Drusen-like deposits in patients with SLE were independent of renal disease and were best detected with SDOCT. Lupus-related glomerulonephritis was associated with more fundus abnormalities and a screening SDOCT should be considered in all patients with SLE. Drusen-like deposits in the absence of glomerulonephritis may support the recent proposal that complement alteration is the primary cause of these lesions.
PurposeTo evaluate the long-term functional and morphological changes occurring in myopic eyes with a dome-shaped macula (DSM), with or without untreated serous retinal detachment (SRD).MethodsThis prospective, single-centre study enrolled consecutive cases of highly myopic patients with DSM with or without a SRD. Patients underwent complete ophthalmological examinations, optical coherence tomography, axial length measurements and autofluorescence. Follow-up visits were performed with a maximum interval of 6 months for 4 years. Eyes with choroidal neovascularisation were excluded.ResultsTwenty-six eyes from 18 patients (mean age 61.2) were included. At baseline, 13 eyes had SRD and 13 did not. The DSMs were either horizontal (69%) or round (31%). There were no significant differences in best-corrected visual acuity (BCVA) between eyes with and without SRD during the 48-month follow-up period. Multivariate analysis showed that baseline BCVA was the only parameter among those analysed (age and SRD height) to have a significant effect on the final BCVA (p<0.0001). SRD fluctuated overtime and SRD height was significantly influenced by choroidal thickness (p=0.002). The scleral bulge thickness had no effect on SRD thickness.ConclusionsBCVA remained clinically stable over 4 years without treatment despite the fluctuations and persistence of the SRDs.
Kyrieleis plaques (KP) represent a peculiar type of vasculitis affecting retinal arterial branches in a beaded segmental pattern that can be found in several posterior inflammatory ocular conditions. The nature and precise location of KP is unclear. Adaptive Optics (AO) provides an in vivo visualization of retinal vasculature on a microscopic level, thus permitting a more detailed characterization of KP as compared to traditional imaging techniques. This study aims to report AO imaging of KP in Varicella Zoster virus (VZV)-associated posterior uveitis and to correlate the findings with traditional imaging techniques. Three patients diagnosed with VZV posterior uveitis underwent adaptive optics (AO) imaging and traditional multimodal imaging techniques, including fundus photography, fluorescein angiography, indocyanine green angiography and optical coherence tomography. In all subjects, AO imaging revealed segmental hyporeflectivity confined to the vessel wall, with no evidence of arterial wall disruption or extravascular involvement. In our series, AO findings support the view that KP are localized within the inner arterial wall, possibly at the endothelial level.
Purpose: To compare the 2-year outcome to antivascular endothelial growth factor therapy for myopic choroidal neovascularization (CNV) in the eyes with or without domeshaped macula (DSM).Methods: Data from treatment-naive myopic CNV with a 2-year follow-up were retrospectively collected and divided into two groups according to the presence of DSM. The best-corrected visual acuity was acquired at baseline, 3, 12, and 24 months. The association between visual outcomes and CNV type and area, presence of scleral-derived feeder vessel, macular atrophy, and lacquer cracks at baseline was also evaluated.Results: Fifty-four eyes of 54 patients were included; 18 eyes (33.4%) had DSM. Choroidal neovascularization was foveal in 10 DSM eyes (55.6%) and in 30 non-DSM eyes (83.9%), P = 0.033. At baseline, the mean best-corrected visual acuity was significantly higher in the DSM group (68.33 ± 12.04 Early Treatment Diabetic Retinopathy Study letters, 20/40 Snellen) compared with the non-DSM group (57.75 ± 13.46 Early Treatment Diabetic Retinopathy Study letters, 20/72 Snellen; P = 0.007). This difference disappeared after 3 months and did not reoccur afterward. All other parameters were not significantly associated with visual outcomes.Conclusion: Overall, DSM does not represent a negative prognostic factor in response to antivascular endothelial growth factor therapy in myopic CNVs after 2 years. However, in DSM eyes, CNVs tend to be extrafoveal, thus ensuring a good visual prognosis from the earliest stage of the disease.
