Pathogenic STX3 variants affecting the retinal and intestinal transcripts cause an early-onset severe retinal dystrophy in microvillus inclusion disease subjects.

Biallelic STX3 variants were previously reported in five individuals with the severe congenital enteropathy, microvillus inclusion disease (MVID). Here, we provide a significant extension of the phenotypic spectrum caused by STX3 variants. We report ten individuals of diverse geographic origin with biallelic STX3 loss-of-function variants, identified through exome sequencing, single-nucleotide polymorphism array-based homozygosity mapping, and international collaboration. The evaluated individuals all presented with MVID. Eight individuals also displayed early-onset severe retinal dystrophy, i.e., syndromic-intestinal and retinal-disease. These individuals harbored STX3 variants that affected both the retinal and intestinal STX3 transcripts, whereas STX3 variants affected only the intestinal transcript in individuals with solitary MVID. That STX3 is essential for retinal photoreceptor survival was confirmed by the creation of a rod photoreceptor-specific STX3 knockout mouse model which revealed a time-dependent reduction in the number of rod photoreceptors, thinning of the outer nuclear layer, and the eventual loss of both rod and cone photoreceptors. Together, our results provide a link between STX3 loss-of-function variants and a human retinal dystrophy. Depending on the genomic site of a human loss-of-function STX3 variant, it can cause MVID, the novel intestinal-retinal syndrome reported here or, hypothetically, an isolated retinal dystrophy.

A Clinical Trial Comparing the Safety and Efficacy of Topical Tacrolimus versus Methylprednisolone in Ocular Graft-versus-Host Disease.

PURPOSE: To evaluate the safety and efficacy of topical tacrolimus 0.05% versus topical methylprednisolone 0.5% in patients with ocular graft-versus-host disease (GVHD). DESIGN: Phase 1/2 prospective, randomized, double-masked clinical trial. PARTICIPANTS: Eighty eyes of 40 patients diagnosed with chronic ocular GVHD were enrolled. METHODS: Forty patients with ocular GVHD were randomized; 24 patients were treated with topical tacrolimus 0.05% and 16 patients were treated with topical methylprednisolone 0.5% twice daily for 10 weeks, in addition to continuing their baseline treatment regimen. MAIN OUTCOME MEASURES: Safety was evaluated based on occurrence of adverse events. Tolerability was assessed based on subject reports of discomfort after drop instillation. Intraocular pressure (IOP) was monitored. The main efficacy end points were corneal fluorescein staining (CFS), tear film break-up time (TBUT), Schirmer test results, and expression of the ocular surface inflammatory markers human leukocyte antigen-DR (HLA-DR) and intercellular adhesion molecule-1 (ICAM-1). Symptoms were evaluated using the Ocular Surface Disease Index (OSDI). RESULTS: After 10 weeks of treatment, no major adverse events occurred in either treatment group, and there was no significant difference in the composite tolerability scores between the 2 groups (P = 0.06). However, burning sensation was more pronounced with tacrolimus (P = 0.002). Topical tacrolimus was more effective than methylprednisolone in reducing the CFS score at week 10 (55% vs. 23% reduction, respectively; P = 0.01) and achieved significant improvement in TBUT when compared with baseline (P < 0.001). Reduction in OSDI score achieved statistical significance with tacrolimus (27% reduction; P = 0.02), but was marginal with methylprednisolone (32% reduction; P = 0.06). Expression of ICAM-1 by ocular surface epithelium decreased significantly in both groups (tacrolimus, P = 0.003; methylprednisolone, P = 0.008), whereas HLA-DR expression decreased significantly only in the tacrolimus group (P = 0.03). Schirmer test scores did not change significantly in either group during the study; IOP increased significantly with methylprednisolone at week 10 (P = 0.04). CONCLUSIONS: Topical tacrolimus 0.05% is safe, generally well tolerated, and effective for the treatment of ocular GVHD without the hypertensive effects of topical corticosteroids.

