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    Follow-up a lungo termine nei pazienti con “dome-shaped macula” associata a distacco sieroso del neuroepitelio foveale trattati con la terapia fotodinamica

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    FOLLOW-UP A LUNGO TERMINE NEI PAZIENTI CON “DOME-SHAPED MACULA” ASSOCIATA A DISTACCO SIEROSO DEL NEUROEPITELIO FOVEALE TRATTATI CON LA TERAPIA FOTODINAMICA Ilir Arapi, MD Introduzione: Il distacco sieroso del neuroepitelio foveale (DSNF) nei pazienti con macula a conformazione cupoliforme (DSM) rappresenta la causa più frequente di riduzione dell’acuità visiva e invio presso le unità di cura oculistiche. Lo scopo di questo studio è quello di valutare i risultati a lungo termine del trattamento con la terapia fotodinamica (PDT) in una serie di pazienti miopi associati a DSM con DSNF. Materiali e Metodi: Lo studio è stato disegnato come serie retrospettiva interventistica. Sono state esaminate le cartelle cliniche di 18 pazienti consecutivi miopi (20 occhi) con DSM associata a DSNF e trattati con la PDT. I seguenti dati sono stati valutati: miglior acuità visiva corretta (BCVA), errore di rifrazione, le caratteristiche in fluorangiografia (FA) e angiografia al verde di indocianina (ICGA), la morfologia foveale tramite la tomografia a coerenza ottica (OCT) in modalità EDI. Un miglioramento o peggioramento della BCVA è stato definito come un aumento o diminuzione di 2 o più linee della BCVA, mentre gli occhi associati a risoluzione del fluido sotto la fovea dopo il trattamento sono stati considerati come rispondenti alla PDT. Risultati: Tutti gli occhi sono stati sottoposti a diversi trattamenti PDT con una mediana di 3 (1 °; 3 quartili 2; 3,75; intervallo: 1 - 7), mentre il follow-up mediano è stato di 22 mesi (1 °, 3 ° quartili 12; 40; intervallo: 4 - 55). Nell'ultimo follow-up 7 occhi (35%) hanno mostrato una completa risoluzione del DSNF e sono stati considerati rispondenti alla PDT. Nell’ultima visita del follow-up 5 occhi (25%) hanno mostrato un aumento della BCVA, 13 occhi (65%) hanno mantenuto una BCVA stabile, mentre 2 occhi (10%) hanno avuto una diminuzione della BCVA. L'analisi statistica ha mostrato che il miglioramento della BCVA era significativamente maggiore negli occhi rispondenti alla PDT (p = 0,027). L'area mediana di ipocianescenza maculare al baseline osservata durante i tempi tardivi dell’ICGA è risultata significativamente inferiore [2,6 mm² (1 °; 3 quartili 2.3, 2.8 mm², intervallo: 1,61-3,28)] nel gruppo di pazienti che hanno risposto alla PDT e hanno avuto un aumento di ≥2 linee Snellen della BCVA rispetto ai pazienti che sono stati considerati non rispondenti [8.1 mm² (1 °; 3 ° quartili 5.1; 10.2 mm²,intervallo: 4,50-14,26)] (p <0.001). Conclusioni: I nostri dati suggeriscono che gli occhi miopi affetti da DSM e associati a DSNF potrebbero reagire positivamente al trattamento PDT mostrando la risoluzione completa del distacco foveale e un aumento della BCVA se il quadro iniziale angiografico all’ICG presenta un’area limitata di ipocianescenza maculare