Background:Uveitis is the main extraarticular complication of juvenile idiopathic arthritis (JIA) with still a significant impact on JIA morbidity, despite continuous improvement in systemic treatment. Although antinuclear antibody positivity and early onset of JIA have been associated with a high risk of uveitis onset, so far no clinical features have been widely recognized as predictive factors for JIA-associated uveitis (JIA-U) response to treatment.Objectives:To investigate clinical features associated with response to systemic treatment in a long-term cohort of patients with JIA-UMethods:Clinical records of patients with JIA-U were retrospectively reviewed with regard to clinical features, therapeutic choices and outcome. Clinical and laboratory variables were compared by means of Mann-Whitney U test or chi-square/Fisher exact test, as appropriate.Results:Data from 164 JIA-U patients were analysed (81.7% female), with a median follow up of 12.1 years (7.1-17.3). Median age at JIA and uveitis onset was 2.6 (1.6-4.8) and 4.8 (2.9 – 7.0) years, respectively. Monotherapy with a conventional disease-modifying antirheumatic drug (DMARD) was used in 25.0% of patients, while 111 patients (67.7%) received at least one biologic DMARD (bDMARDs). Compared to patients responsive to DMARDs, children requiring a bDMARDs for uveitis had a lower median age at both JIA (2.4 vs 4.3 years, p 0.0234) and uveitis onset (4.1 vs 6.2 years, p 0.0023). Despite no differences in ocular damage at onset and median disease duration, patients not responsive to conventional DMARDs showed a higher frequency of ocular damage at the last visit (66.2% vs 33.3%, p 0.011). Children requiring more than one bDMARD for uveitis presented a more frequent polyarticular course (87.0% vs 20.2%, p 0.0022), a longer disease duration (median follow-up: 14.2 vs 10.4 years, p 0.0397) and a higher frequency of visual loss (best corrected visual acuity < 4/10: 23.3% vs 6.3%, p 0.0069).Conclusion:JIA-U patients with a lack of response to conventional DMARDs were significantly younger both at JIA and uveitis onset. Severe JIA-U requiring more than one bDMARDs was associated with polyarticular JIA course and longer disease duration. Children resistant to conventional treatment need prompt recognition and additional strategies to improve long-term outcome.References:[1]Heiligenhaus et al. Predictive factors and biomarkers for the 2-year outcome of uveitis in juvenile idiopathic arthritis. Rheumatology 2019.Disclosure of Interests:None declared
Background:Juvenile idiopathic arthritis (JIA) is the main cause of chronic uveitis in childhood and JIA associated uveitis (JIA-U) is the most common extraarticular complication of JIA. Despite continuous improvement in its management, pediatric uveitis still represents a serious condition with potential sight-threatening complications and a significant impact on quality of life (QoL).Objectives:To evaluate visual function (VF) and QoL in children with JIA-U and idiopathic uveitisMethods:A cross-sectional study was conducted in two tertiary Pediatric Rheumatology Centres, enrolling all patients seen with JIA-U, JIA without uveitis and idiopathic uveitis. VF was assessed by a translated form of the available EYE-Q, adapted for cross-cultural feasibility into a 10-question tool, while QoL was evaluated by the Italian version of the Pediatric Rheumatology Quality of Life scale part of the Juvenile Arthritis Multidimensional Report (JAMAR), shortened for feasibility to a 8-question tool. JAMAR section on treatment compliance and school attendance was also included. Parents, and patients when appropriate, were asked to complete each patient/parent-reporting outcome measure, answering on a 4-point Likert scale, with a total score ranging from 0 to 72 (worst condition). Medical charts were reviewed regarding JIA and uveitis features and outcome. Quantitative and qualitative variables were compared by means of Mann-Whitney U test or chi-square/Fisher exact test, as appropriate; correlations among quantitative non-parametric variables were evaluated by Spearman’s test.Results:We herein describe results from the first 100 patients enrolled (76% female), with a median age at study time of 12.8 (9.0-17.6) years. Forty-nine had JIA-U, 37 JIA without uveitis and 14 idiopathic uveitis. Uveitis was active in 14/63 patients (22.2%), with a median of uveitis duration of 9.0 years (3.6-14.8). Almost all children with uveitis were on systemic treatment (58/63, 92%) at the time of interview; 54.0% of patients presented an ocular damage, with 8.0% having a best corrected visual acuity (BCVA) < 4/10. Total score, VF and QoL scores resulted significantly higher in JIA-U patients compared to JIA without uveitis, while no differences were noticed among children with uveitis with or without JIA (Table 1). School absence was reported more frequently in JIA-U compared to JIA only (32.7% vs 10.8%, p 0.0211). VF was significantly worse in patients with ocular damage and BCVA < 4/10 (p 0.0351 and 0.0123, respectively). In patients with uveitis, VF and QoL showed a significant correlation (r 0.50, p <0.0001) especially in patients with idiopathic uveitis (r 0.74, p <0.0001).Conclusion:Visual function is a crucial component of QoL in children with uveitis and it correlates with ocular damage. Since eye involvement significantly affect QoL in patients with JIA, a specific tool widely validated and cross-cultural adapted is highly demanded in the clinical care of JIA-U patients.References:[1]Angeles-Han ST et al. The importance of visual function in the quality of life of children with uveitis. J AAPOS, 2010. Filocamo et al. A New Approach to Clinical Care of Juvenile Idiopathic Arthritis: The Juvenile Arthritis Multidimensional Assessment Report. J Rheumatol, 2011.Table 1.JIA-Un = 49Idiopathic uveitisn = 14JIAn = 37p-value*p-value#Total score5.0 (3.0-11.0)4.5(2-9.8)2.0(0-4.0)0.5739<0.0001VF score2.0 (0-3.0)1.0(0-3.0)0(0-0)0.9098<0.0001QoL score3.0(2.0-6.0)3.5(2.0-4.8)2.0(0-4.0)0.56110.0005Numbers are medians (IQR). * JIA-U vs idiopathic uveitis; #JIA-U vs JIADisclosure of Interests:None declared
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