Prevalence of ocular hypertension and glaucoma in patients with chronic ocular graft-versus-host disease.

PURPOSE: To evaluate the prevalence of ocular hypertension (OHT) and glaucoma in patients with chronic ocular graft-versus-host disease (GVHD). METHODS: We performed a retrospective chart review of 218 patients diagnosed with chronic ocular GVHD. Ocular hypertension was defined as intraocular pressure (IOP) ≥ 24 mmHg in either eye without any glaucomatous optic disc changes. Glaucoma suspect was defined as optic disc changes with a cup-to-disc ratio ≥ 0.7 in either eye or asymmetry of ≥ 0.3 between the two eyes. Glaucoma was defined by glaucomatous optic disc changes plus glaucomatous visual field defects in two consecutive reliable visual field tests. The number of cases of ocular hypertension, glaucoma, and glaucoma suspects was evaluated. RESULTS: Thirty-three patients (15 %) were diagnosed with OHT, eight patients (3.6 %) with suspicion of glaucoma, and one patient (0.4 %) with glaucoma. OHT occurred within 6 months of developing ocular GVHD in 60 % of the cases and within the first year in 76 %. High IOP normalized in 67 % of patients when the dosage of topical or systemic corticosteroids was lowered, and 27 % of patients required anti-glaucoma therapy. CONCLUSIONS: Ocular hypertension is a common complication in patients with ocular GVHD, with a prevalence of 15 %. The rise in intraocular pressure is often transient and resolves with management of corticosteroids in most cases. However, clinicians should be aware that nearly one-third of the patients with OHT might require anti-glaucoma treatment. The prevalences of glaucoma and suspicion of glaucoma were not higher than in the general population.

Vision-Related Quality of Life in Patients with Ocular Graft-versus-Host Disease.

PURPOSE: To assess the vision-related quality of life (QOL) in a cohort of patients with ocular graft-versus-host disease (GVHD). DESIGN: Prospective study. PARTICIPANTS: Eighty-four patients diagnosed with chronic ocular GVHD. METHODS: We assessed the vision-related QOL with the 25-item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25). The symptoms of ocular GVHD were assessed using the Ocular Surface Disease Index (OSDI) and Symptom Assessment in Dry Eye (SANDE) questionnaires. MAIN OUTCOME MEASURES: We assessed vision-related QOL with the NEI-VFQ-25 and compared the scores obtained from patients with ocular GVHD with those from a healthy population. In the ocular GVHD population, we also evaluated the associations between the NEI-VFQ-25 and the dry eye symptoms measured by the OSDI and SANDE questionnaires, age, duration of disease, best-corrected visual acuity (BCVA), corneal fluorescein staining (CFS), tear break-up time, and Schirmer test. RESULTS: The mean composite NEI-VFQ-25 score in patients with ocular GVHD was 76.5±17. Compared with healthy subjects, patients with ocular GVHD reported reduced scores on all NEI-VFQ-25 subscales (each P < 0.001) with the exception of color vision (P = 0.11). The NEI-VFQ-25 composite scores significantly correlated with OSDI (R = -0.81, P < 0.001), SANDE (R = -0.56, P < 0.001), CFS (R = -0.36, P = 0.001), and BCVA (R = -0.30, P = 0.004). CONCLUSIONS: Patients with ocular GVHD experience measurable impairment of vision-related QOL. This study highlights the impact of ocular GVHD on the vision-related QOL, and thus the importance of comprehensive diagnosis and treatment of this condition.

Outcomes of phacoemulsification in patients with chronic ocular graft-versus-host disease.

