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Purpose and context We report two cases of optic neuritis following Pfizer-BioNTech COVID-19 vaccination in patients with autoimmune diseases. Post-vaccine optic neuritis, although rare, is reported in the literature and an autoimmune mechanism seems to be involved, especially in genetically predisposed subjects. Method Report of two cases of optic neuritis following COVID-19 vaccination in patients with autoimmune diseases. Key results He novelty of this article is the fact that the two patients had a medical history of autoimmune diseases (Hashimoto thyroiditis and ankylosing spondylitis), a point of interest for research because it is not yet clear whether this could constitute a risk factor for adverse events after vaccination. Conclusions The reactogenicity of COVID-19 mRNA vaccine in individuals suffering from immune-mediated diseases is yet to be elucidated and it is being increasingly investigated. Our paper supports the hypothesis that patients who are already affected or predisposed to autoimmune or autoinflammatory disorders should be carefully evaluated for the benefits and risks of COVID-19 mRNA vaccination.

Introduction: Incidental finding of mass lesion in the choroid represents a very challenging situation for the ophthalmologist. We describe a case of an incidental, computed tomography (CT)-hyperintense, choroidal lesion in a patient with parotid malignancy and renal failure, and how multimodal imaging helped us reaching the correct diagnosis. Case description: A 63-year-old man with parotid gland malignancy was brought to our attention because preoperative staging CT showed a hyperintense choroidal lesion of the right eye. Fundus examination showed a yellow elevated lesion near the superior temporal branch retinal artery. Enhanced depth imaging optical coherence tomography (EDI-OCT), A- and B-scan ultrasonography, and Indocyanine Green Angiography (ICGA) allowed us to exclude a malignant lesion and to diagnose a sclerochoroidal calcification. Conclusion: Multimodal imaging can guide the clinician to choose the appropriate therapeutic approach even in case of uncommon conditions like sclerochoroidal calcification.

To evaluate the correlations between anatomical and functional changes in idiopathic macular hole (IMH) surgery in long-term follow-up. This is a prospective, interventional single centre case series. The final sample is formed by 14 eyes of 14 patients who had undergone IMH surgery in our institution between 2005 and 2009 and were still followed up in 2019. Reported data were pre- and post-operative best-corrected visual acuity (BCVA), retinal sensitivity and fixation stability values on MP-1 and structural macular features on spectral domain optical coherence tomography. Optical coherence tomography angiography (OCTA) was utilized to assess microvascular characteristics at the final visit. Only patients with a successful MH closure were enrolled, excluding eyes affected by other relevant pathologies. Mean BCVA improvement was significant after surgery (t test, p value

Purpose: To compare immediate versus delayed introduction of immunosuppressives for naive noninfectious inflammatory choroidal neovascularization (iCNV).Methods: a retrospective, consecutive, compa...

Background/objectives: To compare the number of eye surgical procedures performed in Italy in the 2 months following the beginning of lockdown (study period) because of COVID-19 epidemic with those performed in the two earlier months of the same year (intra-year control) and in the period of 2019 corresponding to the lockdown (inter-year control). Methods: Retrospective analysis of surgical procedures carried out at 39 Academic hospitals. A distinction was made between elective and urgent procedures. Intravitreal injections were also considered. Percentages for all surgical procedures and incidence rate ratios (IRR) for rhegmatogenous retinal detachment (RRD) events were calculated. A p value Results: A total of 20,886 versus 55,259 and 56,640 patients underwent surgery during the lockdown versus intra-and inter-year control periods, respectively. During the lockdown, only 70% of patients for whom an operation/intravitreal injection was recommended, finally underwent surgery; the remaining patients did not attend because afraid of getting infected at the hospital (23%), taking public transportation (6.5%), or unavailable swabs (0.5%). Elective surgeries were reduced by 96.2% and 96.4%, urgent surgeries by 49.7% and 50.2%, and intravitreal injections by 48.5% and 48.6% in the lockdown period in comparison to intra-year and inter-year control periods, respectively. IRRs for RRDs during lockdown dropped significantly in comparison with intra- and inter-year control periods (CI: 0.65–0.80 and 0.61–0.75, respectively, p Conclusion: This study provides a quantitative analysis of the reduction of eye surgical procedures performed in Italy because of the COVID-19 epidemic.

