Use of corticosteroids in non-infectious uveitis - expert consensus in Taiwan.

PURPOSE: To offer consensus on the utilization of corticosteroids (CS) for treating non-infectious uveitis in the context of clinical practice in Taiwan. This entails examining the different administration methods, their advantages and disadvantages, and considering alternative treatments according to the prevailing evidence and health policies. METHODS: Ten ophthalmologists and one rheumatologist convened on December 11, 2022, to review and discuss literature on the topic. The databases explored were the Central Cochrane library, EMBASE, Medline, PUBMED, and Web of Science using relevant keywords. The search spanned from January 1996 to June 2023. After the initial results of the literature review were presented, open voting determined the final statements, with a statement being accepted if it secured more than 70% agreement. This consensus was then presented at significant meetings for further discussions before the final version was established. RESULTS: A flow chart and nine statements emerged from the deliberations. They address the importance of CS in uveitis management, guidelines for using topical CS, indications for both periocular or intravitreal and systemic therapies, and tapering and discontinuation methods for both topical and systemic CS. CONCLUSION: While CS are a cornerstone for non-infectious uveitis treatment, their administration requires careful consideration, depending on the clinical situation and the specific type of uveitis. The consensus generated from this article provides a guideline for practitioners in Taiwan, taking into account local health policies and the latest research on the subject. It emphasizes the significance of strategic tapering, the potential for alternative therapies, and the importance of patient-centric care.

Taiwan ocular inflammation society consensus recommendations for the management of juvenile idiopathic arthritis-associated uveitis.

We presented the development of a consensus guideline for managing juvenile idiopathic arthritis-associated uveitis (JIAU) in Taiwan, considering regional differences in manifestation and epidemiology. The Taiwan Ocular Inflammation Society (TOIS) committee formulated this guideline using a modified Delphi approach with two panel meetings. Recommendations were based on a comprehensive evidence-based literature review and expert clinical experiences, and were graded according to the Oxford Centre for Evidence-Based Medicine's "Levels of Evidence" guideline (March 2009). The TOIS consensus guideline consists of 10 recommendations in four categories: screening and diagnosis, treatment, complications, and monitoring, covering a total of 27 items. These recommendations received over 75% agreement from the panelists. Early diagnosis and a coordinated referral system between ophthalmologists and pediatric rheumatologists are crucial to prevent irreversible visual impairment in children with JIAU. However, achieving a balance between disease activity and medication use remains a key challenge in JIAU management, necessitating further clinical studies.

Literature- and Experience-Based Consensus for Acute Post-operative Endophthalmitis and Endogenous Endophthalmitis in Taiwan.

Clinical practices on acute post-operative and endogenous endophthalmitis (EnE) are highly variable among clinicians due to a lack of up-to-date, high-quality evidential support. An expert consensus is thus much needed. A panel consisting of ten retinal specialists in Taiwan was organized. They evaluated relevant literature and developed key questions regarding acute post-operative and EnE that are cardinal for practice but yet to have conclusive evidence. The panel then attempted to reach consensus on all the key questions accordingly. There were eight key questions proposed and their respective consensus statements were summarized as follows: Gram staining and culture are still the standard procedures for the diagnosis of endophthalmitis. Vitrectomy is recommended to be performed earlier than the timing proposed by the Endophthalmitis Vitrectomy Study (EVS). Routine intracameral antibiotic injection for post-cataract surgery endophthalmitis prophylaxis is not recommended because of potential compounding error hazards and a lack of support from high-quality studies. Routine fundus examination is recommended for all patients with pyogenic liver abscess. In EnE, vitrectomy is recommended if diffused and dense vitritis is present, or if the disease progresses. These consensus statements may work as handy guidance or reference for clinical practices of acute post-operative and EnE.

Clinical features of pachyvessels associated with polypoidal choroidal vasculopathy in chronic central serous chorioretinopathy.