PURPOSE: The purpose of this study was to evaluate the outcomes of phacoemulsification in patients with ocular graft-versus-host disease (GVHD). METHODS: The occurrence of cataracts, cataract surgery, and its outcomes were analyzed in the medical records of 229 patients (458 eyes) with ocular GVHD. Outcome measures included pre- and postoperative corrected distance visual acuity (CDVA) and the rate of postoperative complications. RESULTS: Of the 458 eyes evaluated, 58 were pseudophakic; from the 400 phakic eyes, 238 (59 %) presented with cataracts and 62 (26 %) underwent cataract surgery. Analysis of postoperative complications and visual outcomes at 1 month was performed in 51 eyes in which detailed surgical and immediate postoperative records were available. Preoperatively, the mean CDVA was 0.67 ± 0.57 LogMAR (Snellen 20/93), improving postoperatively to 0.17 ± 0.18 (Snellen 20/29) at 1 month (P < 0.0001), and to 0.13 ± 0.14 (Snellen 20/26) by the final follow-up visit (P < 0.0001). Postoperative complications included corneal epithelial defects (8 %), filamentary keratitis (6 %), worsening of corneal epitheliopathy (16 %), posterior capsular opacification (18 %), and cystoid macular edema (4 %). A corrected distance visual acuity of 20/30 or better was achieved in 87 % of the eyes; suboptimal CDVA improvement was attributable to severe ocular surface disease, pre-existing advanced glaucoma, and prior macular surgery. CONCLUSIONS: Phacoemulsification in patients with chronic ocular GVHD is a safe and efficacious procedure resulting in significant visual improvement. Overall, postoperative adverse events responded well to timely management.

The Detection of Occult Retinal Vasculitis on Fluorescein Angiography in Pediatric Uveitis.

PURPOSE: To evaluate the usefulness of fluorescein angiography (FA) to detect occult retinal vasculitis in children with otherwise apparently quiescent intermediate uveitis, posterior uveitis, and panuveitis based on clinical examination alone. DESIGN: Retrospective chart review. PARTICIPANTS: Pediatric uveitis patients evaluated at the Children's Medical Center in Dallas, Texas. METHODS: Retrospective chart review of pediatric patients treated with immunosuppressive therapy in the uveitis clinic at the Children's Medical Center, Dallas, Texas, between September 2015 and September 2016. Patients with noninfectious uveitis requiring immunosuppressive therapy, in which posterior segment involvement (intermediate uveitis, posterior uveitis, or panuveitis) was known or suspected, and whose disease apparently was quiescent on clinical examination were included. MAIN OUTCOME MEASURES: The incidence of occult retinal vasculitis detected on FA alone. RESULTS: Fourteen pediatric patients met inclusion criteria. Six patients (43%) demonstrated intermediate uveitis, and 8 patients (57%) demonstrated panuveitis. Eleven patients (79%) were found to show additional evidence of occult retinal vasculitis on FA. CONCLUSIONS: Fluorescein angiography can be an important tool in evaluating pediatric uveitis patients with known or suspected posterior involvement for the presence of occult retinal vasculitis. Failure to control occult retinal vasculitis adequately may be a contributing factor to seemingly recalcitrant cases, inability to wean off immunomodulatory therapy, and long-term complications leading to poor prognosis.

The effect of patient compliance on remission rates in pediatric noninfectious uveitis.

PURPOSE: To identify barriers to compliance in pediatric noninfectious uveitis, and to examine its association with achieving steroid-free remission. METHODS: A retrospective analysis was performed on pediatric patients with noninfectious uveitis on immunomodulatory therapy treated at the University of Texas Southwestern Medical School and Children's Medical Center (Dallas, TX) between September 2015 and March 2017. Compliance barriers were identified and rates of achieving steroid-free remission were calculated. RESULTS: A total of 57 patients with noninfectious uveitis requiring immunosuppressive therapy met inclusion criteria. Thirty-three (58%) of patients were compliant. Notable barriers to compliance included regimens requiring >3 medications, patient/parent negligence, transportation issues, family strife, and presence of an associated systemic autoimmune disease (P < 0.050). At a median follow-up of 24 months, a total of 28 (49%) achieved steroid-free remission. The presence of 3 or more compliance barriers was associated with decreased remission rates (P < 0.050). Poor compliance was associated with decreased rates of steroid-free remission (21% vs 79% [P = 0.002]). CONCLUSIONS: Noncompliant patients with noninfectious pediatric uveitis requiring immunomodulatory therapy were found to have a lower rate of achieving steroid-free remission compared to patients who exhibited full compliance.

