Background: According to the recent recommendations for Systemic Lupus Erythematosus (SLE), a progressive tapering until withdrawal of glucocorticoids (GC) is considered one of the main goals of SLE management (1). However, which patient may be a candidate for safe GC withdrawal has not been determined yet and a proportion of patients are kept on long-term low-dose prednisone despite clinical remission. Objectives: to evaluate the rate of low-dose GC withdrawal in SLE patients in remission and to identify predictors of flares. Methods: Eligible patients were SLE patients according to the ACR criteria (2) who were in prolonged clinical remission defined by a cSLEDAI=0 for at least 2 years and on a stable SLE treatment (immunosuppressive drugs and/or hydroxychloroquine (HCQ) and daily 5 mg prednisone). A SACQ period was defined as at least 1-year period with persistent serologic activity without clinical manifestations. Flares were defined by SELENA-SLEDAI Flare Index (3). Damage was assessed by SLICC damage index (SDI). Data were compared by the unpaired student’s t test or chi-squared test as appropriate. Predictors of flares after GC withdrawal were analyzed by Cox regression. Results: Out of 246 SLE patients registered in the Naples Lupus Clinic database, 132 eligible patients were identified. Among them, we selected 57 (43%) patients in whom a GC withdrawal was attempted. 75 (57%) patients were in the prednisone maintenance group. There were no significant differences between the two treatment groups (table 1). The proportion of patients experiencing a flare was not significantly lower in the maintenance group than in the withdrawal group (15/75 vs 16/57; p=0.28). Moreover, the proportion of patients who had an increase in the SDI at the end of follow up was similar between the two groups (14/75 vs 8/57; p=0.48). However, among the withdrawal group, the rate of flares was significantly higher in SACQ patients (10/22 vs 6/35; p=0.02), while the majority of serologically inactive patients (82%) successfully stopped GCs without subsequent flares. At Cox regression analysis (Table 2), duration of HCQ therapy and >4 year remission at withdrawal were protective factors, while a SACQ disease and history of lupus nephritis (LN) increased the risk of disease flare. Table 1. Baseline characteristics of 132 patients at study entry Characteristics Withdrawal group (n=57) Maintenance group (n=75) p-value Female, no. (%) 54 (94) 70 (93) 0.73 Age, years 26.7±10.1 28.5±11.7 0.37 Disease duration, years 8.5±2.9 9.1±12.9 0.73 History of lupus nephritis, no. (%) 13(22) 22(29) SDI score 0.40±0.7 0.57±0.8 0.26 Immunosuppressive drugs, no. (%) 31 (54) 33(44) 0.16 HCQ, no. (%) 52 (91) 66 (88) 0.06 Low C3, no. (%) 28 (49) 41(54) 0.52 Increased dsDNA Ab, no. (%) 11 (19) 19 (25) 0.41 Table 2. Factors predicting lupus flares during follow-up at Cox regression analysis Variables HR 95% CI p value SACQ 2.99 1.08 – 8.25 0.03 Age 0.97 0.93 – 1.02 0.29 Disease duration 0.99 0.94 – 1.04 0.84 History of LN 3.38 1.22-9.33 0.01 SDI score 1.13 0.63 – 2.01 0.66 Immunosuppressive drugs 2.39 0.86 – 6.62 0.09 HCQ, ever 2.92 0.43 – 35.2 0.95 Duration of HCQ 0.84 0.72 – 0.98 0.03 5years remission 0.12 0.04 – 0.39 0.0003 Conclusion: GC withdrawal is an achievable target in SLE and may be attempted in patients in complete remission. In SACQ patients, maintenance of 5mg prednisone is superior to its withdrawal in order to prevent flares. Long-term HCQ therapy and prolonged remission can significantly reduce the risk of disease relapse after GC withdrawal. References: [1]Fanouriakis A, et al. Ann Rheum Dis. 2019;78(6):736–45. [2]Tan EM, et al. Arthritis Rheum. 1982;25(11):1271–7. [3]Petri M, et al. Lupus. 1999;8(8):685–91. Disclosure of Interests: Serena Fasano: None declared, Luciana Pierro: None declared, Melania Alessia Coscia: None declared, laura Bucci: None declared, silvia scriffignano: None declared, Antonella Riccardi: None declared, francesco ciccia Grant/research support from: pfizer, novartis, roche, Consultant of: pfizer, novartis, lilly, abbvie, Speakers bureau: pfizer, novartis, lilly, abbvie