Purpose: The purpose of this study is to report our experience with a case of punctate inner choroidopathy (PIC) reactivation following COVID-19. Case report: A 29-year-old caucasian woman with past ophthalmological history of bilateral PIC reported sudden visual acuity decrease in her right eye (RE) 3 weeks after SARS-CoV-2 infection. Her best-corrected visual acuity (BCVA) was 20/32 in RE; fundus examination and multimodal imaging (including indocyanine-green angiography, fundus autofluorescence, and optical coherence tomography) was consistent with unilateral PIC reactivation. The active choroidal lesions responded to high-dose corticosteroids, with functional improvement. Conclusion: Sars-CoV-2 infection could induce autoimmune and autoinflammatory dysregulation in genetically predisposed subjects. We report a case of PIC reactivation following COVID-19.

Purpose: To report our experience with a peculiar case of asynchronous bilateral retinal vascular occlusion in a patient suffering from membranoproliferative glomerulonephritis. Case report: A 57-year-old dialysed male affected by membranoproliferative glomerulonephritis who underwent kidney transplantation complained of a sudden vision loss in his right eye (RE). His best-corrected visual acuity (BCVA) was 20/40 in RE and 20/20 in the left eye (LE); ophthalmological and fluorangiographic examinations revealed unilateral retinal obliterative vasculitis with panuveitis and apparent sparing of contralateral eye. About 6 months later the patient developed a branch retinal vein occlusion associated with a papillary neovascular membrane in LE. Corticosteroid therapy was administered and immunosuppressant dosage was increased with macular oedema reduction in both events. Conclusion: We report a case of unilateral retinal obliterative vasculitis and subsequent contralateral retinal neovascularization and branch retinal vein occlusion in a patient affected by membranoproliferative glomerulonephritis.

Aging, chronic oxidative stress, and inflammation are major pathogenic factors in the development and progression of age-related macular degeneration (AMD) with the loss of retinal pigment epithelium (RPE). The human RPE contains a subpopulation of progenitors (i.e., RPE stem cells—RPESCs) whose role in the RPE homeostasis is under investigation. We evaluated the paracrine effects of mature RPE cells exposed to oxidative stress (H2O2) on RPESCs behavior through co-cultural, morphofunctional, and bioinformatic approaches. RPESCs showed a decline in proliferation, an increase of the senescence-associated β-galactosidase activity, the acquisition of a senescent-like secretory phenotype (SASP), and the reduction of their stemness and differentiation competencies. IL-6 and Superoxide Dismutase 2 (SOD2) seem to be key molecules in RPESCs response to oxidative stress. Our results get insight into stress-induced senescent-associated molecular mechanisms implicated in AMD pathogenesis. The presence of chronic oxidative stress in the microenvironment reduces the RPESCs abilities, inducing and/or maintaining a pro-inflammatory retinal milieu that in turn could affect AMD onset and progression.

The EX-PRESS glaucoma filtration device appears to be an effective addition to our options to treat refractory glaucoma. The possibility to create a sclerostomy without tissue excision provides a safe and reliable outflow pathway for aqueous that is standard in size, reducing much of the variability associated with a surgical procedure. Prospective randomized studies comparing EX-PRESS implantation with trabeculectomy show encouraging results. However, complications usually encountered in filtration surgery have been reported, and EX-PRESS implantation can also lead to specific device-related complications. This article reviews the most common complications associated with this procedure.

Uveitis is an important cause of blindness worldwide. Its reported incidence in the United States ranges from 17 per 100,000 to 24 per 100,000 people per year, and its prevalence ranges from 38 per 100,000 to 204 per 100,000 people per year. Uveitis may be associated with autoimmune inflammatory or infectious conditions. Some of the severe sequela that are associated with chronic uveitides include retinal and ciliary body (CB) detachment with consequent ocular hypotony and phthisis bulbi. In the principle of ultrasonography (USG), the depth of the tissue structures is determined by directly measuring the time delay of the returning ultrasound signal. Most ophthalmologists are familiar with B-scan USG techniques; however, the employment of high frequency ultrasound bio-microscopy (UBM), which is based on high frequency transducers incorporated into a B-mode clinical scanner, allows for the achievement of high-resolution images, useful in distinguishing subtle changes in the anatomy and pathology of structures like the retroiridal face, CB, and pars plana (PP), which are not precisely accessible by routine USG. This technology has a tissue depth penetration of approximately 3–6 mm and can visualize structures through opaque media, unlike optical coherence tomography (OCT). The addition of UBM in the armamentarium of diagnostic methods in the evaluation of uveitis patients, has yielded the level of clinical information considerably towards a definitive diagnosis and therapeutic efficiency. Inflammatory involvement of the iris, ciliary sulcus, CB and PP are among the main conditions in which the employment of UBM is highly recommended.