Successful treatment with infliximab after adalimumab failure in pediatric noninfectious uveitis.

PURPOSE: To describe the use of infliximab after adalimumab failure in the treatment of pediatric noninfectious uveitis. METHODS: A retrospective analysis was performed on the medical records of pediatric patients with noninfectious uveitis treated with infliximab for a minimum of 6 months after previously failing to achieve steroid-free remission using adalimumab at the University of Texas Medical School and Children's Medical Center between September 2015 and March 2018. Rates of achieving disease activity quiescence and steroid-free remission as well as incidence of adverse events were calculated. RESULTS: A total of 13 patients with noninfectious uveitis refractory to treatment with adalimumab met inclusion criteria. Three (23%) had anterior uveitis, 4 (31%) had pars planitis, and 6 (46%) had panuveitis. Eleven (85%) patients had preexisting ocular comorbidities. Of these, 4 (31%) had retinal vasculitis, and 1 (7.7%) had cystoid macular edema. There was a 100% response rate to treatment with infliximab following failure to achieve disease quiescence on adalimumab. At mean follow-up time of 21 months (range, 8-31) from initiation of infliximab, there was a reduction in steroid dependence from 100% to 15% after transitioning from adalimumab to infliximab (P < 0.001). Nine patients (69%) had achieved steroid-free remission on infliximab therapy. The mean time to steroid-free remission was 8.7 months. CONCLUSIONS: In our study cohort, infliximab was used successfully in all cases of recalcitrant pediatric noninfectious uveitis that previously failed adalimumab therapy.

Overestimation of Corneal Endothelial Cell Density in Smaller Frame Sizes in In Vivo Confocal Microscopy.

PURPOSE: To evaluate the effect of frame size on the calculated corneal endothelial cell density (CECD) in images of laser scanning in vivo confocal microscopy (IVCM). METHODS: Forty-nine corneal endothelial images acquired by laser scanning IVCM (Heidelberg Retina Tomograph 3 with Rostock Corneal Module) with different endothelial cell densities were analyzed. In each image (160,000 µm), the CECD was calculated using the fixed-frame method by counting cells in the following frame sizes: 80,000 µm, 40,000 µm, 20,000 µm, 10,000 µm, 5000 µm, and 2500 µm. The calculated CECD was then compared with that of the variable-frame method as the reference value. RESULTS: There was no significant difference in the calculated CECD between the variable-frame method (2004 ± 832 cells/mm), and the fixed-frame method using a 40,000-µm frame (2023 ± 810 cells/mm). On the other hand, the calculated CECD showed significant overestimations in frame sizes of 20,000 µm (2066 ± 820 cells/mm), 10,000 µm (2156 ± 785 cells/mm), 5000 µm (2352 ± 783 cells/mm), and 2500 µm (2715 ± 754 cells/mm), with P < 0.001 in all. This resulted in overestimations of 4.8 ± 9.8%, 11.9 ± 16.2%, 24.9 ± 23.1%, and 49.1 ± 38.8% for these frame sizes, respectively. Images with lower CECD demonstrated higher overestimations of cell density in smaller frame sizes. CONCLUSIONS: In laser scanning IVCM images, there is significant overestimation of CECD if the cells are counted in frames smaller than 25% of the image. Similar frame sizes should be used when monitoring CECD over time.

Multidetector Computed Tomography Features in Differentiating Exophytic Renal Angiomyolipoma from Retroperitoneal Liposarcoma: A Strobe-Compliant Observational Study.