Objective To evaluate the impact of duration of remission on the health-related quality of life (HRQoL) of patients with systemic lupus erythematosus (SLE). Methods We conducted a 5-year retrospective study on two Italian cohorts. Remission was defined as a continuative period of no clinical disease activity, according to the Systemic Lupus Erythematosus Disease Activity Index 2 K, and a permitted maximum prednisone dose of 5 mg/day. HRQoL was measured using the 36-Item Short-Form Health Survey (SF36) during the last visit. Results We enrolled 136 female SLE patients. During observation, 15 (11%) patients had been in remission for ≥1 and <2 years, 15 (11%) for ≥2 and <3 years, 19 (14%) for ≥3 and <4 years, 9 (7%) for ≥4 and <5 years, and 53 (39%) had been in prolonged remission for ≥5 years. In the multivariate model, considering depression and fatigue as covariates, patients in prolonged remission showed significantly better scores in the physical functioning ( p = 0.039), role physical ( p = 0.029), bodily pain ( p = 0.0057), general health ( p = 0.0033) and social functioning ( p = 0.0085) components of the SF36, compared with those in remission <5 years or unremitted. Subsequent mediation analyses found that these effects were partly influenced by depression. Conclusion Lupus remission could improve the HRQoL of SLE patients, particularly when associated with appropriate management of depression and fatigue.

Objective. To identify the distribution of patients with systemic lupus erythematosus (SLE) in clusters according to the levels of health-related quality of life (HRQoL), entity of pain, fatigue and depression. Methods. We performed a hierarchical cluster analysis. The following measures were used as clustering variables, after canonical transformation: the SF36 physical and mental component summary (PCS and MCS), the Beck Depression Inventory II (entity of depression), the Facit-Fatigue, all assessed during the last visit. Consecutive SLE patients were enrolled from two Italian cohorts. Lupus remission was retrospectively assessed over a period of 5 years before the last visit and was defined as a continuative period of no clinical disease activity according to SLEDAI2K and the maximum dose of prednisone allowed of 5 mg/day. Results. We enrolled 130 female SLE patients. We identified three clusters. The first cluster (43 patients) was characterised by the highest levels of MCS and PCS and the lowest entity of pain, fatigue and depression. Cluster 2 (35 patients) was defined by a reduction of MCS and increase of pain, fatigue and depression; conversely, PCS levels were similar to cluster 1. In cluster 3 (52 patients) we found a reduction of MCS and increase of depression and fatigue (similar to cluster 2) but also a decrease in PCS levels and Bodily Pain (meaning increase in pain). In cluster 3 we found a decreased prevalence of remission >= 5 years. Conclusion. Identification of clusters of patients according to HRQoL levels could be useful to improve SLE management, aiming at personalised medicine.

Background Patients with Systemic Lupus Erythematosus (SLE) present an increased incidence of Cardio-Vascular Events (CVE) compared to general population, and the difference with healthy subjects is particularly evident in young SLE women. Objectives The aim of this study is to assess the predictive ability of established 10 years CV risk models in SLE Methods A retrospective analysis of two Italian SLE prospective cohorts was performed. SLE patients without previous CVE, with age ≥25 years, a minimum continuative follow-up of 10 years and sufficient data to calculate the 10 years risk scores were enrolled. The 10 years CVE risk scores were calculated at the first observation and all CVE were prospectively recorded in the following 10 years. We calculated the following scores: the QRisk3, the Framingham CV disease 10 years score, the HeartScore (Europe Low Risk) and the SLE CV Risk Score proposed by Petri et al. Discriminatory ability for CV risk prediction was estimated by the area under the receiver operating characteristic curve. Hosmer-Lemeshov (HL) tests was used to evaluate calibration comparing the observed versus expected number of events Results Analysis was performed on 131 SLE patients (mean baseline age of 37±11 years). We observed 10 CVE during the 10 years follow-up from baseline (3 acute coronary syndrome, 4 stroke, 1 transitory ischaemic attack and 2 peripheral artery disease). The AUC values were 0.75 (95% CI 0.55–0.94) for QRisk3, 0.66 (0.45–0.88) for Framingham score, 0.62 (0.41–0.82) for the HeartScore and 0.7 (95% CI 0.55–0.85) for the SLE CV risk score. The p values of HL test were 0.8 for Qrisk3 and SLE CV score and 0.4 for Framingham score and HeartScore, suggesting a good model fit for all the CV risk scores. Considering scores with better discriminative ability and calibration, 20% of CVE were observed with Qrisk3 score lower then 3.6% and with SLE CV risk score between 6% and 8%. Discriminative ability and calibration were not improved by multiplying by 2 the Framingham score and the HeartScore. Conclusions The available CV risk scores demonstrate a moderate predictive ability of 10 years CVE in SLE. We observed a better model fit for QRisk3 and SLE CV risk score. Nevertheless, a considerable proportion of patients, with very low predicted CV risk, developed CVE during follow-up. References [1] Petri MLS. Systemic lupus erythematosus Cardiovascular Risk Equation. [Abstract]. Arthritis Rheum2012;64. [2] Urowitz MB, Ibañez D, Su J, et al. Modified Framingham Risk factor score for systemic lupus erythematosus. J Rheumatol2016;43:875–9. Disclosure of Interest None declared