    Symptoms and signs of anterior uveitis

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    Purpose To review the symptoms and signs of anterior ueveitis (AU), based on the anatomic classification of uveitis, iritis and iridocyclitis.Methods Review of symptoms and signs of AU.Results Perikeratic injection, small keratic precipitates (KPs), cells and flare in the anterior chamber are peculiar findings of alternating unilateral acute non-granulomatous anterior uveitis, which is commonly described in association with HLA-B27 antigen and spondyloarthropaties. In such cases, hypopyon or fibrinous exudate can also occur. Patients presenting acute anterior uveitis typically show red eyes, photophobia, ocular pain, and often blurred vision. In chronic anterior uveitis, the onset is usually subtle and patients may be asymptomatic until the development of complications. Chronic flare, Koeppe and Busacca nodules of the iris, medium-size KPs or large mutton-fat KPs, peripheral anterior synechiae and broad-based posterior synechiae represent hallmarks of granulomatous anterior uveitis which tends to chronicity. Viral anterior uveitis is typically unilateral, characterized by recurrent episodes of anterior uveitis. Endotheliitis, high intraocular pressure, and patchy/sectoral iris atrophy are also present. Juvenile Idipoathic Arthritis (JIA)-associated anterior uveitis is peculiarly a bilateral non-granulomatous chronic anterior uveitis, frequently worsened by several complications.Conclusion The typology of AU influences its clinical presentation: the clinical findings can vary on the basis of its acute or chronic, granulomatous or non-granulomatous nature. Specific AU subtypes are characterized by a large number of distinct ocular signs

    OCT in uveitis

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    Purpose To describe the role of optical coherence tomography (OCT) in uveitis.Methods The current literature is reviewed and the experience of a tertiary referral centre is reported.Results The involvement of the posterior pole during an active eye inflammatory disease can entail a reduction of visual acuity, which is sometimes difficult to treat. Albeit fluorescein angiography (FA) is still the gold standard for the detection of blood-retinal barrier (BRB) disruption and has been the best option for the diagnosis of cystoid macular oedema (CMO), OCT represents an essential tool that can ameliorate the interpretation of FA findings. Epiretinal membrane (ERM) and tractional maculopathy may represent a sight-threatening sequela of uveitis. OCT is extremely useful in documenting pathologies at the vitreo-retinal interface, such as the formation of ERM, as well as it is also helpful in proving early infiltrates at the posterior vitreous interface. Although FA still remains the gold standard for the choroidal neovascularization (CNV) assessment, OCT is very helpful in documenting CNV nearby a chorioretinal scar. OCT has been used to study the type of CNV and to find its correlation to RPE.Conclusion OCT may demonstrate a variety of morphological changes, that may point towards a specific uveitic disease: different forms of macular oedema may be described in uveitis as well as OCT features which can be peculiarly found in several diseases. In addition, OCT can be very useful in documenting both ERM and CNV

    Complications of uveitis and their management

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    Purpose To review the uveitis complications and their management.Methods The current literature is reviewed and the experience of a tertiary referral centre is reported.Results Visual recovery in uveitis is hampered all too often by the onset of complications arising from the frequent, recurrent episodes of inflammation. The most frequent complications in uveitis are: cataract, cystoid macular edema (CMO), glaucoma, neovascularization, and vitreous opacities. Moreover, these sequela may progress, despite an apparent good control of the ocular inflammatory disease. An early recognition, as well as an appropriate and timely management are mandatory for a successful outcome. In addition, the treatment alternatives for these entities should be considered, depending on the severity of the condition.Conclusion Several sequela have been described in uveitis. A prompt recognition may orient correctly the ophthalmologist towards the best treatment option, lowering the risks of severe and irreversible ocular damages

    Inflammatory choroidal neovascularization: how combined anti-VEGF and inflammation suppressive therapy is going to become the treatment of choice

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    Purpose To describe the treatment strategy in the management of inflammatory choroidal neovascularization (CNV).Methods The current literature is reviewed and the experience of a tertiary referral centre is reported.Results 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 has become the recommended strategy for the management of inflammatory CNV.Conclusion 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

    PUK from diagnosis to treatment

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    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

    ICGA: why I think that it is still relevant today

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    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

    Biologic agents in inflammatory eye disease.