This study aims to evaluate the multidetector computed tomography (CT) imaging features in differentiating exophytic renal angiomyolipoma (AML) from retroperitoneal liposarcoma. We retrospectively enrolled 42 patients with confirmed exophytic renal AML (31 patients) or retroperitoneal liposarcoma (11 patients) during 8 years period to assess: renal parenchymal defect at site of tumor contact, supply from branches of renal artery, tumoral vessel extending through the renal parenchyma, dilated intratumoral vessels, hemorrhage, non-fat-containing intratumoral nodules with postcontrast enhancement, calcification, renal sinus enlargement, anterior displacement of kidneys, and other associated AML. Renal parenchymal defect, renal arterial blood supply, tumoral vessel through the renal parenchyma, dilated intratumoral vessels, intratumoral/perirenal hemorrhage, renal sinus enlargement, and associated AML were seen only or mainly in exophytic renal AML (all P value < 0.05); however, non-fat-attenuating enhancing intratumoral nodules, intratumoral calcification, and anterior displacement of the kidney were more common in liposarcoma (all P value < 0.05). AMLs reveal renal parenchymal defect at the site of tumor contact, supply from renal artery, tumoral vessel extending through the renal parenchyma, dilated intratumoral vessels, intratumoral and/or perirenal hemorrhage, renal sinus enlargement, and associated AML. Non-fat-attenuating enhancing intratumoral nodules, intratumoral calcifications, and anterior displacement of kidney were more commonly seen in liposarcoma.

Reduced Corneal Endothelial Cell Density in Patients With Dry Eye Disease.

PURPOSE: To evaluate corneal endothelial cell density (ECD) in patients with dry eye disease (DED) compared to an age-matched control group. DESIGN: Cross-sectional, controlled study. METHODS: This study included 90 eyes of 45 patients with moderate to severe DED (aged 53.7 ± 9.8 years) and 30 eyes of 15 normal controls (aged 50.7 ± 9.8 years). All subjects had a complete ophthalmic evaluation including symptom assessment using the Ocular Surface Disease Index (OSDI) and corneal fluorescein staining. In addition, laser scanning in vivo confocal microscopy was performed to measure the density of the following parameters in the central cornea: endothelial cells, subbasal nerves, and subbasal immune dendritic cells. RESULTS: Corneal ECD was significantly lower in the DED group (2595.8 ± 356.1 cells/mm(2)) than in the control group (2812.7 ± 395.2 cells/mm(2), P = .046). The DED group showed significantly lower corneal subbasal nerve density (17.1 ± 6.9 mm/mm(2)) compared to the control group (24.7 ± 4.4 mm/mm(2), P < .001). Dendritic cell density was significantly higher in the DED group than in the controls (111.7 ± 137.3 vs 32.0 ± 24.4 cells/mm(2), respectively, P = .002). There were statistically significant correlations between corneal ECD and dry eye severity parameters including the OSDI score (rs = -0.26, P = .03), and corneal fluorescein staining (rs = -0.28, P = .008). CONCLUSIONS: There is a significant reduction in corneal ECD in DED that correlates with clinical severity of the disease.

Use of intravitreal rituximab for treatment of vitreoretinal lymphoma.

AIM: Vitreoretinal lymphoma is a diffuse large B cell non-Hodgkin lymphoma. Targeting malignant cells with rituximab is being used increasingly as local chemotherapy, but information on this treatment is scant. We aimed to describe current therapeutic approaches, as well as responses to and complications of, intravitreal rituximab in patients with vitreoretinal lymphoma. METHODS: Clinical data were collected in a standardised manner retrospectively on patients with vitreoretinal lymphoma treated with intravitreal rituximab. RESULTS: 48 eyes (34 patients) with vitreoretinal lymphoma were treated with a median of 3.5 intravitreal injections of rituximab (1 mg/0.1 mL) for new diagnosis (68.8%), progressive disease (29.9%) and maintenance therapy (2.1%). Intravitreal rituximab±methotrexate was the sole treatment in 19 eyes (39.6%). 31 eyes (64.6%) eyes achieved complete remission, after a median of 3 injections; 7 of these eyes developed recurrent disease. 11 eyes (22.9%) achieved partial remission. Although rituximab may have contributed to complications reported in 12 eyes (25.0%), a 2-line loss of Snellen visual acuity occurred in only 2 of those eyes (4.2%). CONCLUSIONS: Approaches in rituximab-based intravitreal chemotherapy vary widely, but our findings suggest that this treatment may be safe and effective in inducing remission in a majority of eyes with vitreoretinal lymphoma.