To investigate the association between clinical features of chronic central serous chorioretinopathy (CSC) and subsequent development of polypoidal choroidal vasculopathy (PCV). Characteristics and treatment response of PCV secondary to CSC were described. This retrospective observational study included 18 patients with chronic CSC (18 eyes) with subsequent PCV and 36 controls (36 eyes) with chronic CSC without PCV development during follow-up. Clinical features were compared between the two groups. A logistic regression model was used to evaluate the risk factor of PCV formation. Treatments for PCV included anti-vascular endothelial growth factor (VEGF) monotherapy, photodynamic therapy (PDT), or PDT and anti-VEGF combination treatment. Subretinal fluid on optical coherence tomography images were assessed after treatments. Significant between-group differences were observed in best-corrected visual acuity after disease resolution and presence of pachyvessels (P = .001 and P = .003, respectively). The presence of pachyvessels in chronic CSC was associated with subsequent PCV (odds ratio = 6.00; 95% CI, 1.74-20.68; P = .005). CSC recurrence and subfoveal choroidal thickness (SFCT) were not significantly associated with subsequent PCV development (P = .393 and P = .911, respectively). The mean age of PCV diagnosis was 51 years, and the mean time from CSC diagnosis to PCV confirmation was 77.8 months. The mean (range) SFCT of PCV was 327.7 (134-599) µm. Nine patients received anti-VEGF monotherapy and 5 had disease remission. Four patients received PDT and anti-VEGF combination treatment and all of the 4 had disease remission. In chronic CSC, pachyvessel characteristics are associated with subsequent PCV development. This result will assist clinicians to evaluate CSC in clinical practice and provide insights into the pathogenesis of PCV.

Acute zonal occult outer retinopathy presenting as optic neuritis.

Acute zonal occult outer retinopathy (AZOOR) is an outer retinal disorder characterized by the acute loss of visual functions. Herein, we report a case of AZOOR presenting features mimicking optic neuritis. A 17-year-old healthy male reported fogginess in the right eye for 2 weeks. His best-corrected visual acuity was 20/20 in both eyes. Results of a color vision test and pupillary reaction were unremarkable. Funduscopic examination revealed a subtle hyperemic disc surrounded by hyperpigmentation in the right eye. Visual field examination confirmed an enlarged blind spot in the affected eye. Fundus autofluorescence imaging revealed zonal hyperautofluorescence around the optic disc. Fluorescein angiography showed optic disc staining and a window defect in the retinal pigment epithelium. Optical coherence tomography demonstrated loss of the ellipsoid line at the corresponding hyperautofluorescent region. All these characteristics indicated a diagnosis of AZOOR. However, the prolonged P100 wave observed through visual-evoked potential examination, hyperintensity T2 signal at the retrobulbar optic nerve through magnetic resonance imaging, and mild hyperemic optic disc along with optic disc staining through fluorescein angiography resemble the characteristics of optic neuritis. Because the clinical features of AZOOR are similar to those of optic neuritis, ophthalmologists should be able to differentiate between these two diseases to achieve a timely and correct diagnosis.

Ophthalmologic manifestations in Taiwanese patients with mucopolysaccharidoses.

BACKGROUND: Mucopolysaccharidoses (MPSs) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans in various tissues and organs. Ocular problems that affect the cornea, trabecular meshwork, sclera, retina, and optic nerve are very common in these patients. However, there was limited literature focusing on comprehensive ocular findings in different types of MPS. METHODS: We retrospectively reviewed the clinical ophthalmologic features and electrodiagnostic results of 50 Taiwanese patients with a diagnosis of MPS (34 males and 16 females; age range, 1.1-34.9 years; nine with MPS I, 17 with MPS II, 17 with MPS IV, and seven with MPS VI). RESULTS: Among 44 patients with available data for visual acuity, 15 patients (34%) had a visual acuity of less than 0.5 (6/12) equivalent in their better eye, including 71% of those with MPS VI, 38% with MPS IV, 29% with MPS I, and 14% with MPS II. Severe corneal opacities existed in 57% of MPS VI patients and 11% of MPS I patients, compared with none for MPS II and MPS IV patients. Among 80 eyes with available data of refraction, 11 eyes (14%) had myopia (≦-0.50 D), 55 eyes (69%) had hyperopia (≧0.50 D), and 55 eyes (69%) had high astigmatism (≧1.50 D). Ocular hypertension was found in 45% (28/62) of eyes. There were 16% (14/90), 11% (10/90), 13% (12/90), 31% (27/86), and 79% (30/38) of MPS eyes with lens opacities, optic disc swelling, optic disc cupped, retinopathy, and visual pathway dysfunction, respectively. Intraocular pressure was positively correlated with the severity of corneal opacity (p < 0.01). CONCLUSIONS: Ocular complications with significant reduction in visual acuity are common in MPS patients. Diagnostic problems may arise in these patients with severe corneal opacification, especially for those with MPS VI and MPS I.

One-year real-world outcomes of ranibizumab 0.5 mg treatment in Taiwanese patients with polypoidal choroidal vasculopathy: a subgroup analysis of the REAL study.

