Anterior Scleritis Manifesting After Coronavirus Disease 2019: A Report of Two Cases.

PURPOSE: The purpose of this study was to report 2 patients with anterior scleritis manifesting after coronavirus disease 2019 (COVID-19). METHODS: The patients with confirmed COVID-19 developed anterior scleritis after their systemic symptoms were markedly improved. A thorough systemic workup identified no underlying autoimmune diseases. Ocular characteristics and safety and efficacy of systemic immunosuppressive therapy were evaluated. RESULTS: Case 1 was a 67-year-old woman who presented with necrotizing anterior scleritis in both eyes 3 weeks after the onset of COVID-19. One-week treatment with topical betamethasone and oral prednisolone (65 mg daily) did not result in improvement, so she was started on intravenous cyclophosphamide and subcutaneous adalimumab in addition to oral prednisolone. Necrotizing scleritis was gradually improved over 3 months. Case 2 was a 33-year-old man who presented with sectoral anterior scleritis in his right eye 2 weeks after the onset of COVID-19. He was started on topical betamethasone and oral prednisolone (85 mg daily). One week later, all signs and symptoms disappeared, and topical and oral corticosteroids were gradually tapered off over 2 weeks. There was no recurrence of respiratory symptoms or active scleritis in any cases after discontinuation of treatment. CONCLUSIONS: These cases suggest that COVID-19 can be associated with anterior scleritis, which responds to immunosuppressive and biologic agents. Ophthalmologists should consider anterior scleritis in patients with COVID-19 who present with ocular pain and redness during the convalescent phase of the illness.

Nodular Necrotizing-Scleritis Associated with Epstein-Barr Virus Infection: A Case Report.

PURPOSE: To report a case of necrotizing scleritis associated with Epstein-Barr virus (EBV) infection. CASE REPORT: A 68-year-old woman with a history of scleral ulcer, perforation, and scleral graft, with initial negative studies for infectious processes and autoimmune systemic-diseases consulted our service for a second opinion after 2 years of treatment without improvement. She presented severe conjunctival and scleral hyperemia, vascular tortuosity in 360°, areas of scleral thinning and an elevated lesion of nodular appearance. EBV serologic antibody IgG titers were strongly positive and IgM titers were borderline. A scleral biopsy showed granulation tissue with severe inflammation without the presence of tumor cells; PCR was positive for EBV. The patient was treated with antiviral therapy with resolution of the scleritis. CONCLUSIONS: EBV although rare, should be considered as a differential diagnosis in patients with necrotizing scleritis with no improvement to conventional therapy.

Assessment of the Accuracy of Using ICD-9 Codes to Identify Uveitis, Herpes Zoster Ophthalmicus, Scleritis, and Episcleritis.

IMPORTANCE: With the increased use of data from electronic medical records for research, it is important to validate International Classification of Diseases, Ninth Revision (ICD-9) codes for their respective diagnoses. OBJECTIVE: To assess the accuracy of using ICD-9 codes to identify ocular inflammatory diseases. DESIGN, SETTING, AND PARTICIPANTS: Retrospective secondary database analysis. The setting was Kaiser Permanente Hawaii, an integrated managed care consortium that serves approximately 15% of the general Hawaiian population. Participants were patients with ICD-9 diagnosis codes that might be associated with a diagnosis of ocular inflammation seen at Kaiser Permanente Hawaii between January 1, 2006, and December 31, 2007. The data collection and analysis took place from January 2011 to August 2015. MAIN OUTCOMES AND MEASURES: The main outcome was the positive predictive value (PPV) of ICD-9 codes for identifying specific types of ocular inflammatory disease. The PPVs were calculated by determining the ratio of the confirmed cases found by medical record review to the total number of cases identified by ICD-9 code. RESULTS: Of the 873 patients identified by a comprehensive list of ICD-9 codes for ocular inflammatory diseases, 224 cases were confirmed as uveitis after medical record review. Using a set of uveitis-specific codes and eliminating patients with a history of ocular surgery, the overall PPV for uveitis was 61% (95% CI, 56%-66%). The PPVs for individual uveitis codes ranged from 0% to 100%, and 11 uveitis codes had a PPV exceeding 80%. Herpes zoster ophthalmicus and scleritis/episcleritis ICD-9 codes had PPVs of 91% (95% CI, 86%-95%) and 60% (95% CI, 54%-66%), respectively. CONCLUSIONS AND RELEVANCE: Our results suggest that using ICD-9 codes alone to capture uveitis and scleritis/episcleritis diagnoses is not sufficient in the Kaiser Permanente Hawaii healthcare system, although there were specific uveitis codes with high PPVs. However, the electronic medical record can reliably be used to identify herpes zoster ophthalmicus cases. Medical record review, as was done in this study, is recommended to elucidate diagnoses for uveitis and scleritis/episcleritis.

