To explore retinal abnormalities using spectral domain optical coherence tomography (SD-OCT) and OCT-angiography (OCT-A) in a highly selective cohort of patients with type I hereditary angioedema (HAE). This prospective case-control study included 40 type I HAE patients and 40 age-/sex-matched healthy subjects (HC). All participants underwent SD-OCT-scanning of retinal posterior pole (PP), peripapillary retinal nerve fiber layer (pRNFL), and optic nerve head (ONH). Superficial/deep capillary density was analyzed by OCT-A. A total of 80 eyes from 40 HAE and 40 eyes from HC were evaluated. The pRNFL was thicker in HAE than in HC in nasal superior (p < 0.0001) and temporal quadrants (p = 0.0005 left, p = 0.003 right). The ONH thickness in HAE patients was greater than in HC in the nasal (p = 0.008 left, p = 0.01 right), temporal (p = 0.0005 left, p = 0.003 right), temporal inferior (p = 0.007 left, p = 0.0008 right), and global (p = 0.005 left, p = 0.007 right) scans. Compared to HC, HAE showed a lower capillary density in both superficial (p = 0.001 left, p = 0.006 right) and deep (p = 0.008 left, p = 0.004 right) whole images, and superficial (p = 0.03 left) and deep parafoveal (p = 0.007 left, p = 0.005 right) areas. Our findings documented subclinical retinal abnormalities in type I HAE, supporting a potential role of the retinal assessment by SD-OCT/OCT-A as a useful tool in the comprehensive care of HAE patients.

ObjectiveOcular and renal microvascular damage in lupus nephritis (LN) share similar physiopathological pathways that have investigated using traditional fundus examination and high-resolution colour electroretinography. Optical coherence tomography angiography (OCTA) is a recent, non-invasive technique for imaging the microvasculature of retina and choroid. Aim of the study was to investigate through OCTA analysis the relationship between retinal microvasculature alterations and renal function and histologic features. Methods Systemic lupus erythematosus (SLE) patients with LN, SLE without renal involvement and healthy controls were recruited and accomplished an ophthalmological evaluation, including OCTA. SLE-LN patients underwent a rheumatological evaluation, including disease-related clinical and laboratory features collection and kidney biopsy examination. Results This cross-sectional study enrolled forty-six eyes of 23 LN patients, thirty-two eyes of 16 SLE patients and forty-two eyes of 21 controls. Thirteen SLE-LN patients (56.5%) displayed lupus retinopathy, 10 at moderate (77%) and 3 at severe stage (23%) by fundus oculi examination. Analysis of OCTA data showed with high/moderate accuracy a reduction of retinal capillary vessel density in both SLE and SLE-LN patients compared to controls in superficial and deep plexi.A reduction in fovea thickness and an increase in foveal avascular zone were also detected. OCTA data of LN patients correlated with LN duration, disease activity, kidney function and the presence of LN-vascular lesions at kidney biopsy. ConclusionOur results suggest the role of OCTA in early detection of systemic vascular involvement in SLE-LN patients and related kidney functional-histological impairment.

Background Both cardiovascular and complement-mediated disorders might lead to microvascular damages in anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV). We aimed at investigating, for the first time, subclinical microvascular abnormalities with non-invasive techniques in AAV patients by analyzing both retinal and nailfold capillary changes. Retinal plexi were investigated using optical coherence tomography angiography (OCT-A), while nailfold capillary changes by video-capillaroscopy (NVC). Potential correlations between microvessels’ abnormalities and disease damage were also explored. Methods An observational study was conducted on consecutive patients who met the inclusion criteria of defined diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA), age ≥ 18 ≤ 75 yrs, and no ophthalmological disorders. Disease activity was assessed by Birmingham Vasculitis Activity Score (BVAS), damage by Vasculitis Damage Index (VDI), and poorer prognosis by the Five Factor Score (FFS). Quantitative analysis of vessel density (VD) was performed by OCT-A in both superficial and deep capillary plexi. Figures and detailed analysis from NVC were performed for all subjects in the study. Results Included AAV patients (n = 23) were compared with 20 age/sex-matched healthy controls (HC). Retinal VD in superficial whole and parafoveal plexi resulted significantly decreased in AAV compared to HC (P = 0.02 and P = 0.01, respectively). Furthermore, deep whole and parafoveal vessel density was strongly reduced in AAV than HC (P ≤ 0.0001 for both). In AAV patients, significant inverse correlations occurred between VDI and OCTA-VD in both superficial (parafoveal, P = 0.03) and deep plexi (whole, P = 0.003, and parafoveal P = 0.02). Non-specific NVC pattern abnormalities occurred in 82% of AAV patients with a similar prevalence (75%) in HC. In AAV, common abnormalities were edema and tortuosity in a comparable distribution with HC. Correlations between NVC changes and OCT-A abnormalities have not been described. Conclusion Subclinical microvascular retinal changes occur in patients with AAV and correlate with the disease-related damage. In this context, the OCT-A can represent a useful tool in the early detection of vascular damage. AAV patients present microvascular abnormalities at NVC, whose clinical relevance requires further studies.

