Juvenile idiopathic arthritis-associated uveitis in the era of biological therapy: how the disease changed in more than 20 years of observation in a tertiary referral center in Rome (Italy)

Objectives To describe the ophthalmological characteristics in a Juvenile idiopathic arthritis (JIA) cohort and to evaluate how therapeutic advances have changed the course of the uveitis. Methods Analysis of a retrospective cohort study of consecutive JIA pediatric patients including JIA-associated uveitis (JIA-U) and comparison with a previous study in the same uveitis center assessed before the wide-spread of biological therapy. Results The total of 49 JIA patients were analyzed, of whom 18 JIA-U, compared with a JIA-U past cohort of 66 patients. Systemic corticosteroids were used significantly less in the current JIA-U group (p = 0.008) than in the past one. JIA-U present cohort was on therapy more frequently with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) than the past group (p = 0.039), mostly treated with methotrexate (93.3%). Furthermore, a larger use of biologic disease-modifying anti-rheumatic drugs (bDMARDs) was described in the current JIA-U group (p = 0.005) also associated with csDMARDs (p = 0.003). Adalimumab was used more (72.7%) in the present JIA-U cohort compared to a larger treatment with infliximab (61.5%) in the past (p = 0.005). Higher number of uveitis recurrences was observed in the previous cohort compared to the current one (p = 0.005). Fewer complications were described in this study than in the previous: posterior synechiae (p = 0.007), cataract (p < 0.001), band keratopathy (p < 0.001), and elevated intraocular pressure (IOP) (p = 0.047). Conclusion Current therapies reduced the uveitis recurrences and ocular complications including cataract due also to the lower use of corticosteroids. The new close collaboration with the pediatric rheumatologic center in the same University has contributed to the care improvement and decrease of uveitis complications.

Case Report: Phototherapeutic Keratectomy for Band Keratopathy Secondary to Chemo-Laser-Cryotherapy for Retinoblastoma

Purpose: To report the clinical outcomes of phototherapeutic keratectomy (PTK) for pediatric band keratopathy after treatment for retinoblastoma.Methods: A 5-year-old boy presented with a 2-year history of poor visual acuity and a horizontal gray-white band across the central cornea in the right eye. He was diagnosed with band keratopathy after chemo-laser-cryotherapy for retinoblastoma. The band keratopathy was treated via PTK using the Mel-90 excimer laser with an optical treatment zone of 7.0 mm and ablation depth of 120 μm. The patient was followed at 1 week and 3 months postoperatively.Results: Surgery and postoperative follow-up were uneventful. At the 3-month follow-up, the uncorrected distant visual acuity of the right eye improved to 20/125, and the corrected distance visual acuity improved to 20/70 with a refraction of +10.00 D/−2.50 DC × 15. The clarity of the ablated area was evidently improved. The central corneal thickness decreases from 612 to 584 μm. The optical coherence tomography showed the thin band of hyperreflectivity in the ablated area disappeared, corneal transparency improved and the corneal surface smoothened.Conclusions: PTK is a safe and effective procedure to treat band keratopathy following treatment of retinoblastoma in children. Early intervention can reduce the risk of developing deprivation amblyopia.

Risk Factors for Band Keratopathy in Aphakic Eyes With Silicone Oil Tamponade for Open-Globe Injuries: A Multicenter Case-Control Study

Band keratopathy (BK) is a common complication in aphakic eyes with silicone oil tamponade for open-globe injury (OGI), characterized by the grayish-white opacities in the cornea, resulting in a significantly decreased vision when extending to the visual axis. To identify the risk factors for BK in aphakic eyes following vitreoretinal surgical treatment with silicone oil tamponade for OGIs, we performed a multicenter case-control study. The incidence of BK was 28% (28/100 eyes). The multivariate binary logistic regression revealed the silicone oil retention time (SORT) ≥6 months and zone III injury were significant risk factors for BK. From the hierarchical interaction, SORT ≥6 months had a significant risk for BK in eyes with rupture, aniridia, and zone III injury, while zone III injury had a significant risk for BK in eyes with rupture, incomplete/complete iris, and SORT ≥6 months. By using restricted cubic splines with three knots at the 25th, 50th, and 75th centiles to model the association of SORT with BK, we also found a marked increase in the risk for BK at ≥10 months and a slow increase after 6 months, but almost stable within 4–6 months.

Long Term Experience in Patients With JIA-Associated Uveitis in a Large Referral Center

Objectives: To describe demographic, clinical and therapeutic findings of a large cohort of patients with JIA-associated uveitis in a nationwide referral pediatric rheumatology and uveitis center in Northern Italy.Methods: Retrospective study of 125 patients with JIA-associated uveitis followed from 2009 to 2019. Demographic and rheumatologic features including JIA ILAR classification, age at onset, and laboratory data were recorded. Ocular findings collected were: anatomic location of uveitis, laterality, type, recurrence rate, visual acuity, ocular complications, and local therapy. Systemic therapy with conventional and biologic immunosuppressants, occurrence of adverse events, and duration of treatments were recorded.Results: One hundred and twenty-five patients with JIA-associated uveitis were followed for a meantime of 9.2 (±1.7) years. Oligoarticular JIA was present in 92.8% of patients and anterior uveitis in 96%. The most common ocular complications recorded in our sample were posterior synechiae (37.6%), cataract (20.8%), band keratopathy (19.2%), glaucoma (7.2%), and macular edema (5.6%). Conventional immunosuppressants were used in 75.2% of patients with a mean duration of 9.1 years (±5.4), while biologics were administered in 47.2% of them for a period of 5.4 years. Adverse events (AE) were seen in 23% of patients being treated with Methotrexate, in 10.4% of patients treated with Adalimumab, in 38.5% of patients in therapy with Infliximab, and in 14.3% of patients being treated with Tocilizumab. No AE were reported in patients treated with Golimumab, Certolizumab, Abatacept and Rituximab.Conclusions: An aggressive treatment approach for patients with JIA-associated uveitis ensured a low number of ocular complications with a good safety profile.