Regenerative medicine approaches based on mesenchymal stem cells (MSCs) are being investigated to treat several aging-associated diseases, including age-related macular degeneration (AMD). Loss of retinal pigment epithelium (RPE) cells occurs early in AMD, and their transplant has the potential to slow disease progression. The human RPE contains a subpopulation of cells - adult RPE stem cells (RPESCs) – that are capable of self-renewal and of differentiating into RPE cells in vitro. However, age-related MSC changes involve loss of function and acquisition of a senescence-associated secretory phenotype (SASP), which can contribute to the maintenance of a chronic state of low-grade inflammation in tissues and organs. In a previous study we isolated, characterized, and differentiated RPESCs. Here, we induced replicative senescence in RPESCs and tested their acquisition of the senescence phenotype and the SASP as well as the differentiation ability of young and senescent RPESCs. Senescent RPESCs showed a significantly reduced proliferation ability, high senescence-associated β-galactosidase activity, and SASP acquisition. RPE-specific genes were downregulated and p21 and p53 protein expression was upregulated. These findings document the effects of senescence and SASP acquisition on RPESC differentiation ability and highlight the need for a greater understanding of their role in AMD pathogenesis.

Background: Non-infectious uveitis represents a leading cause of visual impairment, and inflammation control represents a major priority in tackling visual acuity loss due to complications such as macular edema, different immunomodulatory drugs are currently being used, including anti-TNF-alpha Adalimumab. Methods: This was a monocentric observational study of 18 eyes of 18 patients with non-infectious uveitis treated with Adalimumab. The primary endpoint was the control of ocular inflammation. The secondary endpoints included the study of macular and choroidal thickness and architecture, visual acuity, changes in other treatments, and adverse effects. Results: Ocular inflammation was controlled at 12 months for 83.3% of patients. Central macular thickness improved from a median of 229.75 &micro, m at baseline to 213 &micro, m at 12 months, while choroidal thickness decreased by 11.54% at the end of the follow-up. A reduction of vasculitis on fluorescein angiography and of hyperreflective spots on optical coherence tomography was noted. Visual acuity also improved from 0.51 (logMAR) before treatment to 0.24 at more than 12 months (p = 0.01). A total of 11.1% of patients experienced side effects. Conclusion: Our study confirms the efficacy of adalimumab for the control of ocular inflammation, visual acuity preservation, and for corticosteroid sparing.

Purpose: To investigate papillary microvascular changes in patients affected by macular edema due to Central Retinal Vein Occlusions (CRVO) after anti-Vascular Endothelial Growth Factor (VEGF) therapy. Methods: Prospective analysis of papillary and peripapillary vessel density (VD) changes in 18 eyes of 18 hypertensive patients affected by CRVO before and after the loading-phase of intravitreal Ranibizumab (IVR) injections. Data were quantitatively measured by optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) before as well as 1 month and 4 months after injections. The correlation between post-treatment best-corrected visual acuity (BCVA) and changes in the retinal microvasculature evaluated by OCTA was assessed. Results: 18 eyes of 18 consecutive patients with a known history of arterial hypertension and affected by an acute CRVO episode were enrolled. Central macular thickness (CMT) was significantly reduced after IVR injections (p &lt, 0.001), while mean BCVA improved from 0.70 &plusmn, 0.26 logarithm of the minimal angle of resolution (logMAR) units at baseline to 0.25 &plusmn, 0.18 logMAR units after 4 months (p &lt, 0.001). VD inside disc and peripapillary significantly increased (p &lt, 0.001 and p = 0.01, respectively) after treatment. Conclusions: OCTA showed VD increase in the papillary area in patients affected by CRVO after anti-VEGF therapy. This area could represent a new region of interest to study microvasculature changes concomitant with severe macular edema.