Background Previous study from our group have pointed out a lower number of cardiovascular (CV) events in Italian patients with Systemic Lupus Erythematosus (SLE) than in North European and American ones. This study aims to assess the incidence of the first CV event in a large, multicenter, Italian cohort of patients with SLE and search for differences in disease and traditional risk factors among distinct cohorts. Methods Clinical charts of SLE patients consecutively admitted to five Italian rheumatologic centres from November 1 st 2000 and December 31 st 2015 were retrospectively studied. Patients selected were free of CV events at baseline. CV incidence rate was expressed as the number of events in the cohort divided by the total number of years at risk. CV cumulative incidence were evaluated as the proportion of patients who experienced a new CV event over the follow up period. Our incidence was compared with that detected in the Italian general population and those reported in SLE cohorts from other countries. Results The median duration of follow-up was 6 years (IQR=3–11). During the observational period, 37(cumulative incidence=7.2%) patients had a first episode of CV event with an incidence rate of 10.1/1000 person-years i.e 12 times higher than in the general population. The CV incidence rate and cumulative incidence detected in our Italian cohort was lower than those from North European and American cohorts. The Italian cohort differed from other SLE cohorts in some traditional risk factors (smoke, hypertension, dyslipidemia) and treatment with aspirin and hydroxychloroquine. Conclusion Our results confirmed that Italian lupus patients suffer a high incidence of CV disease compared with general population. However, this incidence was lower than that detected in North European and American lupus cohorts

BackgroundCardiovascular disease (CVD) has emerged as one of the most important causes of mortality in systemic lupus erythematosus (SLE)1. In previous studies, disease activity, as assessed by SLEDAI (at the first visit or as mean annual value registered during follow-up), did not result to have any predictive role on the subsequent occurrence of CVD2–3.ObjectivesTo investigate the relationship between prolonged remission and the occurrence of a subsequent first CV event in patients with SLE.MethodsOut of 452 patients consecutively admitted to two tertiary Italian centres from November 1st 2000 to December 31st 2016, the 409 patients, who, at admission, had not experienced any CV event, had not received any anticoagulation therapy and had been visited at least biannually during follow-up, were considered for the present study. Prolonged remission was defined as a 5 year consecutive period of no disease activity based on SLEDAI-2K3.. Patients with prolonged remission were furtherly subdivided according to Zen et al4 into 3 groups: complete remission, clinical off-corticosteroids remission (offCR), clinical on-corticosteroids remission (onCR). Kaplan-Meier curves and the log-rank test were used to analyse differences in event-free survival between groups. Cox regression analysis was used to investigate disease and therapeutic features associated with the development of a first CV event.ResultsDuring 72 months median follow-up time, 29 (7.0%) CV events occurred (two events in patients who had undergone prolonged remission). Out of the 409 patients, 28 patients (6.8%) achieved a prolonged complete remission, 13 (3.1%) prolonged clinical offCR and 64 (15.5%) prolonged clinical onCR. Kaplan-Meier analysis revealed a greater overall CV event-free rate in patients achieving a prolonged remission compared to those in remission but for less than 5 years and patients not in remission (logrank test χ2=19.82; p=0.0001; figure 1). However, at Kaplan-Meier analysis, CV outcome was similar among patients in prolonged remission, irrespective of the type of remission achieved (p>0.05). At multivariate analysis, treatment with hydroxychloroquine for more than 5 years and prolonged remission were protective (HR 0.38; 95% CI 0.16–0.90; HR 0.08, 95% CI 0.01–0.53) while antiphospholipid syndrome increased the risk of a first CV event (HR 3.80; 95% CI 1.68–8.61). No differences were found between patients treated or not with aspirin. Nevertheless, among patients from Rome cohort, aspirin was only prescribed to patients with high traditional CV risk score.ConclusionsA prolonged remission, whichever the subtype, is associated with a better CV outcome and should be considered as a treat-to-target goal in the CV risk management of the lupus patient.References[1] Cervera R, et al. Medicine (Baltimore)2003. [2] Iudici M, et al. Rheumatology (Oxford)2016. [3] Fasano S, et al. Lupus2017. [4] Zen M, et al. Ann Rheum Dis2017.Disclosure of InterestNone declared

