Successful Medical and Surgical Management of Recalcitrant Acanthamoeba Keratitis, Scleritis, and Culture-Positive Scleral Abscess.

PURPOSE: The purpose of this study was to describe the management of a case of recurrent scleritis and Acanthamoeba -positive scleral abscess in a patient after the use of miltefosine for recalcitrant Acanthamoeba keratitis. METHODS: This is a case study. RESULTS: In this study, we report a case of advanced Acanthamoeba keratitis with resultant corneal perforation with therapeutic keratoplasty and associated scleritis who later developed a scleral abscess after treatment with oral miltefosine. The scleral abscess was positive for Acanthamoeba cysts and trophozoites, and after treatment for an additional several months, the patient had complete resolution of her disease. CONCLUSIONS: Acanthamoeba scleritis is a rare complication associated with Acanthamoeba keratitis. It has traditionally been treated as an immune reaction and associated inflammation, especially with the use of miltefosine. Management can require a multitude of different approaches, and in this situation, it has been demonstrated that scleritis can be infectious and that conservative management can be effective.

Systemic Vasculitis and Its Association with the Eye.

Vasculitis is a group of diseases characterized by the inflammation of the blood vessel walls. They are classified according to the size of the main vessel involved: large vessel, medium vessel, and small vessel vasculitis. Ophthalmic manifestations are quite common in most of these diseases. Episcleritis and scleritis are the most prevalent manifestation of vasculitis. However, there are certain ocular features characteristic of specific vasculitis entities. Given the severity and potential life-threat of these diseases, knowledge of the ocular manifestations is mandatory for the ophthalmologists.

Diagnosis and Treatment of Central Serous Chorioretinopathy in Patients with Scleritis.

Central serous chorioretinopathy (CSCR) is characterized by central neurosensory retinal detachment from the retinal pigment epithelium. While the association between CSCR and steroid use is widely recognized, it is difficult to distinguish whether the subretinal fluid (SRF) in ocular inflammatory disease results from steroid use or an inflammation-related uveal effusion. We report the case of a 40-year-old man who presented to our department with intermittent redness and dull pain in both eyes that had persisted for three months. He was diagnosed with scleritis with SRF in both eyes and steroid therapy was started. Inflammation improved with steroid use, but SRF increased. This indicated that the fluid was not caused by the posterior scleritis-related uveal effusion but by steroid use. SRF and clinical symptoms subsided after steroids were discontinued completely and immunomodulatory therapy was initiated. Our study highlights that steroid-associated CSCR must be considered in the differential diagnosis of patients with scleritis, and prompt diagnosis with an immediate shift from steroids to immunomodulatory therapy can resolve SRF and clinical symptoms.

Network Approaches to Uncover Pathogenesis and Therapeutic Targets of Inflammatory Bowel Diseases.

Inflammatory bowel diseases (IBD) are currently recognized to involve chronic intestinal inflammation in genetically susceptible individuals. Patients with IBD mainly develop gastrointestinal inflammation, but it is sometimes accompanied by extraintestinal manifestations such as arthritis, erythema nodosum, episcleritis, pyoderma gangrenosum, uveitis, and primary sclerosing cholangitis. These clinical aspects imply the importance of interorgan networks in IBD. In the gastrointestinal tract, immune cells are influenced by multiple local environmental factors including microbiota, dietary environment, and intercellular networks, which further alter molecular networks in immune cells. Therefore, deciphering networks at interorgan, intercellular, and intracellular levels should help to obtain a comprehensive understanding of IBD. This review focuses on the intestinal immune system, which governs the physiological and pathological functions of the digestive system in harmony with the other organs.

Predictors of glaucoma in patients with uveitis and scleritis.