Background Disease activity evaluation is mandatory according to the OMERACT Core Set of Domains for Behcet’s Syndrome (BS). Poor data are available on Patient (PtGA) and physician (PGA) global assessment of disease activity in BS. Objectives To assess PtGA performance in patients with BS and how different disease manifestations influence the patient and physician’s perception of disease activity. Methods A multicenter cross-sectional cohort of consecutive BS patients was enrolled. Disease activity was evaluated by PtGA, PGA and the Behcet’s disease current activity form (BDCAF). PtGA and PGA were assessed trough a single question (“How active was BS during the last week?”) in a 10-cm visual analogic scale. Health related quality of life (HRQoL) perception was evaluated by the Physical Component Summary (PCS) and the Mental Component summary (MCS) of the SF36v2 questionnaire. Correlation between PtGA, PGA, BDCAF and between them and SF36v2 component summaries was assessed trough the Spearman’s correlation coefficient (rho). Two separated multiple regression models were built to measure the independent effect (β regression) of each active clinical manifestation on PtGA and PGA. Results Overall, 226 BS patients from 5 Mediterranean Countries were enrolled. Out of them, 111 (49.1%) were males and the median (IQR) age and disease duration were 46.9 (35.6-55.2) and 11.7 (5.9-20.8) years, respectively. The median (IQR) value of PtGA, PGA and BDCAF were 2.0 (0.3-5.0), 1.0 (0.0-3.0) and 3.0 (0.0-5.0), respectively. PtGA significantly correlated (Figure) with both PGA (rho 0.759. p 0.001) and MCS (rho -451. p > 0.001). Conclusion PtGA seems to be a valid outcome measure in BS and should be considered as a separate outcome measure or better be included in composite index for overall disease activity evaluation. Disclosure of Interests Alberto Floris: None declared, Matteo Piga: None declared, Gerard Espinosa: None declared, Nikolaos Kougkas: None declared, Andrea Lo Monaco: None declared, Giuseppe Lopalco Speakers bureau: SOBI, BMS, Ida Orlando: None declared, Vittorio Pirani: None declared, Ernestina Santos: None declared, Luisa Serpa Pinto: None declared, George Bertsias: None declared, Luca Cantarini: None declared, Alberto Cauli: None declared, Ricard Cervera: None declared, Joao Correia: None declared, Marcello Govoni: None declared, Florenzo Iannone Consultant for: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Speakers bureau: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Ana Martins da Silva: None declared, Piergiorgio Neri: None declared, Carlos Vasconcelos: None declared, Alessandro Mathieu: None declared

Background Irreversible organ damage is considered a core outcome by the OMERACT working group. However, no specific tools are currently available to detect and measure damage accrual in Behcet’s syndrome (BS). Objectives To develop and preliminarily validate the Behcet’s syndrome Overall Damage Index (BODI). NCT03803462. Methods A preliminary version of the instrument (p-BODI) was developed by reviewing pre-existing tools [e.g. Vasculitis damage index (VDI)] and through an extensive literature review. p-BODI was then reviewed and implemented by a multi-rounds Delphi process, involving an international and multidisciplinary (5 rheumatologists, 4 internist, 1 ophthalmologist, 1 neurologist) panel of experts in BS management and a patients’ delegate. A group of clinicians (CG), not involved in the BODI development, was asked to independently score a set of clinical vignettes, in order to test the instrument reliability, after a training process consisting of a user manual and a video-tutorial. Then, Cohen’s K and Intra-class correlation coefficient (ICC) between assessors and gold standard were calculated. Afterwards, BODI validation was conducted according to the OMERACT Filter 2.0 in a multicenter BS cohort. Results Starting from a list of 120 candidate items, the final version of BODI consisted of 4 overarching principles, 30 items and 12 sub-items (each of them scores one point) grouped in 8 domains (figure). In terms of reliability, the mean K coefficient was 0.84 (95%CI 0.78 to 0.90) and the ICC was 0.88 (95%CI 0.80-0.95). Validation cohort consisted of 228 BS patients (49.1% males), with a median (IQR) age and disease duration of 46.9 (35.5-55.0) and 11.7 (5.8-20.7) years, respectively. Overall, prevalence of any BODI damage (BODI ≥1) was 56.1% with a median score of 1.0 (0-2.0). In regard of construct validity, BODI score significantly correlated with VDI (Spearman’s rho 0.693, p Full agreement among the CG was reached in judging BODI as a credible, comprehensive, easy to use, timesaving and acceptable instrument. Conclusion BODI is the first tool specifically developed to assess damage in BS. Preliminary data encourage further validation of BODI in more extended and multi-ethnic BS cohorts before being applied in clinical practice and as a therapeutic outcome. Disclosure of Interests Matteo Piga: None declared, Alberto Floris: None declared, Gerard Espinosa: None declared, Nikolaos Kougkas: None declared, Andrea Lo Monaco: None declared, Giuseppe Lopalco Speakers bureau: SOBI, BMS, Ida Orlando: None declared, Vittorio Pirani: None declared, Ernestina Santos: None declared, Luisa Serpa Pinto: None declared, George Bertsias: None declared, Luca Cantarini: None declared, Alberto Cauli: None declared, Ricard Cervera: None declared, Joao Correia: None declared, Marcello Govoni: None declared, Florenzo Iannone Consultant for: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Speakers bureau: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Ana Martins da Silva: None declared, Piergiorgio Neri: None declared, Carlos Vasconcelos: None declared, Monica Muntoni: None declared, Alessandro Mathieu: None declared