Background Systemic lupus erythematosus is associated with an increased risk of cardiovascular disease. Low-dose aspirin, hydroxychloroquine and statins have been suggested to play a prophylactic role of cardiovascular events. This study is devoted to reviewing the literature on the topic and assessing the effects of these drugs in preventing a first cardiovascular event in a two-centre Italian series. Methods A PubMed search on cardiovascular prevention in systemic lupus erythematosus was performed. Moreover, systemic lupus erythematosus patients admitted to two centres from 2000–2015, who at admission had not experienced any cardiovascular event, were investigated. Aspirin, hydroxychloroquine and statin use, and the occurrence of any cardiovascular event, were recorded at each visit. Kaplan-Meier and Cox regression analyses were performed to evaluate the role of traditional, disease-related cardiovascular risk factors and of each of the three drugs in the occurrence of new cardiovascular events. Results The literature search produced conflicting results. Two hundred and ninety-one systemic lupus erythematosus patients were included in the study and followed for a median of eight years. During follow-up, 16 cardiovascular events occurred. At multivariate analysis, taking aspirin (hazard ratio: 0.24) and hydroxychloroquine for more than five years (hazard ratio: 0.27) reduced, while antiphospholipid antibody positivity (hazard ratio: 4.32) increased, the risk of a first cardiovascular event. No effect of statins emerged. Conclusion Our study confirms an additive role of aspirin and hydroxychloroquine in the primary prophylaxis of cardiovascular events in Italian patients with systemic lupus erythematosus. The lack of any detected effect in previous reports may depend on the design of studies and their short follow-up period.

Background Systemic lupus erythematosus (SLE) is associated with an increased cardiovascular (CV) risk (1). By retrospectively investigating a one centre cohort, we have recently reported that low-dose aspirin (ASA) use is associated with a reduced CV risk in SLE (2) and long-term hydroxychloroquine (HCQ) exposure may have an additive effect (3). Objectives This study was conducted on 2 Italian SLE cohorts to confirm these results and assess the role, if any, of statins. Methods Clinical charts of SLE patients consecutively admitted to 2 University Rheumatology Units from November 2000 to December 2014 who, at admission, had not experienced any CV event, were investigated. ASA, HCQ and statins use and the occurrence of any CV event, were recorded at each visit. Kaplan-Meier analysis was performed to determine the HCQ exposure status associated with a higher CV-free survival. Cox regression analysis was carried out to identify factors independently associated with a first CV event. Results A total of 291 SLE patients were included in the study and followed for a median of 8 years. During follow-up, 16 CV events occurred. Kaplan Meier analysis revealed a greater CV event-free rate in the 120 ASA-treated patients taking HCQ at standard dose for more than 5 years than in the 98 patients treated with ASA alone or with HCQ for less than 5 years (Figure 1). At univariate analysis, patients with a first CV event compared with those without any thrombotic events were antiphospholipid antibody (aPL) positive (P=0.017 HR 2.91) and had significantly higher blood pressure (P=0.017 HR 3.58), hypercholesterolemia (P=0,015 HR 3.40) and higher disease damage at last visit (P=0,032 HR 1.56). Moreover, ASA treatment (P=0,012 HR 0.27) and HCQ use (P=0,012 HR 0.26) for more than 5 years were negative predictors, while statins use did not show any association (P=0.619). All other variables examined, including smoking, obesity, hypertriglyceridaemia, diabetes mellitus, disease activity, severe SLE, other medications (immunosuppressive agents, steroids) were not associated, either positively or negatively, with the occurrence of CV events. At multivariate analysis, taking ASA and HCQ for more than 5 years were protective against thrombosis (HR 0.24 and HR 0.27, respectively), while aPL positivity (HR 4.32) increased the risk of a first CV event. Conclusions Use of antimalarials for more than 5 years is associated with a reduced risk of a first thrombosis in SLE patients and the HCQ-ASA combination seems to synergistically reduce further the CV risk. Larger, prospective studies are needed to provide a better definition of the role of these drugs in CV primary prevention in SLE. References Cervera R, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients.Medicine (Baltimore). 2003. Iudici M, et al. Low-dose aspirin as primary prophylaxis for cardiovascular events in systemic lupus erythematosus: a long-term retrospective cohort study. RheumatolOxfEngl 2016. Fasano S, et al. Long-Term Hydroxychloroquine Therapy and Low-Dose Aspirin May Have an Additive Effectiveness in the Primary Prevention of Cardiovascular Events in Patients with Systemic Lupus Erythematosus [abstract]. Arthritis Rheumatol 2016. Disclosure of Interest None declared