Necrotizing Scleritis, Conjunctivitis, and Other Pathologic Findings in the Left Eye and Brain of an Ebola Virus-Infected Rhesus Macaque (Macaca mulatta) With Apparent Recovery and a Delayed Time of Death.

A 3.5-year-old adult female rhesus macaque (Macaca mulatta) manifested swelling of the left upper eyelid and conjunctiva and a decline in clinical condition 18 days following intramuscular challenge with Ebola virus (EBOV; Kikwit-1995), after apparent clinical recovery. Histologic lesions with strong EBOV antigen staining were noted in the left eye (scleritis, conjunctivitis, and peri-optic neuritis), brain (choriomeningoencephalitis), stomach, proximal duodenum, and pancreas. Spleen, liver, and adrenal glands, common targets for acute infection, appeared histologically normal with no evidence of EBOV immunoreactivity. These findings may provide important insight for understanding sequelae seen in West African survivors of Ebola virus disease.

Conjunctival Sensation in Scleritis.

PURPOSE: To investigate conjunctival sensation in patients with scleritis. METHODS: Retrospective cross-sectional study of patients with scleritis. Conjunctival sensation was tested by Cochet-Bonnet aesthesiometer in four quadrants of the bulbar conjunctiva plus the area(s) of active or previously active inflammation; sensation was compared with the contralateral eye. RESULTS: Of 28 patients with scleritis, nine had active scleritis and 19 had inactive scleritis. Eleven patients had a systemic autoimmune condition and five had infectious scleritis. The mean conjunctival sensation score of areas of inactive scleritis was significantly less than that of corresponding normal areas in the contralateral eye (p<0.001). Among patients with presumed herpetic scleritis, conjunctival sensation was significantly lower in the affected eye than in the unaffected eye (p<0.001). CONCLUSIONS: Conjunctival sensation is decreased in areas of previously active inflammation from scleritis. Eyes with herpetic scleritis had reduced conjunctival sensation, even in areas without previous active inflammation.

[Scleritis, clinical features, etiologies and treatment: a case series of 32 patients]. / Sclérites, aspects cliniques, étiologiques et thérapeutiques : à propos d'une série de 32 observations.

PURPOSE: To report on the various clinical presentations, etiological diagnosis, prognosis and treatment of patients with scleritis evaluated at a tertiary care eye center. METHODS: Retrospective, monocentric study on a series of 32 patients in a tertiary center. RESULTS: The mean age of included patients with scleritis was 46.8 years (range, 22 to 77 years). Nineteen patients were women and 13 were men. Twenty-six patients (81%) had anterior scleritis (15 nodular, 8 diffuse and 3 necrotizing), six (19%) had posterior scleritis. Unilateral inflammation was present in 24 patients (75%). Twelve out of the 32 patients (37.5%) had an underlying systemic disease: granulomatosis with polyangiitis (n=3), Behçet's disease (n=2), unspecified inflammatory arthritis (n=2), psoriatic arthritis (n=1), ankylosing spondylitis (n=1), sarcoidosis (n=1), Cogan's syndrome (n=1) and ulcerative colitis (n=1). Six patients (18.8%) were suspected of having infectious disease with herpes virus: clinical context and positive treatment response with oral valacyclovir. Systemic agents and topical agents were required in 28 patients (87.5%). The first line therapy was mainly oral non-steroidal anti-inflammatory drugs in 15 patients (47%) and oral corticosteroids in 8 (25%). Immunosuppressive drugs were required in 6 patients. The mean follow-up was 16.3 months. Six patients (19%) had a decrease in visual acuity. CONCLUSION: The number of systemic disease in our series is similar to the main series in the literature. Treatment with valaciclovir might be effective in patients with suspected herpes simplex scleritis.