Non-infectious uveitis can be a potentially sight threatening disease. Very recently, therapeutic strategies have turned towards a new methodology, which includes biologic agents. The introduction of biologic drugs has started a Copernican revolution in ophthalmology: biologic therapies represent a revolutionary option for those patients who present non-responder, sight threatening uveitis. The availability of these therapies has improved the uveitis outcome. The present review shows the most relevant medical literature on biologic agents in ophthalmology, such as tumor necrosis factor blockers, anti-interleukins and other related biologics. Several papers reported the efficacy of biologic agents in a large number of refractory uveitides, which suggest a promising role of biologic drugs for selected cases. On the other hand, the medical literature does not have consistent numbers yet, which hopefully will validate the promising preliminary results. Biologic agents are not only promising drugs for the treatment of nonresponder uveitis, but also they show an apparently favourable safety profile, although several topics remain unsolved: it is still not clear when commencing the treatment, which agent to choose, and the length of biologic therapy. Moreover, the high costs and the still not clear safety profile have very often limited their use only for severe, non-responder uveitis in highly specialized uveitis centres.

Dome-shaped macula (DSM) is characterized by an inward convexity of the macula in myopic eyes, as visualized on optical coherence tomography (OCT). Serous retinal detachment (SRD) is a common complication of DSM, leading to a deterioration of visual acuity, in association with retinal pigment epithelium (RPE) atrophic changes. Many different therapeutic approaches to SRD secondary to DSM have been attempted, with variable results. We report our experience (11 treatment-naïve patients) with a combined new treatment approach, using half-fluence and half-dose photodynamic therapy (PDT) followed by subthreshold 577 nm micropulse laser treatment (STLT), for SRD related to DSM after a 6-month follow-up. Central foveal thickness was significantly decreased at the end of the follow-up. SRD diminished in all cases, and 5 eyes (45.4 %) registered complete resolution at the end of the 6-month follow-up, while 6 eyes (54.5 %) experienced two lines of improvement in BCVA at the end of the follow-up. No case showed postoperative complications. Therefore, this represents the first study evaluating the visual and anatomical outcomes of a safe and effective combined treatment of SRD in DSM.

The authors report a multimodal imaging analysis of a case of acute syphilitic posterior placoid chorioretinitis (ASPPC) occurring in a 51-year-old man. Best-corrected visual acuity (BCVA) was 0.5 and 0.8 in the right and left eyes, respectively. The authors performed spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, and indocyanine green angiography. All of the examinations were suggestive of the diagnosis of ASPPC, a rare manifestation of syphilis that has distinctive anatomical characteristics that are detectable early on with multimodal imaging. Moreover, serological tests were positive for syphilis infection, so the patient received intravenous penicillin G for 14 days. Final BCVA was 1.0 in the right eye and 0.9 in the left eye. [ Ophthalmic Surg Lasers Imaging Retina . 2019;50:e179–e184.]