AIM: To assess the effectiveness and safety of ranibizumab 0.5 mg in Taiwanese patients with polypoidal choroidal vasculopathy (PCV) by performing a retrospective exploratory subgroup analysis of the REAL study. METHODS: REAL was a 12-month, observational, prospective, non-interventional phase IV post-marketing surveillance study conducted at 9 centers in Taiwan. The study collected data as part of the routine patient visits from the medical records of patients with neovascular age-related macular degeneration treated with ranibizumab 0.5 mg according to local standard medical practice and local label and/or reimbursement guidelines. The presence of PCV at baseline was determined using indocyanine green angiography. RESULTS: At baseline, PCV was diagnosed in 64 of the 303 enrolled patients (21.1%). Of these, 41 patients (64.1%) had received prior treatment; 15 (23.4%) patients had received ranibizumab. The intent-to-treat population included 58 patients; 47 (80%) who received ranibizumab and 11 (20%) who received ranibizumab plus photodynamic therapy (PDT; 9 patients received once, 2 patients received twice). Bevacizumab was used as a concomitant medication in a similar percentage of patients who received ranibizumab (43%, n=20) or ranibizumab plus PDT (45%, n=5). In patients who received ranibizumab, visual acuity (VA) at baseline was 50.1±12.9 Early Treatment Diabetic Retinopathy Study letters, and the gain at month 12 was 1.1±17.8 letters. In patients who received ranibizumab plus PDT, VA at baseline was 51.4±15.9 letters, and there was a marked gain in VA at month 12 (14.0±9.2 letters, P=0.0009). In the intent-to-treat population, the reduction in central retinal subfield thickness from baseline at month 12 was 69.6±122.6 µm (baseline: 310.8±109.8 µm, P=0.0004). The safety results were consistent with the well-characterized safety profile of ranibizumab. CONCLUSION: In real-world settings, ranibizumab 0.5 mg treatment for 12mo results in maintenance of VA and reduction in central retinal subfield thickness in Taiwanese patients with PCV. Improvements in VA are observed in patients who received ranibizumab plus PDT. There are no new safety findings.

Recommendation of using systemic anti-tumor necrosis factor-alpha for the treatment of noninfectious uveitis in Taiwan.

Noninfectious uveitis is a sight-threatening disease with an autoimmune or autoinflammatory basis. Systemic treatment is required if intraocular inflammation threatens a patient's vision or cannot be controlled locally and when it is associated with systemic rheumatic diseases. Corticosteroids and immunomodulatory chemotherapy are the conventional initial treatments. However, the various side effects of these therapies increase the burden on patients, not only physically but also mentally. Moreover, uncontrolled inflammation and poor visual outcomes have sometimes been recorded despite the combination of these medications or their high dosage. Antitumor necrosis factor-alpha (anti-TNF-α) and other biologic agents have been widely used to treat rheumatic diseases for >15 years. Randomized controlled clinical trials have demonstrated that anti-TNF-α can reduce and delay episodes of intraocular inflammation not only in patients with active uveitis but also in corticosteroid-dependent patients with inactive uveitis. The Taiwan Food and Drug Administration approved the use of adalimumab, an anti-TNF-α agent, for treating nonanterior noninfectious uveitis (NANIU) in 2017. This report provides a recommendation and a proposed stepladder approach for using anti-TNF-α agents to treat NANIU in Taiwan.

Interferon-α2a treatment for refractory Behçet's disease.

We report a young male patient with Behçet's disease who suffered from sight-threatening recurrences under treatment with azathioprine, cyclosporine, and prednisolone. His uveitis responded well to antitumor necrosis factor (TNF)-alpha (adalimumab) for 5 months subsequently. Severe uveitis recurred soon after discontinuation of anti-TNF alpha therapy and could not be controlled well with reinstitution of the anti-TNF alpha therapy. Interferon-α2a (IFN-α2a) was then given along with low-dose oral prednisone (10 mg/day), and the uveitis responded well to this therapy. We continued a maintenance dose with of IFN-α2a three times/week for 2 years. Sight-threatening uveitis did not recur under IFN-α2a therapy, and the visual acuity improved from "counting fingers" to 20/100 in the right eye, while remaining stable with 20/20 vision in the left eye. The patient had flu-like symptoms, fever, and severe depression during IFN therapy, but an attempt to discontinue INF led to relapse within 1 month. This case report suggests that IFN-α2a could be an option for treatment in Behçet's uveitis. Further study is needed to clarify the efficacy and appropriate strategy for IFN-α2a therapy for Behçet's uveitis in Taiwan.

Choroidal metastasis from esophageal squamous cell carcinoma.