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    Non-infectious uveitis is a potentially sight threatening disease. Along the years, several therapeutic strategies have been proposed as a means to its treatment, including local and systemic steroids, immunosuppressives and more recently, biologic agents. The introduction of biologics can be defined as a new era: biologic therapies provide new options for patients with refractory and sight threatening inflammatory disorders. The availability of such novel treatment modalities has markedly improved the therapy of uveitis and considerably increased the possibility of long-term remissions. This article provides a review of current literature on biologic agents, such as tumor necrosis factor blockers, anti-interleukins and other related biologics, such as interferon alpha, for the treatment of uveitis. Several reports describe the efficacy of biologics in controlling a large number of refractory uveitides, suggesting a central role in managing ocular inflammatory diseases. However, there is still lack of randomized controlled trials to validate most of their applications. Biologics are promising drugs for the treatment of uveitis, showing a favorable safety and efficacy profile. On the other hand, lack of evidence from randomized controlled studies limits our understanding as to when commence treatment, which agent to choose, and how long to continue therapy. In addition, high cost and the potential for serious and unpredictable complications have very often limited their use in uveitis refractory to traditional immunosuppressive therapy

    Successful treatment of an overlapping choriocapillaritis between multifocal choroiditis and acute zonal occult outer retinopathy (AZOOR) with adalimumab (HumiraTM)

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    To report a case of overlapping choriocapillaritis that initially presented as multifocal choroiditis (MFC) but later showed features compatible with acute zonal occult outer retinopathy (AZOOR) resistant to standard immunosuppression that responded only to adalimumad therapy. A 41-year-old patient presented with multiple small, discrete yellow-whitish spots in both eyes, compatible with MFC. A few weeks later, despite treatment with sub-Tenon and systemic corticosteroids, a choroidal neovascularization occurred in the right eye. The patient was treated with intravitreal anti-vascular endothelial growth factor. After 2 months, reduced visual acuity, photopsia and visual field defect in the left eye occurred. Spectral domain optical coherence tomography revealed photoreceptor outer segment defects common to all choriocapillaritis. The additional finding of an annular scotoma and a 360° ring on indocyanine green angiography led us to make the diagnosis of presumed AZOOR. Despite the combination of several immunosuppressive agents leading to temporary control of the disease, the patient experienced a further worsening. At that point, adalimumab was introduced, which led to an obvious improvement. This case supports the hypothesis that two different entities of the so-called AZOOR complex can be possible in the same eye, even asynchronously. In our case, anti-tumor necrosis factor alpha monoclonal antibody therapy represented a valid treatment option in a patient unresponsive to traditional immunosuppressive treatments

    Latest diagnostic possibilities in unsolved uveitis, suspicious for malignancy

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    Vitreo Retinal Lymphomas (VRL) are rare malignancies that display a wide spectrum of clinical patterns. Almost all VRL are non-Hodgkin's lymphomas, B-cell sub-type. Since the clinical presentation of VRL might be confused with a non-responder eye inflammation, VRL are also known as ‘masquerade syndromes’. On the basis of the potential severity of such disease, vitreous biopsy might be indicated for all the cases which can be potential VRL. Since vitreous biopsy is not diagnostic in all cases, further techniques can provide further essential data. Immunohistochemistry can be used for identifying important Cluster of Differentiation (CD) such as CD45 for leukocytes, CD20, CD79a, PAX-5 for B-cells, CD45RO for T-cells, and CD68 for macrophages. Furthermore, clonality can be established by using antibodies targeting the kappa and lambda light chains. Polymerase chain reaction gene rearrangement studies can identify monoclonality of the heavy chain variable (V), diversity (D), and joining (J) immunoglobulin gene segments. Recently, measurement of Interleukin (IL)-6 and IL-10 in aqueous and/or vitreous fluid can lead to the correct diagnosis, even though an elevated IL-10/IL-6 ratio is not specific for VRL
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