mimicking Wet Age-Related Macular Degeneration.

An otherwise healthy 72-year-old Chinese patient diagnosed with exudative age-related macular degeneration and decreased vision in left eye was fully investigated. The retrospective analysis of past multimodal imaging revealed bilateral severe choroidal neovascularization and choroiditis associated with a positive tuberculin skin testing and interferon-gamma release assay (QuantiFERON-TB Gold - Cellestis®, Chadstone, VIC, Australia) suggestive of latent ocular tuberculosis. The variable presentation and tests' results interpretation represent the greatest limitations in understanding and treating intraocular TB (IOTB). This may present without any other systemic symptoms, the intraocular tissues are of limited access to biopsies and other tests, including imaging and immunological tests, are of relative value. This case highlights how variable may be the presentation of IOTB, which can be easily misdiagnosed leading to a delayed treatment and worse prognosis.

Standardization of red flags for referral to rheumatologists and ophthalmologists in patients with rheumatic diseases and ocular involvement: a consensus statement.

Ocular involvement is a common manifestation of inflammatory rheumatic diseases, often requiring a multidisciplinary collaboration between rheumatologists and ophthalmologists. The aim of this study was to standardize "red flags" for referral for rheumatologists and ophthalmologists using a Delphi consensus for the management of rheumatic diseases with ocular involvement. The scientific board comprised 11 Italian hospital-based rheumatologists (N = 6) and ophthalmologists (N = 5). A systematic review identified potential red flags for referral. The panel developed 19 statements consisting of (a) referral from ophthalmologist to rheumatologist (b) referral from rheumatologist to ophthalmologist and (c) overarching principles relating to multidisciplinary roles/goals and management. Voting was performed anonymously using an online Delphi method. Each participant expressed a level of agreement on each statement using a 5-point scale (1="strongly disagree"; 5="strongly agree"). Total cumulative agreement was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"), consensus defined as ≥ 80% cumulative agreement for each statement. Positive consensus among 11 participants was reached for 15/19 (78.9%) statements. Statements not reaching consensus were discussed in a face-to-face meeting prior to the second vote (10 participants). Positive consensus was reached for all 19 statements, with final total cumulative agreement of 90-100%. This is the first Delphi consensus undertaken to standardize red flags for referral to rheumatologists and ophthalmologists for patients with rheumatic diseases and ocular involvement.

Regression of unusual optic disk neovascularization after adalimumab in a case of juvenile idiopathic arthritis-related uveitis.

We report the case of a 9-year-old girl with a severe and uncommon presentation of juvenile idiopathic arthritis-related posterior uveitis, with neovessels at the optic disk associated with peripheral vasculitis. After two failed bevacizumab intravitreal injections, uveitis responded to systemic adalimumab administration, with a complete and long-term remission of ocular inflammation.

Adalimumab for treatment of severe Behçet's uveitis: a retrospective long-term follow-up study.

OBJECTIVES: Behçet's disease (BD) is a chronic multisystem inflammatory disorder associated to uveitis that may represent a serious sight-threatening condition. The purpose of the present study is to assess the effectiveness of adalimumab as new strategic therapeutic approach in patients affected by severe Behçet's uveitis. METHODS: Clinical data from twelve selected patients (22 eyes) were retrospectively analysed. All patients received 40 mg of adalimumab subcutaneously, once every 2 weeks, in addition to traditional immunosuppressive on-going therapy and eight of them were switched to adalimumab after failure of infliximab therapy. Primary outcome measures included ocular inflammatory activity, frequency of uveitis attacks and steroid-sparing effect. Secondary outcomes were changes of best-corrected visual acuity (BCVA), impact on traditional immunosuppressive therapy and occurrence of adalimumab-related side effects. RESULTS: Mean age of patients (11 males and 1 female) at the onset of disease was 24.34 years (±8.62 SD). Ocular involvement resulted bilateral in 83% of cases and mainly consisted in panuveitis (68% of eyes). After mean follow-up of 21 months (±9.63 SD) all patients but one (92%) achieved uveitis remission with BCVA improvement at least in one eye. Average uveitis attacks decreased from 2 to 0,42 during adalimumab (p<0.001) and daily-steroid dose was tapered in all adalimumab responders up to suspension in seven of them. No patient developed related side effects during adalimumab administration. CONCLUSIONS: Our results demonstrate that adalimumab is a very effective and safe option for treatment of patients with severe and resistant Behçet's uveitis, providing an appropriate and long-term control of ocular inflammation.

Adalimumab for juvenile idiopathic arthritis-associated uveitis.

PURPOSE: To assess the long-term outcomes and complications of patients with uveitis from juvenile idiopathic arthritis (JIA) treated with adalimumab. METHODS: Prospective interventional case series. All patients who underwent treatment with adalimumab for JIA and anterior uveitis were prospectively included in the study. The anterior chamber inflammation was evaluated according to the Standardization of Uveitis Nomenclature criteria. RESULTS: Twenty-one patients (16 females, five males, 38 eyes) were included in the study. Mean age of patients at referral was 11.1 ± 3.8 (5-17) years. Before initiation of treatment, mean duration of arthritis was 7.0 ± 5.5 (median, 6) months, mean duration of uveitis was 7.0 ± 4.4 (median, 7) months. Oligoarticular arthritis was present in 15 cases (71 %), polyarticular arthritis in six cases (28 %). After a mean follow-up of 18.2 ± 7.7 (9-41) months, resolution of anterior chamber inflammation was obtained in 29/38 eyes (76 %). The anterior uveitis flare rate during the 12 months prior to enrollment was 1.6 ± 0.4/year, and was reduced during adalimumab treatment to 0.7 ± 0.3/year (p<0.001). A significant decrease of the number of relapses/month was present after onset of treatment with adalimumab (0.18 ± 0.2 before versus 0.02 ± 0.1 after treatment onset, p<0.001). No significant correlation was found between relapse number and age, sex, type of JIA and doses of previous steroid treatment (p>0.05). CONCLUSION: Adalimumab showed to be effective and relatively safe for treatment of JIA-associated uveitis.

Efficacy of adalimumab in patients with Behçet's disease unsuccessfully treated with infliximab.

OBJECTIVES: To evaluate the clinical response after switching from infliximab to adalimumab in patients with Behçet's disease (BD). METHODS: In this ongoing, prospective, longitudinal and observational study, data were collected on efficacy and safety of every patient with BD beginning anti-TNF therapy in the last 8 years. The present analysis was restricted to patients who were switched to adalimumab after failing or not tolerating infliximab. RESULTS: A total of 69 patients with BD have been treated with infliximab so far. Seventeen of these (25%) have been switched to adalimumab for lack or loss of efficacy or infusion reactions. In 10 out of these 17, the main manifestations requiring switching were the mucocutaneous lesions, in 4 retinal vasculitis and in 3 the neurological involvement. Of the 17 treated patients, 9 showed sustained remission of the disease and 3 a good response. No side effects were observed in any patient. CONCLUSIONS: The results of our study suggest that patients with BD showing a scarce response or adverse events to infliximab may successfully be treated with adalimumab, regardless of the reason for switching.

Successful treatment of severe Behçet's disease with infliximab in an Italian Olympic athlete.

An Italian Olympic athlete with a severe form of Behçet's disease was given infliximab. He had oral aphthosis, papulopustular and follicular lesions, and a bilateral cystoid macular edema. After the first 3 infusions, the mucocutaneous lesions disappeared and cystoid macular edema was much improved. Cyclosporine was stopped and the prednisone dose was progressively tapered until the cystoid macular edema disappeared.