We presented a rare case of a sole choroidal metastatic tumor from esophageal squamous cell carcinoma (ESCC) without other organ metastasis in Taiwan. A 43-year-old male with ESCC was referred for a 1-month history of decreased vision in his left eye. A 5.7 mm thick, yellow choroidal tumor occupied posterior pole, featured with pinpoint hyperfluorescence on angiography and subretinal fluid on optical coherence tomography. Positron emission tomography showed a singular hypermetabolic focus in the left eye. The tumor regressed with complete response and the vision preserved after radiation with total 57.60 gray applied by tomotherapy. The gastrointestinal system is the third most common metastatic origin in Taiwan while esophageal cancer metastasizing to choroid is rare. The discrepancy between the high prevalence of primary ESCC and the rareness of choroidal metastasis from ESCC is undetermined.

Real-World Experience with Half-Time Versus Half-Dose Photodynamic Therapy in Chronic Central Serous Chorioretinopathy.

PURPOSE: To evaluate the real-world experience with half-time photodynamic therapy (PDT) versus half-dose PDT for chronic central serous chorioretinopathy (CSC). METHODS: This multicenter retrospective study enrolled patients who received half-time PDT (with irradiation time shortened to 42 s) or half-dose PDT (with the dosage of verteporfin reduced to 3 mg/m2) for chronic CSC and who were followed up for ≧12 months. The success rate, central subfield retinal thickness (CST), and best-corrected visual acuity (BCVA) were documented in each group of patients. RESULTS: A total of 53 eyes from 49 patients were enrolled in this study. Seventeen eyes (15 patients) received half-time PDT and 36 eyes (34 patients) received half-dose PDT. The success rates in both groups were similar at 12 months (94.1% vs. 94.4%; P = 0.543). The mean CST at 1, 6, 12 months decreased significantly when compared with the baseline in both groups (all P < 0.001). The BCVA significantly improved at 6 and 12 months in both groups (all P < 0.05). There were no significant differences in changes of BCVA and changes of CST between the 2 groups at any time point. CONCLUSIONS: Half-time PDT is a feasible treatment for chronic CSC. It has success rates similar to half-dose PDT at 12 months. There were no significant differences in changes of BCVA and changes of CST between the 2 groups at 1, 6, and 12 months after treatment.

Half-dose verteporfin combined with half-fluence photodynamic therapy for chronic central serous chorioretinopathy.

PURPOSE: This study evaluated the safety and efficacy of half-dose verteporfin combined with half-fluence photodynamic therapy (half-half photodynamic therapy (PDT) for chronic central serous chorioretinopathy (CSC). METHODS: This was a retrospective case series. Fourteen eyes with chronic CSC receiving half-half PDT were included in group 1. Another 28 eyes receiving half-dose verteporfin combined with standard fluence PDT were included in group 2 as a control group. Main outcome measures were the success rates, major complications, best-corrected visual acuity (BCVA), and central subfield foveal thickness (CFT) on optical coherence tomography (OCT) at 6 months in both groups. Success of treatment was defined as complete resolution of subretinal fluid on OCT after treatment without recurrence. RESULTS: There was no significant difference between groups in their age, gender, duration of symptoms, baseline BCVA, baseline CFT, PDT spot size, and follow-up duration. The success rate was 64% (9/14 eyes) in group 1 and 93% (26/28 eyes) in group 2 at 6 months (P=0.031). No major complications were found in either group. Mean CFT showed significant reduction at 6 months in both groups (-115 µm and P<0.001 in group 1; -150 µm and P<0.001 in group 2). The mean BCVA in group 2 improved significantly (P<0.001) at 6 months. The mean BCVA in group 1 showed a trend of improvement but was not statistically significant (P=0.25) at 6 months. CONCLUSIONS: Half-half PDT is a feasible treatment for chronic CSC. However, there was a lower success rate at 6 months compared with the control group.

Eclipse photodynamic therapy for a presumed peripapillary metastatic tumor.

PURPOSE: We report a successful use of a modified photodynamic therapy (PDT) termed Eclipse PDT in treating a patient with peripapillary metastatic choroidal tumor. METHODS: Optic disk protection effect was measured with different colored paper disk attached to the reflecting mirror of the laser machine. RESULTS: Black paper disk was chosen to perform Eclipse PDT because of its maximal blocking effect. A patient with peripapillary metastatic choroidal tumor was treated using this method, and the postoperative outcome was favorable, with improvement in visual acuity and resolution of subretinal fluid. CONCLUSION: With the new technique, treatment can be modified according to the lesion's shape and location without damaging the optic disk. Eclipse PDT can further extend the indication of PDT treatment to peripapillary choroidal neovascularization as well as choroidal tumors close to the optic disk.