Age-related macular degeneration (AMD) is still referred to as the leading cause of severe and irreversible visual loss world-wide. Advances in medical research have identified vascular endothelial growth factor (VEGF) as an important pathophysiological player in neovascular AMD and intraocular inhibition of VEGF as one of the most efficient therapies. Anti-VEGFs currently used to treat AMD included a monoclonal antibody (bevacizumab), an antibody fragments (ranibizumab), a fusion protein (aflibercept), and an aptamer (pegaptanib). The wide introduction of anti-VEGF therapy has led to an improvement in the prognosis of patients affected by AMD, with a consequent effects on the burden of care due to highly priced drugs, increasing patient numbers, and long-term disease chronicity. Aim of this review is to present an overview of available therapeutic strategies in AMD in term of clinical efficacy and economic sustainability.

PURPOSE To compare the changes in postoperative peripapillary retinal nerve fiber layer (p-RNFL) thickness after vitrectomy for epiretinal membrane in eyes with preexisting posterior vitreous detachment (PVD) and eyes with surgically induced PVD. METHODS This study included consecutive patients who underwent 25-gauge vitrectomy for epiretinal membrane. Eyes were divided, according to intraoperative PVD status, into a preexisting PVD group and surgically induced PVD group. Best-corrected visual acuity, p-RNFL thickness, and central retinal thickness were performed before and at 1, 3, and 6 months after surgery. RESULTS One hundred and twenty eyes of 120 patients were enrolled: 64 eyes in the preexisting PVD group and 56 eyes in the surgically induced PVD group. In the preexisting PVD group at 6 months, the mean global p-RNFL thickness did not change, whereas it was reduced in the temporal sector (P = 0.034). In the surgically induced PVD group at 6 months, significant decreases were observed in global p-RNFL thickness (P = 0.027), temporal (P = 0.021), temporal inferior (P = 0.030), and nasal inferior sectors (P = 0.010). At 6 months, the two groups differed significantly in temporal (P < 0.001) and temporal inferior sectors (P = 0.004). The preoperative mean best-corrected visual acuity improved significantly at 6 months in both groups. CONCLUSION Postoperative p-RNFL thickness after vitrectomy for epiretinal membrane tended to decrease in the temporal sector in all eyes and in the temporal inferior and nasal inferior sectors in eyes with surgically induced PVD.

To report a case of bilateral acute macular neuroretinopathy (AMN) occurring in a 32-year-old woman, analyzed using the multimodal imaging technique. A 32-year-old Caucasian woman presented with 20 days history of acute onset of blurred vision in the right eye. The best-corrected visual acuity (BCVA) was 0.8 and 1.0 in the right and left eyes, respectively. She reported a lower urinary tract infection associated with fever, 7 days before the onset of the ocular symptoms. Serological tests demonstrated the presence of IgM specific for cytomegalovirus (CMV), while all the other laboratory tests were negative. SD-OCT exhibited the disruption of the inner segment–outer segment junction, associated with hyper-reflectivity of a thickened outer plexiform layer overlying such area associated with thinning of the outer nuclear layer. The patient was diagnosed with AMN and received a corticosteroid treatment. During all the follow-up, OCT features did not change, although BCVA improved. Four months after the first visit, we found also in the left eye a subfoveal IS/OS disruption but differently from the right eye, in which the abnormalities persisted during all the follow-up visits, in the left one they disappeared only after a month. The IgM specific for the CMV remained positive during the whole follow-up. To our knowledge, this is the first patient reported with a diagnosis of AMN associated with persisting presence of IgM specific for CMV.

Idiopathic retinal vasculitis aneurysms and neuroretinitis (IRVAN) is the acronym used to describe a rare syndrome characterised by a peripheral retinal vascular occlusion secondary to a retinal vasculitis with multiple posterior retinal arterial aneurysms (Fig. 88.1). In 1995, Chang et al. [3] proposed “IRVAN”, describing ten cases with these features. This disorder is also known as “bilateral neuroretinopathy with multiple retinal arterial aneurysms”.

