PATIENTS AND METHODS

Cases of newly diagnosed scleritis seen at the Wilmer Eye Institute from January 1, 2012 through December 31, 2020 were identified using the SlicerDicer application in the EPIC electronic medical record system (EPIC Systems Corp, Verona, WI). Lyme disease scleritis cases were defined based on the Infectious Diseases Society of America, American Academy of Neurology, and the American College of Rheumatology 2020 joint guidelines for the diagnosis of Lyme disease. In brief, the diagnosis of Lyme disease required either erythema migrans (EM) or a positive serologic test for Lyme disease. Serologic diagnosis required a positive enzyme-linked immunosorbent assay test followed by confirmatory Western blot test (2 immunoglobulin M [IgM] or 5 immunoglobulin G [IgG] positive bands) concurrent with or after the diagnosis of scleritis. To determine if there was active Lyme disease in a patient with a positive IgM test result alone, a repeat Western blot with 5 positive IgG bands was required. If EM rash was present, a documented clinical response to antibiotic therapy was required in patients without a Western blot result. In addition to screening for Lyme disease in all patients with scleritis in our practice, each was tested for alternative scleritis-associated diseases, including both treponemal and nontreponemal syphilis serologies, a chest radiograph, human leukocyte antigen-B27 typing, antineutrophil cytoplasmic antibody testing, and, if nodular scleritis was present, an interferon gamma release assay test for tuberculosis. ^, In addition, to decrease the likelihood that the Lyme serology and the scleritis were merely coincidental, we required that the scleritis responded to appropriate antibiotic therapy. The response had to be either to antibiotic use alone or if the patient had been treated with antiinflammatory treatment, after the failure of antiinflammatory therapy alone to achieve resolution of the scleritis. Clinical resolution was defined as an absence of injection of the blood vessels of the episcleral plexus, absence of scleral nodules or necrosis, grade 0 anterior chamber inflammation, and resolution of corneal infiltrates (if sclerokeratitis had been present) on examination. These investigations adhered to the Declaration of Helsinki, and approval from the Johns Hopkins Hospital Institutional Review Board was obtained for the study.

Data analyzed included demographics, anatomic class of scleritis, presence of nodules or necrosis, corneal infiltrates, laterality, systemic Lyme disease manifestations, history of tick bites, presenting best corrected visual acuity and intraocular pressure, Western blot results, antibiotic and antiinflammatory medication use, time until scleritis resolution with antibiotic therapy alone or with antiinflammatory agents after the start of antibiotic therapy, recurrences, and postantibiotic ocular treatments.

The primary outcome was determining the incidence of both Lyme disease and Lyme scleritis in the greater Baltimore area. Secondary outcomes included determining the percent of scleritis cases attributable to Lyme disease, the percent of Lyme disease patients with scleritis, and identification of associated clinical and demographic features of patients with Lyme scleritis. No additional cases of Lyme scleritis were identified by the other academic medical centers in the greater Baltimore metropolitan area after querying each of these organizations to identify such cases.

RESULTS

Nine hundred eighty cases of scleritis were seen between January 1, 2012 and December 31, 2020. Six of these cases were caused by Lyme disease (0.61%). A seventh case of Lyme scleritis was identified between January 1, 2021 and September 1, 2021. Table 1 lists the clinical characteristics of the 7 patients with Lyme disease scleritis. The average age was 43 years (range 24-81 years); 4 of the patients were male (57%) and 5 were white (72%). Lyme disease–associated scleritis accounted for 0.6% of all cases of scleritis, and 0.052% of patients with Lyme disease had scleritis.