BACKGROUND: To examine risk factors for development of glaucoma in a large cohort of subjects with uveitis and scleritis. METHODS: Retrospective review of subjects diagnosed with uveitis or scleritis between 2006 and 2019 at Auckland District Health Board. Subjects were excluded if they had glaucoma due to another cause. Main outcome measure was development of glaucoma. Data for local steroid use was not available. RESULTS: 3462 eyes of 2414 subjects were included in the study. Mean follow-up was 5.7 years (total follow-up time 19,897 eye years). Median age was 44.3 years and 1189 (49.3%) were female. Glaucoma developed in 222 eyes (6.3%) during the follow-up. Five-year cumulative risk of glaucoma was 6.2% (CI 5.0-7.5%) for anterior uveitis, 5.4% (CI 3.2-9.0%) for intermediate uveitis, 1.6% (CI 0.4-6.7%) for posterior uveitis, 8.7% (CI 6.5-11.7%) for panuveitis, and 3.2% (CI 1.0-9.5%) for scleritis. Five-year cumulative risk of glaucoma was lowest in HLA-B27 uveitis at 0.9% (CI 0.4-2.1%) and highest in viral uveitis 15.1% (CI 10.1-22.3%), sarcoidosis 9.9% (CI 6.1-15.9%) and tuberculosis 9.7% (CI 5.4-17.0%). On multivariate analysis, risk factors for development of glaucoma were older age at presentation, higher presenting intraocular pressure, chronic inflammation, and cystoid macular oedema. CONCLUSIONS: Glaucoma is a common complication of uveitis and scleritis and was more frequent in older subjects, high presenting IOP, chronic inflammation and those with cystoid macular oedema. Local steroid therapy contributes to this, but is not quantifiable in this study. Targeted screening is required to avoid irreversible progression of glaucomatous optic neuropathy.

Clinical Profile of Scleritis Presenting for the First Time in the Elderly.

PURPOSE: To analyze clinical characteristics of scleritis in elderly patients and also compare the data with relatively younger patients with scleritis (<60 years). METHOD: Retrospective analysis of medical records of patients with scleritis who visited a tertiary eye care centre between 2008 and 2018. RESULT: Scleritis in ≥60 years accounted for 3% of the total scleritis cases. The mean age of the patients was 67 ± 6 years, and a female (66%) predominance was noted. Of the 44 elderly patients with scleritis, 48% were 66-70 years of age and 20% were above 70 years of age. Diffuse scleritis was the most common subtypes followed by necrotizing scleritis (35%). Overall, 32% of elderly patients with scleritis had underlying disease and the most common systemic association was granulomatous with polyangiitis. When compared with a subset of patients (<60 years of age), the elderly group showed higher rate of recurrences and complications. CONCLUSION: Scleritis in elderly patients is relatively rare but can have a higher recurrence rate with a increased complications.

Posterior scleritis with choroidal detachments and periaortitis associated with IgG4-related disease: A case report.

BACKGROUND: IgG4-related diseases (IgG4-RDs) are known to disrupt the functioning of multiple organs and are usually associated with mass lesions. Periaortitis, an inflammation of the adventitia and tissues surrounding the aorta, is an example of an IgG4-RD. In ophthalmology, an enlargement of the lacrimal gland is a well-known IgG4-RD, and scleritis has also been reported to be an IgG4-RD although it is rare. We report our findings in a case with periaortitis and posterior scleritis that were present at the same time, and they responded well to systemic steroid therapy. PATIENTS CONCERNS: A 79-year-old man with dementia and Lewy bodies was referred to our hospital because of uveitis in both eyes that did not respond to topical steroid therapy. DIAGNOSIS: We found anterior scleritis in the right eye and uveitis with shallow anterior chambers in both eyes. B-mode echography showed choroidal detachments (CDs) and a T sign in the right eye. The CDs were assumed to have progressed to the posterior scleritis which then caused the severe vision reduction. The patient was referred to the Internal Medicine Department because the systemic inflammatory disease was suspected due to the high levels of C-reactive protein (CRP) and the fast erythrocyte sedimentation rate. Systemic CT scans showed periaortitis only at the lumbar region. Because of the high levels of IgG4, the patient was diagnosed with IgG4-RD. INTERVENTIONS: The patient received intravenous and oral steroid therapy. The first 125 mg of methylprednisolone (mPSL) for 3 days was intravenous, after which it was switched to oral prednisolone (PSL) therapy and the dosage was gradually reduced. OUTCOMES: The posterior scleritis and periaortitis responded well to the systemic steroid therapy. One year and a half after the onset of the disease, the patient is still taking 5 mg of PSL. CONCLUSIONS: Scleritis with multiple CDs and periaortitis were strongly suspected to be due to IgG4-RD although no definitive diagnosis was made by biopsy of the lesions. Clinicians should be aware that IgG4-RD should be considered as one of the causes of posterior scleritis.

Central retinal artery occlusion and subsequent amaurosis fugax in the contralateral eye associated with the G20210A prothrombin gene (F2) variant: a case report.