Inflammatory choriocapillaropathies (ICCP) is the name given to a group of diseases affecting the choroicapillaris. Primary inflammation of the choriocapillaris was suspected long before indocyanine green angiography (ICGA) became routinely available. In fact, fluorescein angiography (FA) is not tool capable to detect choroidal ischemia and does not give imaging access to the rest of the choroid. One of the main characteristics described in such group of diseases is represented by their eclectic attitude. The reason why the disease behaviours are so different from one condition to the other is still unknown. Among this group it is recognized a sub-group of diseases known as multifocal choroiditis. When seen by the clinician most of the cases already show chorioretinal scars, as if preceding silent episodes occur before the disease becomes symptomatic at the time of a recurrence. Multifocal choroiditis occurs in the same age groups as other ICCPs, namely in young to middle aged adults with women being predominantly affected. Lesions tend to leave scars and are not spontaneously reversible but seem to respond corticosteroid therapy. Subretinal neovascular membranes occur in one third of patients.

The clinical assessment of most of vitreo-retinal (VR) inflammations is often challenging: the clinical picture of such diseases can be overlapping and, despite the advances in medical technologies, the diagnostic tools might not provide data which can be conclusive. At this point, the possibility to take a vitreous tap via pars plana can help the ocular immunologist to differentiate between different sub-sets of infectious diseases and masquerade syndrome. The term "Masquerade Syndrome" was first used in 1967 to describe a case of conjunctival carcinoma that manifested as chronic conjunctivitis. Masquerade syndromes are disorders that occur with intraocular inflammation and are often misdiagnosed as a chronic idiopathic uveitis. Very often mesquerade syndrome is a synonymous of intraocular lymphoma which can present insidious clinical pictures, which are very often confused. The possibility to test the interleukines' ratio and to analyze the cytology represents a great scientific advance. A clearer role of VR surgery is played for all the complications of uveitis, such as uveitic retinal detachment, inflammatory macular pucker and dense persistent vitritis. VR surgery can warrant an important contribution to the clinical outcome.

Inflammation of uveal tract can be divided into: anterior, intermediate, posterior, and panuveitis. Uveitis can be a sight-threatening disease. The commonest ocular symptoms are: blurred vision, ocular pain, photophobia and floaters, depending on the type of uveitis. The onset of uveitis can be either acute or insidious, involving one or both eyes. Posterior uveitis is usually associated with vitritis. Anterior chamber cells and flare should be graded according to standardized uveitis nomenclature (SUN) working group. Binocular indirect ophthalmoscopy (BIO) score is used to grade vitreous involvement. Vitreous changes may comprehend: vitreous hemorrhage, vitreous strands, and vitreous traction. A further classification of posterior uveitis depends on the primary site of inflammation, which can identify: retinitis, choroiditis, retinochoroiditis, and chorioretinitis. Posterior pole uveal involvement can be: focal, multifocal, and placoid. Retinal vasculitis can be associated with several sub-types of posterior uveitis. Uveitis can present several complications such as, anterior and posterior synechiae, which can lead to uveitic glaucoma, cystoid macular oedema, retinal and choroidal neovascularizations, and retinal ischemia.

Inflammatory Choroidal Neovascularization (CNV) can be one of the most severe sequela in patients with uveitis. The outcome of subfoveal inflammatory CNV is poor if untreated: several procedures have been considered, even though there is lack of guidelines. On the other hand, the better knowledge of CNV pathophysiology may suggest a suitable treatment strategy. The combination of steroids and immunosuppression represents an important aspect of inflammatory CNV treatment. This ensures suitable control of inflammation as well as the reduction concomitant steroids dose. Neverthless there are cases which do not show a fully satisfactory response. Recently, the role of intravitreal anti-Vascular Endothelial Growth Factor (VEGF) has become primary in the treatment of neovascularizations. At this time, the combination of anti-VEGF drugs and immunesuppressives seems to be the best option for the management of inflammatory CNV. In summary, CNV secondary to uveitis is a severe sequela, which can lead to significant visual impairment. Although no guideline is provided, the current medical literature can give the basis for a successful treatment strategy, suggesting that combination of immunesuppresives and anti-VEGF is recommended.