Unique presentation of 3rd and 6th cranial nerve palsies, nodular scleritis and nummular keratouveitis in an immunocompetent patient following an attack of herpes zoster ophthalmicus.

AIM: To report a unique presentation of 3rd and 6th cranial nerve palsies with nodular scleritis and nummular keratouveitis following an attack of herpes zoster ophthalmicus (HZO). METHODS: Case report. CASE REPORT AND RESULTS: A 56-year-old woman with a 1-month history of HZO presented with drooping of the right upper eyelid, diplopia, and pain around the right eye. She was noted to have right 3rd and 6th cranial nerve palsies. She developed nodular scleritis and nummular keratouveitis at 2 and 4 months follow-up, respectively, which were treated with antivirals and steroids. At 10 months follow-up, although the diplopia in right lateral gaze persisted, there was no recurrence of ocular inflammation with complete recovery of ptosis. CONCLUSIONS: A unique presentation of multiple cranial nerve palsies with nodular scleritis and nummular keratouveitis in an immunocompetent patient following an attack of HZO is highlighted in this report.

Clinical features and presentation of infectious scleritis from herpes viruses: a report of 35 cases.

PURPOSE: To describe clinical features and presentation of infectious scleritis resulting from herpes viruses. DESIGN: Retrospective case series. PARTICIPANTS: Thirty-five patients out of 500 with scleritis. METHODS: We reviewed the electronic health records of 500 patients with scleritis, 35 of whom were diagnosed with herpes virus infection, seen at 2 tertiary referral centers. We studied the clinical features and ocular complications of this subset of patient with scleritis. MAIN OUTCOME MEASURES: Correlation between classification, severity, and symptoms (i.e., pain) and diagnosis of herpetic-associated scleritis. Vision loss, presence of associated uveitis, keratitis, glaucoma, or systemic disease were documented over the follow-up period. Other outcome measures included epidemiologic data: age, gender, laterality, visual acuity, duration of symptoms, and underlying systemic or ocular diseases. RESULTS: Of 500 patients with scleritis, 47 (9.4%) had an underlying infectious cause. Thirty-five (74.4%) of these were diagnosed with herpes virus infection, 5 (10.6%) with tuberculosis, and the remaining 7 (14.8%) with other infectious disease. Patients with herpes-associated scleritis were analyzed as a group and then compared with those with idiopathic scleritis. Most patients with herpetic scleritis presented with acute (85.7%) and unilateral (80%) scleral inflammation. Pain was moderate or severe in 68.6% of the patients. The most common type of scleritis was diffuse anterior in 80% (n = 28), followed by nodular anterior 11.4% (n = 4), and necrotizing in 8.6% (n = 3). Necrotizing anterior scleritis was more commonly seen in patients with herpetic scleritis versus patients with idiopathic disease (8.6% vs 1.2%; P<0.05). Unilaterality was also more common in herpetic scleritis (80%) than in idiopathic disease (56.7%; P<0.05). Vision loss was significantly greater in herpetic than idiopathic scleritis (34.3% vs 11.5%; P<0.001). CONCLUSIONS: The association between scleritis and infectious disease may be higher than previously reported by other series. Herpes viruses account for 7% of all scleritis cases and its diagnosis may be challenging when there is not a classically diagnostic clinical picture. We present the observed clinical features of herpetic scleritis and describe the clinical differences at presentation between patients with idiopathic scleritis and those with herpes infection.

Chronic herpes simplex scleritis: characterization of 9 cases of an underrecognized clinical entity.