PURPOSE: Report the case of a patient with a history of central retinal artery occlusion in her right eye and amaurosis fugax associated with acute ischemic changes in her left eye related to a prothrombin G20210A gene variant, in which OCT-A was used as a diagnostic and therapeutic tool. CASE PRESENTATION: 55-year-old woman with a history of central retinal artery occlusion in her right eye and prothrombin gene G20210A (F2) variant diagnosis. She presented to our consultation with amaurosis fugax in her left eye. As medical history, she had an episode of bilateral posterior scleritis diagnosed asynchronously with the current episode. Vascular, autoimmune, and metabolic prothrombotic diseases were ruled out. OCT-A showed areas suggesting acute ischemia consistent with macular retinopathy in her left eye. Anticoagulant therapy with Apixaban was initiated, considering the risk for her vision. Control OCT-A showed perfusion improvement in the previous site of the occlusive vascular event. We also considered the extent of the inflammatory response due to posterior scleritis as a differential diagnosis. Nevertheless, it is less likely, considering the temporality between scleritis and the retinal-vascular episodes. CONCLUSIONS: While the G20210A prothrombin gene (F2) variant is a rare cause of retinal artery occlusion, it is important to consider it a differential diagnosis. Good visual outcomes can be achieved with prompt initiation of antithrombotic treatment. In addition, OCT-A is useful for diagnosing ischemic retinal changes that cannot be observed with other diagnostic methods and monitoring them.

Relentlessly Progressive Sweet Syndrome of the Eye with Scleritis and Choroidal Infiltration.

PURPOSE: To describe a case of Sweet syndrome, a dermatologic inflammatory disease, with progressive, unrelenting ocular findings. METHODS: Case report. RESULTS: A 73-year-old male was evaluated with a six-month history of Sweet syndrome, manifesting as cutaneous erythematous edematous papules on the dorsal arms and shins and confirmed with biopsy demonstrating neutrophil infiltration with nuclei fragmentation and lack of vasculitis. He initially noted a unilateral red eye with ocular pain and was found to have scleritis and choroidal infiltration. The patient's ocular disease progressed despite treatment with systemic corticosteroids, intraocular Ozurdex ®, systemic dapsone, and subtenons triamcinolone. Systemic evaluation was negative for malignancy or other inflammatory syndromes. Following 7 months of non-manageable ocular pain enucleation was offered to the patient, but he declined. CONCLUSION: Sweet syndrome, a dermatologic condition, can be associated with unilateral scleritis and choroidal infiltration that are relentlessly progressive despite maximal systemic and ocular corticosteroid therapy.

Development and Validation of a Model to Predict Anterior Segment Vision-Threatening Eye Disease Using Primary Care Clinical Notes.

PURPOSE: The purpose of this study was to develop a decision-support tool to predict anterior segment vision-threatening disease (asVTD) to aid primary care physicians (PCPs) with patient triage and referral. METHODS: The University of Michigan electronic health record data between January 1, 2016, and May 31, 2019, were obtained from patients presenting to a PCP with anterior eye symptoms and then saw an ophthalmologist within 30 days. asVTD included diagnosis of corneal ulcer, iridocyclitis, hyphema, anterior scleritis, or scleritis with corneal involvement by an ophthalmologist. Elastic net logistic regression with 10-fold cross-validation was used for prediction modeling of asVTD. Predictors evaluated included patient demographics and PCP notes processed using clinical natural language processing software (clinspacy). RESULTS: Two thousand nine hundred forty-two patients met the inclusion criteria, of which 133 patients (4.5%) had asVTD. The age was significantly lower among those with asVTD versus those without (median = 42 vs. 53 yrs, P < 0.001). Sex ( P = 0.8) and race ( P = 0.9) were not significantly different between groups. The final prediction model had an area under the curve of 0.72 (95% confidence interval 0.67-0.77). At a threshold achieving a sensitivity of 90%, the specificity was 30%, the positive predictive value was 5.8%, and the negative predictive value was 99%. CONCLUSIONS: The use of the prediction model increased the positive predictive value for asVTD compared with referral based on prevalence probabilities (17 patients vs. 22 patients needing to be evaluated to identify 1 case of asVTD). A prediction algorithm has potential to improve triage and initial management decision-making for PCPs because it performs better than probabilities in the absence of such a tool.

Epidemiology of keratitis/scleritis-related endophthalmitis in a university hospital in Thailand.

