Scleritis and episcleritis are an overlapping spectrum of diseases and accurate diagnosis is of utmost importance as the treatment and prognosis are vastly different. Predominantly a clinical diagnosis, the challenge lies in those cases with equivocal clinical features. Furthermore, clinical grading of scleritis is subjective and hence is neither very reliable nor reproducible. Existing modalities such as slit lamp examination and clinical photographs in scleritis describe macroanatomy but do not provide details on the microanatomy of the sclera. A recent adjusted algorithm for anterior segment optical coherence tomography (AS-OCT) imaging has improved the ability of this device to aid in the diagnosis of all the major forms of scleritis. To highlight the role of AS-OCT in delineating various anatomical forms of episcleritis and scleritis and explore this tool in monitoring disease course and response to therapy. A comprehensive literature search was carried out in various medical databases using keywords AS-OCT and scleritis; AS-OCT and episcleritis; anterior segment imaging in scleritis; scleritis and episcleritis; Recent advances in anterior segment imaging. Original articles and novel reports describing the potential role of AS-OCT in the diagnosis and management of scleritis and episcleritis were included. After a thorough assessment, it was clear that published literature lacks guidelines for uniform interpretation and also for classification and follow-up in scleritis. We describe a uniform protocol for AS-OCT image acquisition, interpretation of images and list the advantages and limitations. AS-OCT can be used to localize the level of scleral inflammation thus helping in the diagnosis of scleral inflammatory disease. It can be a valuable tool in studying progression.

This article points out the design, methods, development and deployment of the international registry promoted by the AutoInflammatory Disease Alliance (AIDA) Network with the aim to define and assess paediatric and adult patients with immune-mediated scleritis. This registry collects both retrospective and prospective real-world data from patients with non-infectious scleritis through the Research Electronic Data Capture (REDCap) tool and aims to promote knowledge and real-life evidence from patients enrolled worldwide; the registry also allows the collection of standardised data, ensuring the highest levels of security and anonymity of patients' data and flexibility to change according to scientific acquisitions over time. The communication with other similar registries has been also ensured in order to pursue the sustainability of the project with respect to the adaptation of collected data to the most diverse research projects. Since the launch of the registry, 99 centres have been involved from 20 countries and four continents. Forty-eight of the centres have already obtained a formal approval from their local ethics committees. At present, the platform counts 259 users (95 principal investigators, 160 site investigators, 2 lead investigators, and 2 data managers); the platform collects baseline and follow-up data using 3683 fields organised into 13 instruments, including patient's demographics, history, symptoms, trigger or risk factors, therapies and healthcare utilization. The development of the AIDA International Registry for patients with non-infectious scleritis will allow solid research on this rare condition. Real-world evidence resulting from standardised real-life data will lead to the optimisation of routine clinical and therapeutic management, which are currently limited by the rarity of this ocular inflammatory condition.

Dengue fever is a severe mosquito-borne disease which can present with various severe ocular manifestations. We aim to report a case of infectious scleritis in dengue fever, occurring due to Methicillin-resistant Staphylococcus aureus (MRSA), managed with intravenous Teicoplanin. Case report. A 23-year-old woman with recent hospitalization for dengue fever presented with severe pain and a nodular scleral lesion. She was initially diagnosed with immune-mediated scleritis, but as the condition worsened, she underwent scleral scraping which subsequently grew MRSA. She was initially started on topical vancomycin eye drops and topical linezolid but there was further worsening. Therapy was switched to intravenous Teicoplanin which resulted in rapid resolution of the condition. To the best of our knowledge, this is the first reported case of infectious scleritis in association with dengue fever due to MRSA.

A 35-year old soft contact lens wearer with a proven bilateral Acanthamoeba keratitis developed a nodular scleritis. Based on the stepladder approach described by Iovieno et al. [Ophthalmology. 2014 Dec;121(12):2340-7], nonsteroidal anti-inflammatory drugs, methylprednisolone, and later azathioprine were added to the antiamoebic treatment. Unfortunately, there was further deterioration and an endophthalmitis developed. Unbearable pain and concerns of spread to the brain urged an enucleation. Histopathological examination confirmed Acanthamoeba cysts in the cornea, sclera, retina, choroid, and vitreous body. As a side effect of the immunosuppressive treatment, the patient developed myopathy, pulmonary aspergillosis, and an avascular necrosis of the hip. Scleritis is a devastating complication of Acanthamoeba keratitis with a poor prognosis and a high enucleation rate. Acanthamoeba sclerokeratitis is, due to cyst-free biopsies, mostly assigned to an immune-mediated mechanism, justifying the use of immunosuppressive treatment. Scleritis in our case contributed to the extracorneal spread of Acanthamoeba. Our case is the first documented extracorneal spread of Acanthamoeba without previous surgery. Extracorneal spread of Acanthamoeba should be considered, even in the case of false-negative biopsies. We strongly recommend serial sections of the retrieved scleral specimen in case of negative histopathological examination to exclude an infection. Even when an immune-mediated scleritis is suspected, systemic immunosuppressive treatment should always be used with the greatest caution. Awareness of the side effects and monitoring by an experienced physician is mandatory.

Scleritis is a rare painful ocular disorder, associated with severe ocular pain and tissue destruction. Although a majority of these cases are immune mediated and at least half of these are associated with systemic immune-mediated diseases, a smaller minority are due to infections of the sclera. The two conditions closely mimic each other, and a thorough knowledge of the subtle differences is necessary in order to reach a timely diagnosis. Diagnostic delay can lead to a poor outcome both due to the destruction caused by the uncontrolled infection and also due to propagation of the infection with the use of corticosteroids which may have been started for presumed immune mediated scleritis. In this review, we present the clinical features, etiological agents, and the differentiating features between immune and infectious scleritis. We also present diagnostic and management guidelines for managing scleral infection.