Chronic non-infectious uveitis (NU) remains one of the most challenging problems in ophthalmology. Often, early and aggressive treatment is needed for a good visual acuity outcome. Local and oral corticosteroids remain the first line of treatment, even though side effects, such as glaucoma, cataract and Cushing Syndrome, can be serious and not tolerable. Therefore, a variety of immunosuppressive agents are used. Recently, new types of drugs, called “biologic agents”, are available. In the model of experimental autoimmuneuveitis (EAU) it has been demonstrated that tumor necrosis factor-alpha (TNF-α) may play a key role in uveitis. Beside the well known intravenous anti- TNF-α agent called infliximab, other sub cutaneous anti- TNF-α drugs have been introduced, such as Adalimumab and Golimumab. Adalimumab presents a stronger medical literature and it seems to present promising qualities. Few reports are available for Golimumab in uveitis at this time. Adalimumab can control uveitis unresponsive to the traditional immunosuppressive agents and, moreover, its subcutaneous administration makes the procedure user friendly. This drug can be used in selected cases as a rescue therapy for idiopathic non-infectious uveitis.

Purpose To present the clinical pattern of a complex case with atypical clinical features and controversial laboratory results. Methods Case report Results Infectious diseases represent always a challenge both for the clinical presentation and the management. The advent of new laboratory tests has lead to diagnosis of certain diseases which were undetectable in the recent past. These techniques have changed the way of treating several diseases, even though the gold standard still represents a chimera. Infact, it is not uncommon to face diseases which present themselves atypically and with laboratory tests which can be controversial. We want to present an anecdotal case that was defined a "killer case" in our tertiary referral centre. Conclusion The clinical presentation and the laboratory results of certain diseases can often be a tricky challenge even for highly specialized centres. The scientific "methodic doubt" is mandatory in cases which do not present typical clinical features.

Purpose To discuss the role of indocyanine green angiography (ICGA) in ophthalmology and the breaking news on this methodology. Methods The current literature is reviewed and the experience of a tertiary referral centre is reported. Results ICGA is an essential method used to explore the posterior pole in several sight-threatening diseases. Although this technique has been proven effective in detecting anomalies which were unappreciable with the traditional methods, there is still reluctancy in making use of such method. In the recent past months the topic of ICGA has again moved to the forefront of angiographic actuality with two editorial articles in largely diffused ophthalmological journals. The scientific dignity of ICGA is discussed and advocated by the western World, mostly by highly specialized ophthalmic centres in Europe. We will discuss the state of the art of the clinical use and the evidences in the medical literature of the ICGA. Conclusion ICGA is a validated method for the evaluation of the posterior pole, which can provide essential informations for the clinical assessment and management of several diseases. Albeit the validity of such technique has been proven, there is still some unjustified reluctancy in accepting its pivotal role in ophthalmology: the evidences suggest its use for every single disease that can affect the choroid and cannot be appreciated by the traditional tests.

Purpose To describe the treatment strategy in the management of peripheral ulcerative keratitis (PUK). Methods The current literature is reviewed and the experience of a tertiary referral centre is reported. Results Peripheral ulcerative keratitis (PUK) is rare but severe sight-threatening disease affecting the peripheral cornea. The causes of PUK are multiple and insidious. Infectious diseases, both secondary to systemic rather than purely local diseases, and non-infectious diseases can be identified as a cause of PUK. Non-infectious systemic diseases, which can induce deposition of immune complexes in the cornea and, hence, lead to corneal ulcers, include peripheral vasculitides and inflammatory diseases of collagen, such as Rheumathoid arthritis, Wegener Granulomatosis and Systemic Lupus Erythematosus. Mooren’s ulcer is one of the most characteristic PUKs: often unilateral and self-limiting in the elderly, sometimes bilateral and relentless in young patients, may cause severe visual impairment following extensive corneal destruction. Immunosuppressive therapy has been demonstrated effective in improving the prognosis of progressive cases, although a certain number of patients remain refractory to treatment. Resolution of refractory cases of Mooren’s ulcer with new systemic biologic agents, such as campath-1H and anti-tumor necrosis factor (TNF)-α, has been reported. Conclusion PUK can be a severe disease, leading to significant visual impairment. Although no guideline is provided, the current medical literature can give the basis for a successful treatment strategy. The detection of the infectious trigger can lead to the correct, specific therapy. Non-infectious diseases are basically treated with the combination of steroids with immunesuppresives and, when necessary, biologics.