To demonstrate the demographics, associated factors, clinical presentations, microbiology, management, visual outcome and complications of keratitis/scleritis-related endophthalmitis (KSE). A retrospective chart of all endophthalmitis patients diagnosed between September 2001 and August 2011 was reviewed. Only endophthalmitis cases with previous corneal or scleral infection were included in the study. The patients were followed until losing vision or eyeball, becoming phthisis, or the end of 2018. Eighty-seven patients with KSE were identified, all unilateral. The mean age was 56.4 ± 21.4 years. There was a slight male predilection (55 patients, 63.2%). The mean follow-up time was 50 ± 149 weeks. The causative pathogens were identified in 35 patients (40.2%), with the highest frequency being bacteria. The most common bacterium was Pseudomonas aeruginosa (n = 13), and the most common fungus was Aspergillus sp. (n = 5). Fifteen patients achieved (17.2%) final visual acuity (VA) of hand motion or better after treatment. Eyeball removal was performed in 61 (70.1%) patients. From multivariate analysis, the only prognostic factor for poor final VA (worse than hand motion, HM) was poor VA (worse than HM) at the initial visit (relative risk 1.97, 95% confidence interval 1.15-3.36, p = 0.013). KSE is uncommon but has a devastating outcome. We found that the patient's initial VA was the only predictor for their final vision. P. aeruginosa was the most common identifiable organism in this study. However, several fungal infections were recognised. These findings should raise awareness for treatment of KSE in the tropics.

Cataract Surgery in the Setting of Scleritis.

Purpose: To report the clinical profile of cataract and its surgical management in a scleritis cohort from India.Methods: We conducted a retrospective review of medical records of 39 eyes of 32 consecutive patients with scleritis who underwent cataract surgery in a tertiary eye care institute.Results: The mean age at presentation was 50.9 ± 11.1 years and 65.6% of the patients were female. Five patients (15.6%) were ≤ 40 years of age. Necrotizing scleritis (56.4%) was the most common subtype of scleritis in this cohort followed by diffuse anterior scleritis (28.2%), nodular scleritis (12.8%). The preferred incision for phacoemulsification was clear corneal in 30 eyes (77%). In the immediate post-operative period, four eyes (10.2%) developed severe corneal edema, one eye had descemet membrane detachment. Anterior chamber reaction was observed in 18 eyes (46.1%). Fifteen eyes (38.4%) developed raised intraocular pressure and two required surgical intervention after 3 months of cataract surgery. Early posterior capsular opacification was observed in 5 eyes (12.8%) and cystoid macular edema in 2 eyes (5.1%).Conclusion: In addition to providing a distinct pattern of cataract, index study showed that long-term control of scleral inflammation prior to the cataract surgery remains the primary requisite for the successful outcome.

Incidence, prevalence, and risk factors of infectious uveitis and scleritis in the United States: A claims-based analysis.

BACKGROUND: Ocular inflammation causes significant visual morbidity in the United States, yet little is known about the epidemiology of infectious uveitis and scleritis. This study aims to evaluate the epidemiology of infectious uveitis/scleritis employing a large national medical claims database. METHODS: This was a retrospective, case-control study, employing Optum's de-identified Clinformatics® Data Mart Database, containing data from 21.5 million privately insured individuals with enrollment for at least 15 months within 2007-2015. Inclusion in the uveitis/scleritis sample required an index uveitis/scleritis diagnosis based on International Classification of Diseases, Ninth Revision (ICD-9) codes. Exclusion criteria included index date within 3 months after intraocular surgery. Rates for uveitis/scleritis were determined by anatomic site. Multivariable logistic regression analyses were performed to determine odds ratios for the incidence and prevalence of uveitis/scleritis by anatomic category. FINDINGS: Infectious etiologies accounted for less than 20% of uveitis/scleritis, with mean rates of 18.9 (incidence) and 60.6 (prevalence) per 100,000 persons. The mean prevalences of infectious anterior, intermediate, posterior, panuveitis, and scleritis were 27.7, 0.17, 23.4, 4.4, and 4.6, per 100,000, respectively. Overall risk of prevalent infectious uveitis/scleritis increased with age (OR>3.3 for each decade over age 18, p<0.01), female sex (OR = 1.2, p<0.01), non-Hispanic white race (OR<1 for all other races, p<0.01), as well as the East South Central census division (OR = 1.2, p<0.01), comprising Alabama, Kentucky, Missouri, and Tennessee. Medical comorbidities, including HIV infection (OR = 6.4, p<0.01) and rheumatologic disease (OR = 1.9, p<0.01), were common in the infectious uveitis/scleritis cohort. CONCLUSIONS: The incidence and prevalence of infectious uveitis/scleritis in the United States were higher than previously reported estimates but remained lower than in developing countries. Rates varied by age, sex, race, and medical comorbidities, and may reflect differential susceptibility to various infectious agents with disparate geographic distributions within the United States.