Background/objective The objectives of this paper are to assess the extent of and the factors associated with hydroxychloroquine (HCQ) non-adherence in systemic lupus erythematosus (SLE) patients with prolonged inactive disease and to investigate relationships between blood HCQ concentration and quality of life (QoL). Methods Consecutive SLE patients, in remission for at least one year and taking a stable dose of HCQ were investigated. At study entry (T0) and six months later (T6) a blood venous sample was taken to measure whole blood concentration of [HCQ] and desethylchloroquine ([DCQ]). Moreover, at T0 each patient completed validated questionnaires assessing QoL, disability, anxiety, depression and visual analogue scales for fatigue, pain, general health (GH), and self-assessment of disease activity. Results Eighty-three patients with a median [HCQ] of 327 ng/ml were enrolled. At T0, 24 (29%) were defined as non-adherent ([HCQ] < 100 ng/ml). At multiple logistic regression analysis the physical summary of SF-36 ( p = 0.038), and the concomitant use of immunosuppressants ( p = 0.010) were independently associated with non-adherence. A significant increase of HCQ adherence was observed at T6 ( p < 0.05). Conclusions A better health status and the concomitant prescription of immunosuppressants represent risk factors for HCQ non-adherence in SLE patients in remission. Monitoring HCQ levels might represent an important opportunity to improve adherence.

PurposeTo describe multimodal imaging features of choroidal osteoma (CO) complicated by choroidal neovascularization (CNV) and focal choroidal excavation (FCE).MethodsPatients presenting with CO and CNV between January and October 2016 were considered for this study. Diagnosis of CO was confirmed by ultrasound examination. All patients underwent multimodal imaging including optical coherence tomography (OCT), swept-source OCT angiography (DRI OCT Triton, Topcon, Inc., Tokyo, Japan) and fluorescein angiography (Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany).ResultsTwo patients (one with bilateral CO) were included in the study. OCT showed a FCE in two eyes of two patients (one in correspondence of the CNV and the other adjacent to the CNV). OCT-A demonstrated presence of microvascular flow within neovascular network of the CNVs. Decalcification of the tumor was noted in correspondence of one eye with FCE.ConclusionsFCE may be found in eyes with choroidal osteoma and CNV. OCT-A was a valuable tool for detection of CNV complicating choroidal osteoma. Decalcification of choroidal osteoma may represent a common pathogenic pathway for development of FCE and CNV in choroidal osteoma.

PURPOSE:To describe the optical coherence tomography angiography (OCTA) features of diabetic retinopathy.METHODS:Literature review and case series.RESULTS:Four cases are presented.CONCLUSION:OCTA is an effective method for evaluating retinal changes in diabetic retinopathy and represents a novel complement or alternative to fluorescein angiography. Although OCTA should currently be considered an investigational technique, in the near future, it may play key roles in the diagnosis and management of diabetic retinopathy.