Demographic and clinical profile of vernal keratoconjunctivitis at a tertiary eye care center in India.

PURPOSE: To study the demographic and clinical profile of patients with vernal keratoconjunctivitis (VKC) at a tertiary eye care center in India. MATERIALS AND METHODS: Retrospective chart analysis of 468 patients of VKC seen from January 2006 to December 2006. RESULTS: Mean age at presentation was 12 years. Majority of the patients had mixed pattern disease (72%). Chronic perennial disease was seen in 36% patients. Personal or family history of allergies was noted in 5% patients. Severe disease based on clinical grading was present in 37% patients. Moderate to severe vision loss was seen in 12% of total population. Persistent disease beyond 20 years of age was found in 12% patients. VKC-related complications such as corneal scarring (11%), shield ulcer (3%), keratoconus (6%), and limbal stem cell deficiency (1.2%) were seen. Treatment-related complications like corticosteroid-induced cataract and glaucoma were seen in 6% and 4% of patients, respectively. CONCLUSION: Clinical pattern of VKC seen in the tropical climate of India is essentially similar to that seen in other tropical countries. Few distinct features that we noted represent chronic perennial disease, low association with atopy, and higher propensity for disease and treatment-related complications.

Clinical and Cytologic Evidence of Limbal Stem Cell Deficiency in Eyes With Long-Standing Vernal Keratoconjunctivitis.

PURPOSE: We aimed to study the impression cytology (IC) of the ocular surface in eyes with vernal keratoconjunctivitis (VKC) and clinical evidence of limbal stem cell deficiency (LSCD). DESIGN: This is a prospective comparative study. METHODS: This study included 78 eyes of 40 patients with VKC. Limbal stem cell deficiency was diagnosed clinically based on the presence of corneal findings such as dull irregular epithelial reflex, superficial neovascularization, conjunctivalization, and loss of limbal palisades of Vogt. The study group consisted of 28 eyes of 15 patients with clinically diagnosed LSCD and control group of 50 eyes of 25 patients without LSCD. Conjunctival and corneal IC was done in all eyes. Presence of goblet cells in the corneal samples on IC was considered confirmatory of LSCD. RESULTS: Compared with controls, patients with LSCD were older and had longer duration of disease. On IC, goblet cells were present on the cornea in 53.6% of eyes with clinically diagnosed LSCD and in none of the control eyes (P < 0.0001). Clinically diagnosed LSCD in study eyes correlated with cytologic findings of greater conjunctival squamous metaplasia, decreased conjunctival goblet cells, greater corneal cell metaplasia, and increased inflammation as compared with control eyes. CONCLUSIONS: Most of the eyes with VKC and clinical evidence of LSCD have cytologic evidence of LSCD with goblet cells on the cornea.

Bilateral corneal ulceration in ocular graft-versus-host disease.

PURPOSE: To report on corneal ulceration in ocular graft-versus-host disease (GVHD). METHODS: This was a retrospective, observational case series investigating corneal ulceration and perforation in a cohort of ocular GVHD patients seen between June 2007 and October 2012. RESULTS: Four of 243 ocular GVHD patients developed corneal ulcerations attributable to ocular GVHD, and all four cases involved bilateral corneal ulceration. The median length of time from the diagnosis of ocular GVHD to the diagnosis of the first corneal ulceration was 317 days (range 168-434). The median length of time between the diagnosis of corneal ulceration in each patient's first and second eye was 248 days (range 9-645). Outcomes varied from complete resolution with medical treatment to corneal perforation necessitating penetrating keratoplasty. In cases of corneal perforation, the median length of time from the diagnosis of corneal ulceration to perforation was 10 days (range 0-20). Common clinical features included: centrally or paracentrally located ulcerations and perforations, concomitant dry eye, and the use of topical or systemic corticosteroids. CONCLUSION: Frequent follow-up and bilateral monitoring are highly recommended in cases of ocular GVHD-associated stromal thinning, as bilateral involvement or rapid progression to corneal perforation can occur.