Background:Lupus Nephritis (LN) and retinopathy are organ-threatening manifestation of Systemic Lupus Erythematosus (SLE) and both share common pathophysiology represented by microvascular damage. Optical coherence tomography angiography (OCTA) is a recent non-invasive technique showing retinal vascular damage.Objectives:To analyze retinal microvascular alterations in SLE-LN patients and investigate correlations between ocular and renal involvement.Methods:We recruited SLE-LN patients and healthy controls (HC), age and sex -matched. Patients underwent rheumatological evaluation, including clinical, laboratory, kidney function and kidney biopsies examination.Patients and HC underwent a complete ophthalmological evaluation including eye definition color retinography and OCTA whole image, parafovea and fovea vessel density assessment of superficial and deep retinal capillary plexus. Parafovea and fovea thickness, fovea avascular zone (FAZ) area and perimeter were detected.Statistical analysis was performed using: χ2 test, unpaired t-test, Mann Whitney U test, Pearson or Spearman rank correlation and ROC curve analysis.Results:48 eyes of 24 SLE-LN patients and 44 eyes of 21 HC were evaluated. Table 1 shows demographic, clinical, laboratory and histological parameters.Figure 1 shows results of OCTA data and relative AUC curves and ROC analysis. Analysis of OCTA data showed a significative reduction of vessel density in SLE-LN compared to HC regarding the following parameters: superficial whole en face, parafovea and fovea density (Figure 1A-C), deep whole en face and deep fovea density, (Figure 1D-E), parafovea and fovea thickness (Figure 1F-G), FAZ area and perimeter (Figure 1H-I).OCTA data were correlated with demographic, clinical and histologic features of patients showing negative correlation between: SLE duration and both superficial (p=0.03; r=-0.3) and deep (p= 0.004; r=-0.4) whole en face density; LN duration and superficial whole en face (p=0,05; r=-0.4) and parafovea (p=0.007; r=-0.4) density, deep whole en face (p=0.004; r=-0.4) and fovea (p=0.01; r=-0.4) density and parafovea thickness (p=0.004; r=-0.3); SLEDAI-2K and both superficial and deep fovea density (p<0.0001, r=-0.6 and p=0.0, r=-0.4 respectively); BUN and superficial whole en face density (p=0.003; r=-0.5) and parafovea (p=0.004; r=-0.4) density and deep fovea density (p=0.03; r=-0.3); serum creatinine and deep whole en face density (p=0.004; r=-0.4).Positive correlation was found between LN duration and FAZ area (p= 0.01; r= 0.4); creatinine clearance and both deep whole en face (p=0.05; r= 0.3) and fovea (p=0.0007; r= 0.5) density.OCTA data analysis showed a reduction in superficial (p=0.02) and deep (p=0.009) whole en face density in patients with LN-vascular lesions assessed by kidney biopsy. In this group, patients with intimal hyalinosis showed a reduction in deep whole en face density (p=0.04) compared to those without intimal hyalinosis.Conclusion:Preliminary results suggest a correlation between retinal microvascular alterations and kidney function and histologic lesions encouraging the use of OCTA measurement as a potential biomarker of systemic vascular involvement.Table 1.SLEN=24HCN=21P valueAge (years)44.4±13.838.3±10.4nsFemale (n/%)21/87.517/81nsBCVA (logMAR)0.01± 0.050.0 ± 0.1nsDisease duration (months)177.6± 126.6/LN duration (months)108 ± 97/SLEDAI-2K6.8±5/Creatininemia (mg/dl)0.9±0.3/BUN (mg/dl)39,6±17.6/Creatinine clearance (ml/min)99.2±53.7/Proteinuria (mg/24h)432.8±524.5/GMN class III-IV (n/%)19/77.7/GMN class III-V (n/%)5/22.2/Kidney biopsy active lesions (n/%)15/62.5/Kidney biopsy chronic lesions (n/%)15/62.5/Kidney biopsy vascular lesions (n/%)8/33.3/Kidney biopsy intimal hyalinosis (n/%)7/29/Moderate stage lupus retinopathy (n/%)20/83.3/Severe stage lupus retinopathy (n/%)3/12.5/BCVA: best-corrected visual acuity, SLEDAI-2K: Systemic Lupus Erythematosus Disease Activity Index 2000, BUN: blood urea nitrogen; GMN: glomerulonephritisDisclosure of Interests:None declared