PURPOSE: To characterize in detail persistent and recalcitrant infectious scleritis resulting from herpes simplex virus (HSV). DESIGN: Retrospective and interventional clinical and immunopathologic case series. METHODS: Nine patients with chronic scleral redness, edema, and pain refractory to conventional therapy underwent a scleroconjunctival biopsy for routine histopathologic evaluation and definitive immunodiagnosis for the presence of HSV. Immunofluorescent probing for HSV was performed on the patient specimens. Negative controls for HSV included elimination of anti-HSV and anti-varicella zoster virus antibody in the probing process and the use of normal human conjunctiva and sclera as substrates. Response to therapy with acyclovir was monitored and recorded. RESULTS: The average age of the affected patients was 50.2 years, and the average duration of symptoms before tissue diagnosis of herpetic scleritis was 3.2 years (median, 4 years). Three histopathologic patterns were discovered: granulomatous inflammation (2 cases), plasma cell-rich pyogenic granuloma-like pattern (1 case), and reactive fibroinflammatory pattern (6 cases). Herpes antigen was demonstrated uniformly by immunofluorescence in a perivascular distribution and less commonly in the interstitium. Varicella zoster virus was not detected, and all controls for nonspecific antibody reagent binding to tissue showed negative results. Acyclovir caused a dramatic improvement in the chronic or recurrent ocular inflammation in all instances, with an average duration of improvement of inflammation of 15.3 months. CONCLUSIONS: Unrecognized HSV infection can cause longstanding scleritis. Histopathologic features of HSV scleritis are varied and nonspecific; immunofluorescent demonstration of HSV protein can make a definitive diagnosis. Prolonged administration of acyclovir is required for effective therapy.

Ocular manifestations associated with chikungunya.

PURPOSE: To report ocular manifestations associated with chikungunya. DESIGN: Retrospective, nonrandomized, observational case series. PARTICIPANTS: Nine chikungunya patients with ocular involvement. METHODS: All patients with chikungunya infection presenting with ocular complaints from September 2006 to October 2006 were included in the study. The infection was confirmed by demonstration of chikungunya immunoglobulin M antibody in sera of all patients. All patients underwent an ophthalmic examination including fundus photography, fundus fluorescein angiography, confocal microscopy of keratic precipitates, and optical coherence tomography. Positive ocular changes were recorded and tabulated. MAIN OUTCOME MEASURES: Characteristics, frequency, and locations of ocular lesions found in the participants. RESULTS: There were 9 patients with ocular lesions; 1 had nodular episcleritis, 5 presented with acute iridocyclitis, and 3 had retinitis. Four to 12 weeks before the development of ocular manifestations, all of these patients had fever. Although there were no specific changes of iridocyclitis that were diagnostic of the fever, the retinal changes were consistent with viral retinitis. All patients recovered from the infection with relatively good vision. CONCLUSION: It appears that iridocyclitis and retinitis are the most common ocular manifestations associated with chikungunya, with a typically benign clinical course. Less frequent ocular lesions include episcleritis. All the patients responded well to the treatment with preservation of good vision. To the best of our knowledge, similar ocular manifestations associated with chikungunya infection have not been reported. In the differential diagnosis of iridocyclitis and retinitis with features suggestive of a viral infection, the entity of chikungunya-associated ocular changes should be considered in the regions affected by the epidemic.

Scleritis associated with hepatitis C: a case report and review of literature.

There has been an increase in the complications associated with hepatitis C. Scleritis is one of the complications of the condition that is not routinely suspected, and thus underdiagnosed or missed, leading to delay in initiation of treatment. This case report will bring that to the attention of ophthalmologists so necessary systemic therapy can be instituted in a timely fashion.

Herpes zoster virus sclerokeratitis and anterior uveitis in a child following varicella vaccination.

PURPOSE: To report a case of herpes zoster virus sclerokeratitis with anterior uveitis following vaccination with live attenuated varicella vaccine (Oka strain). DESIGN: Case report. METHODS: The case records of the patient were reviewed retrospectively. Pertinent literature citations were identified using MEDLINE. RESULTS: A 9-year-old boy presented with herpes zoster ophthalmicus 3 years following vaccination with live attenuated varicella vaccine (Oka strain). Examination of the affected eye revealed a moderate follicular response on the palpebral conjunctiva, decreased corneal sensation, mildly elevated intraocular pressure, diffuse anterior scleritis with marginal keratitis, and a moderately severe anterior uveitis. Amplified DNA from fluid taken from the base of a cutaneous vesicle produced wild-type varicella zoster virus (VZV) DNA, not Oka strain. CONCLUSIONS: Herpes zoster virus infection needs to be considered in all patients who present with scleritis, keratitis, or anterior uveitis, regardless of their varicella vaccination status.