Background Cardiovascular (CV) morbidity and mortality are significantly greater in Systemic Lupus Erythematosus (SLE) patients than in the general population. Acetylsalicylic acid (ASA) is known to be associated with a decrease in the incidence of CV events in high-risk patients from general population [1]. Objectives We previously investigated ASA efficacy as primary prophylaxis in 167 SLE patients followed for 7 patient-years [2]. Here, we report data collected in 189 patients followed for 8,2 patient-years. Methods SLE patients consecutively admitted to a tertiary center, who, at admission, satisfied 1992 ACR and/or 2012 SLICC classification criteria for SLE and had not experienced any CV event, were enrolled into the study. Low-dose ASA (100 mg/day) was prescribed to each patient at first visit. Fourteen patients (7%) refused to take it. At each 6-monthly visit, ASA side effects, ASA discontinuation and occurred CV events were recorded. Results 189 consecutive SLE patients were followed-up for a median of 8 years (range 1–15). Among them, 164 regularly took the medication (ASA-treated) and 25 refused to take or discontinued it (non-ASA-treated patients). Six CV events occurred in the 164 ASA-treated (4.2/1000 person-years), 4 in the 25 non-ASA-treated patients (25.4/1000 person-years) (p=0.003). No relevant side effect related to ASA treatment was recorded. Conclusions Present data further support the role of ASA in the primary prophylaxis of CV events in SLE patients. References Vandvik PO, Lincoff AM, Gore JM, et al. Primary and secondary prevention of cardiovascular disease: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines.Chest. 2012;141 Iudici M, Fasano S, Gabriele Falcone L, et al. Low-dose Aspirin as Primary Prophylaxis for Cardiovascular Events in Systemic Lupus Erythematosus: A Long-Term Retrospective Cohort Study. Rheumatology (Oxford) Submitted for publication. Disclosure of Interest None declared

Background Sleep disturbances are frequently observed in systemic lupus erythematosus (SLE) patients, but the relationship with disease activity has not been unequivocally established. Objectives To assess the prevalence of and factors associated with sleep disorders in a cohort of SLE patients with persistent complete (no clinical activity, no serological activity and no treatment other than antimalarials) or clinical remission (only serological activity; stable immunosuppressive therapy with or without corticosteroids allowed) (1). Methods Consecutive adult SLE patients attending the outpatient clinic of the Second University of Naples were enrolled in a cross-sectional study. A complete clinical and laboratory assessment was carried out. Moreover, the Pittsburgh Sleep Quality Index (PSQI), Health Assessment Questionnaire – Disability Index (HAQ-DI), Short-Form 36 (SF-36), VAS pain, VAS fatigue, VAS global health, were administered. Results Ninety SLE patients (86 females) with a mean±SD age of 43±18 and a median disease duration of 17 years (range 2–38) were enrolled in the study. A poor sleep quality (i.e. PSQI score>5) was observed in 38 (42.2%) patients, with no difference between patients in complete remission (13/30) and those in clinical remission with or without GCs (25/51; p=0.651). PSQI score was found to be significantly correlated with physical component score of SF-36 (rho -0.467, 95% CI -0.632 to -0.262; p<0.0001), mental component score of SF-36 (rho -0.501, 95% CI -0.657 to -0.303; p<0.0001), HAQ-DI (rho 0.497, 95% CI 0.303 to 0.651; p<0.0001), VAS pain (rho 0.480, 95% CI 0.283 to 0.639; p<0.0001), VAS fatigue (rho 0.491, 95% CI 0.296 to 0.697; p<0.0001). No correlation was found between the physician assessment of disease activity and sleep disturbance (rho 0.113, 95% CI -0.114 to 0.128; p=0.329). Conclusions Sleep disorders are frequent in SLE patients with persistent complete or clinical remission. An impaired mental and physical quality of life, disability, fatigue and pain represent the main features associated with a low quality of sleep. References Zen M, et al. Prolonged remission in Caucasian patients with SLE: prevalence and outcomes. Ann Rheum Dis 2015;74:2117–22 Disclosure of Interest None declared