Management, clinical outcomes, and complications of shield ulcers in vernal keratoconjunctivitis.

PURPOSE: To assess the clinical outcomes and complications of shield ulcers by various treatment methods. DESIGN: Retrospective, interventional case series. METHODS: setting: Cornea and anterior segment service of L.V. Prasad Eye Institute, India. study population: One hundred ninety-three eyes of 163 patients clinically diagnosed with vernal keratoconjunctivitis and shield ulcers. intervention: The treatment algorithm was based on the Cameron clinical grading of shield ulcers. Grade 1 ulcers received medical therapy alone. Grade 2 and grade 3 ulcers received either medical therapy alone or medical therapy combined with debridement, amniotic membrane transplantation (AMT), or both. main outcome measures: Re-epithelialization time and best-corrected visual acuity. RESULTS: Grade 1 ulcers were seen in 71 (37%) eyes, grade 2 ulcers were seen in 79 (41%) eyes, and grade 3 ulcers were seen in 43 (22%) eyes. In the grade 1 group, re-epithelialization was seen in 67 (94%) eyes. In the grade 2 group, re-epithelialization was seen in 36 (88%) eyes that received medical treatment, in 20 (95%) eyes that underwent debridement, and in 17 (100%) eyes that underwent AMT. In the grade 3 group, re-epithelialization was seen in only 1 (1.7%) eye that received medical treatment, whereas it was seen in all eyes that underwent debridement and AMT. The mean best-corrected visual acuity after re-epithelialization of the shield ulcer was 20/30, 20/30, and 20/40 in the grade 1, grade 2, and grade 3 groups, respectively. Recurrence and secondary bacterial keratitis were seen in 28 (14.5%) and 20 (10%) eyes, respectively. CONCLUSIONS: Grade 1 shield ulcers respond well to medical therapy alone, whereas grade 2 ulcers occasionally may require additional debridement or AMT. Grade 3 ulcers, however, largely are refractory to medical therapy and require debridement and AMT for rapid re-epithelialization.

Risk factors associated with the relapse of uveitis in patients with juvenile idiopathic arthritis: a preliminary report.

PURPOSE: To identify risk factors associated with relapse of uveitis in patients with recurrent uveitis associated with juvenile idiopathic arthritis (JIA) after treatment with immunomodulatory therapy (IMT) and durable remission of 1 year. METHODS: The medical records of 30 patients with JIA-associated uveitis who were successfully treated with IMT to a state of corticosteroid-free remission and subsequently remained in remission after discontinuation of IMT for a period of at least 1 year were retrospectively reviewed. In subsequent follow-up, some patients had relapse of uveitis, whereas others continued to be in remission. Remission was defined as <1 + cells in the anterior chamber and <1 + vitreous haze grading; relapse was defined as ≥ 1 + cell in the anterior chamber or ≥ 1 + vitreous haze grading. RESULTS: A total of 30 patients were included. Of these, 17 (56.7%) patients remained in uveitic remission, whereas 13 (43.3%) relapsed. The patients in remission received IMT earlier in the course of disease compared with patients who relapsed (median, 12 months vs 72 months; P = 0.002 [Mann-Whitney test]). Patients in remission had received treatment with IMT at a younger age compared with the relapse group (median age, 7 years vs 13 years; P = 0.02 [Mann-Whitney test]). None of the other factors studied revealed a statistically significant association. CONCLUSIONS: Patients with JIA-associated uveitis who were treated with IMT earlier in the course of disease and at a younger age were associated with a lower rate of relapse of uveitis after durable remission and 1 year of quiescence, compared with similar patients who relapsed.