A Recurrent Central Band Keratopathy in a Child

For the first time, to our knowledge, we present the case of a child with simultaneous occurrence of a C3-glomerulopathy and an anterior uveitis complicated with severe band keratopathy (BK). In addition, repeated evaluations of uveitis disclosed important differences between slit lamp and laser flare photometry (LFP) results due to BK, making treatment choice difficult.

Clinical outcomes of simultaneous phototherapeutic keratectomy and photoastigmatic keratectomy

This study was aimed to assess the outcomes of simultaneous phototherapeutic keratectomy (PTK) and photoastigmatic keratectomy (PAK), with special attention to astigmatic correction. We comprised 70 eyes of 70 patients who underwent simultaneous PTK and PAK in patients having granular corneal dystrophy and band keratopathy with refractive astigmatism of 1 diopter (D) or more. Preoperatively and 6 months postoperatively, we assessed corrected uncorrected distance visual acuity (UDVA), distance visual acuity (CDVA), manifest spherical equivalent, refractive astigmatism, corneal astigmatism, and higher-order aberrations (HOAs). LogMAR CDVA significantly improved, from 0.27 ± 0.27 preoperatively, to 0.13 ± 0.21 postoperatively (Paired t test, p < 0.001). LogMAR UDVA also significantly improved, from 0.70 ± 0.32 preoperatively, to 0.57 ± 0.41 postoperatively (p = 0.043). Refractive astigmatism significantly decreased, from 2.12 ± 0.95 D preoperatively, to 0.89 ± 0.81 D postoperatively (p < 0.001). Corneal astigmatism also significantly decreased, from 2.17 ± 0.90 D preoperatively, to 1.08 ± 0.71 D postoperatively (p < 0.001). Corneal HOAs did not significantly change, from 0.54 ± 0.30 µm preoperatively, to 0.48 ± 0.20 µm postoperatively (p = 0.140). No significant complications occurred in any eye. Simultaneous PTK and PAK treatment is effective not only for improving visual acuity, but also for reducing astigmatism.

Juvenile idiopathic arthritis-associated uveitis in the era of biological therapy: how the disease changed in 14 years of observation in a tertiary referral center in Rome (Italy)

Objectives To describe the ophthalmological characteristics in a Juvenile idiopathic arthritis (JIA) cohort and to evaluate how therapeutic advances have changed the course of the uveitis. Methods Analysis of a retrospective cohort study of consecutive JIA pediatric patients including JIA-associated uveitis (JIA-U) and comparison with a previous study in the same uveitis center assessed before the wide-spread of biological therapy. Results 49 JIA patients analyzed, of whom 18 JIA-U, compared with a JIA-U past cohort of 66 patients. Systemic corticosteroids were used significantly less in the current JIA-U group (p = 0.008) than in the past one. JIA-U present cohort were on therapy more frequently with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs), than the past group (p = 0.039), mostly treated with methotrexate (93.3%). Furthermore, a larger use of biologic disease-modifying antirheumatic drugs (bDMARDs) was described in the current JIA-U group (p = 0.005) also associated with csDMARDs (p = 0.003). Adalimumab was used more (72.7%) in the present JIA-U cohort compared to a larger treatment with infliximab (61.5%) in the past (p = 0.005). Higher number of uveitis recurrences was observed in the previous cohort compared to the current one (p = 0.005). Fewer complications were described in this study than in the previous: posterior synechiae (p = 0.007), cataract (p < 0.001), band keratopathy (p < 0.001), elevated intraocular pressure (IOP) (p = 0.047) Conclusion. Current therapies reduced the uveitis recurrences and ocular complications including cataract due also to the lower use of corticosteroids. The new close collaboration with the pediatric rheumatologic center in the same University has contributed to the care improvement and decrease of uveitis complications.

Scleral Tunnel Fixated Intraocular Lens in Complicated Cataract: A Case Series

When capsular support is inadequate in complicated cataract, scleral fixated IOL (SFIOL) has its advantage whereby it is nearer to physiological nodal point when compared to other types of IOL implantation. Scleral tunnel fixated intraocular lens (STFIOL) technique is gaining popularity due to its simplicity and ease of placement compared to sutured IOL. We report our experience with eight cases of STFIOL implantation from September 2016 to May 2018 in Universiti Kebangsaan Malaysia Medical Centre (UKMMC). All patients had improvement of vision except one case who had unchanged vision. Mean post-operative BCVA was logMAR 0.57±1.13 (which is almost equivalent to 6/21) and improved to logMAR 0.37±0.43 (which is almost equivalent to 6/12) excluding one patient with chronic RRD with band keratopathy. Post-op complications include raised intraocular pressure, cystoid macular oedema, hyphaema, and wound leak. However, all complications were not severe and responded to topical eyedrops. In conclusion, STFIOL insertion technique is safe and works well to restore vision in majority of patients operated with this technique.