Background The early identification of Raynaud’s phenomenon (RP) suspected secondary to a connective tissue disease (CTD) is fundamental to treat promptly the related organ manifestations. Among the potential clinical complications, eye involvement is often overlooked, even if it is known that systemic vascular dysregulation is able to induce vasospasm in the retinal vessels and retinal blood flow abnormalities. Objectives To evaluate the presence of vascular eye involvement measuring the choroidal and macular thickness in a cross-sectional cohort of RP suspected secondary to a CTD. Methods Consecutive RP suspected secondary to a CTD were enrolled. Inclusion criteria were: 1) RP; 2) nosymptoms/signs suggesting a CTD; 3) major capillary abnormalities at nailfold capillaroscopy and/or positive anti-nuclear antibodies (ANA) by IFF and blotting; 4) no visual symptoms. Patients underwent a complete ocular examination including: best corrected visual acuity, slit lamp biomicroscopy, intraocular pressure measurements and fundus examination. Choroidal thickness (CT) was assessed using the Zeiss Cirrus Spectral Domain Optical Coherence Tomography (SD-OCT) with enhanced depth imaging (EDI) scan system at the fovea and up to 1 mm at intervals of 2 mm from the fovea in the superior, inferior, nasal and temporal choroid; central fovea thickness (CFT) was also measured. 26 healthy, sex and aged-matched, subjects were analysed as control group. Statistical analysis was performed by Mann-Whitney test. Results 20 subjects were included in the study (mean age 51.85 years, 18 females), 75% (15 out of 20) had positive ANA test, of whom 11 (55%) with anti-centromere pattern. 13 out of 20 (65%) showed scleroderma pattern at nailfold capillaroscopy. Slit lamp biomicrocopy was within normal limits in all patients and fundus examination revealed normal arterial and venous vessels with no capillary abnormalities. The mean best corrected visual acuity was 20/40 and the mean intraocular pressure measurement was 14 mmHg. Choroidal thickness measurements were significantly thinner than healthy control in the subfoveal region (mean 256.82 µm vs 313.22 µm; p=0.0031). Similar results were observed in the superior, temporal and nasal sectors of the retina at 1 and 2 mm from the fovea: inner superior (mean 250.48 vs 293.22; p=0.0014), outer superior (mean 241.77 vs 293.3; p= 0.016); inner nasal (mean 231.97 vs 297.44; p= 0.0003), outer nasal (mean 185.12 vs 289; p= 0.0001); inner temporal (mean 262.91 vs 313.22; p=0.0031), outer temporal (mean 241.21 vs 282.56; p= 0.045). Choroidal thickness was overall thinned in the inferior sectors: inner inferior (mean 258.29 vs 293.15; p= 0.0003), outer inferior (mean 252.29 vs 289.89; p= 0.067). CFT was also thinner in RP suspected secondary to a CTD than in healthy controls (mean 257.44 vs 275.18; p=0.0061). Conclusions Thinner choroidal thickness was observed in RP suspected secondary to a CTD without evident visual symptoms. This suggests a compromisedchoroidal perfusion, and the presence of an early involvement of ocular microcirculation since the very early phase of a scleroderma spectrum disease. Disclosure of Interest None Declared

Purpose Pathologic myopia (PM) is frequently complicated by choroidal neovascularization (CNV). Diagnosis is mainly clinical and angiographic but in recent years optical coherence tomography (OCT) has been noted to add important information. The authors report on the successfully OCT-guided photodynamic therapy (PDT) of an angiographic occult CNV complicating PM. Methods Observational case report. Fluorescein angiography with a confocal SLO (HRA, Heidelberg Engineering, Germany) and OCT Stratus (Carl Zeiss Meditec, Inc.) imaging were used for diagnosis and monitoring of the CNV. Standard PDT was performed. Results A highly myopic 17-year-old girl complained of a drop in visual acuity (VA) in left eye (LE), dating back a few weeks. Her best-corrected (BC) VA was 20/40 in the LE, with some metamorphopsia. No hemorrhage or evident signs of CNV were visible either at fundus or at dynamic fluorescein angiography. OCT scans indicated a slight elevation of the RPE-choriocapillary complex with rarefaction of neuroretinal tissue that has been interpreted as CNV. PDT was then performed. LE BCVA had improved to 20/25, metamorphopsias disappeared, and at OCT examination no retinal morphologic modification was evident. Nine months later, BCVA and ophthalmoscopy are still stable. Conclusions In this case, OCT was the fundamental tool for the correct diagnosis and post-therapy monitoring of CNV-complicated PM. The CNV, not clearly detectable using angiographic imaging, was treated with PDT, and results in terms of VA and anatomic resolution were good.

The purpose of this article is to describe a case of bilateral neovascularization complicating Best Disease. A 12-year-old patient with bilateral neovascularization was managed with observation in the right eye and surgical removal in the other eye. Visual acuity, biomicroscopy and fluorangiography were carried out from 1997 to 2005. The right eye did not experience any change in visual acuity from baseline (20/50) while left eye varied from 20/200 to 20/32. Macular exudative-hemorrhagic manifestations resolved bilaterally. Eight years later, VA and retinal findings were unchanged. In this case, although VA was reasonably good in both eyes, it is difficult to assess the prognosis of surgically excised neovascularization vs natural history, also considering that nowadays photodynamic therapy and antiangiogenetic drugs are considered the therapy of choice in subfoveal neovascularization.