BackgroundBoth cardiovascular and complement-mediated disorders might lead to microvascular damage in anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV). No evidence of subclinical microvascular retinal abnormalities neither their potential correlation with capillaroscopy anomalies has been reported in eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA).ObjectivesWe aimed at investigating subclinical microvascular abnormalities by analyzing retinal and nailfold capillary changes in an AAV cohort. Retinal plexi were investigated using optical coherence tomography angiography (OCT-A), while nailfold capillary changes with videocapillaroscopy (NVC). Potential correlations between OCT-A abnormalities and both disease damage and periungual capillaries change were also explored.MethodsA monocentric observational study was conducted on consecutive AAV. Main inclusion criteria were a defined diagnosis of EGPA/GPA/MPA in accordance with International Criteria, age ≥18 ≤ 75 yrs, intraocular pressure (IOP) <21 mmHg by Goldmann, a best-corrected-visual-acuity (BCVA) ≥ 0,5 logMAR, and no ophthalmological and/or systemic disorders or treatment with known retinal involvement. Disease activity was assessed by Birmingham Vasculitis Activity Score (BVAS), damage by Vasculitis Damage Index (VDI), and poorer prognosis by the Five Factor Score (FFS). Moreover, OSDI and MIDAS questionnaires have been administered. Quantitative analysis of vessel density (VD) was performed by OCT-A in the superficial capillary plexi (SCP) and deep capillary plexi (DCP) for all subjects. For completeness, a structural analysis of retinal thickness was performed by OCT-scans. Figures and detailed analysis from NVC were performed for all AAV patients.ResultsFrom 47 consecutive AAV patients referring to the Rheumatologic Clinic, 23 consecutive patients who met the inclusion criteria were included and compared with 20 age/sex-matched healthy subjects (HC) (Table 1). In AAV, BVAS correlated directly with VDI (P=0.001) and FFS (P=0.01) while it was inversely related with disease duration (P=0.01). A total of 46 eyes from AAV patients were analyzed. Retinal VD in superficial whole (SWD) and parafoveal (SPFD) plexi were significantly decreased in AAV compared to HC (P=0.02 and P=0.01, respectively, Figure 1A-B). Furthermore, deep whole (DWD) and parafoveal vessel density (DPFD) were strongly reduced in AAV than HC (P ≤0.0001 for both, Figure 1C-D). In AAV patients, significant inverse correlations emerged between VDI and OCTA-VD in both the superficial (parafoveal, P=0.03) and the deep plexi (whole, P=0.003, and parafoveal P=0.02). The retinal thickness measured by OCT-scans was similar between AAV patients and HC.At NVC examination we found non-specific pattern abnormalities in 82% of AAV patients. Common abnormalities were pericapillary edema (73%), tortuosity (65%), while rare cases of ectasias and hemorrhages resulted (0.8%). No meandering capillaries nor empty dermal papillae were observed. Correlations between NVC changes and OCT-A abnormalities were not described.ConclusionSubclinical microvascular retinal changes occur in patients with AAV and correlate with the disease-related damage. In this context, the OCT-A can represent a useful tool in the early detection of vascular damage. AAV patients present microvascular abnormalities at NVC, whose clinical relevance requires further studies.Table 1.AAV (n=23)GPA, N/%5/22EGPA, N/%9/39MPA, N/%9/39Age at the study (yrs, mean ± SD)60.9 ± 8.7Disease duration (yrs, mean ± SD)9.6 ± 9.1ENT, N/%15/65Kidney, N/%4/17Heart, N/%4/17Lung, N/%21/91Skin, N/%6/26Joint, N/%9/39Peripheral Nervous System, N/%13/57ANCA positivity, N/%16/69.6BVAS (mean ± SD)3.3 ± 2.5VDI (mean ± SD)4.5 ± 1.5FFS (mean ± SD)0.3 ± 0.4Figure 1.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.