Purpose
To estimate the incidence of scleritis in Lyme disease and report clinical features.
Design
Incidence rate estimate and case series.
Methods
Data were collected from an electronic medical record on patients with scleritis presenting to the Wilmer Eye Institute between January 1, 2012 and December 31, 2020. A diagnosis of Lyme disease was made using the Infectious Diseases Society of America, American Academy of Neurology, and the American College of Rheumatology 2020 joint criteria plus a response to antibiotic therapy. After identifying all new-onset cases of scleritis in the database, the proportion of new-onset scleritis with Lyme disease was calculated. The proportion of Lyme disease cases with scleritis was estimated using the number of cases with Lyme disease from the Baltimore metropolitan area reported to the Centers for Disease Control and Prevention. After querying other major eye centers in the area for any cases of Lyme disease scleritis, none were identified, and the incidence of Lyme disease scleritis was estimated using published U.S. Census data for the greater Baltimore metropolitan area.
Results
Six cases of Lyme disease scleritis were identified in the 8-year time frame; 1 additional case was identified in the following year. Lyme disease scleritis accounted for 0.6% of all cases of scleritis, and 0.052% of patients with Lyme disease had scleritis. The estimated incidence of Lyme scleritis was 0.2 per 1,000,000 population per year (95% confidence interval 0-0.4), whereas the estimated incidence of Lyme disease in the area was 3 per 10,000 population per year (95% confidence interval 2.9-3.1). All scleritis cases were anterior, unilateral, without necrosis, and resolved with antibiotic use without relapse in a median of 39.5 days (range 29-57 days). Other features of Lyme disease were present in 4 of 7 patients, including a history of erythema migrans in 2 of 7 patients.
Conclusions
Lyme disease is an uncommon cause of scleritis in endemic areas.
N orth American Lyme disease is a multisystem infectious disease caused by the bacterium Borrelia burgdorferi . Skin disease starts at the site of an infected tick bite, and the Lyme spirochete can disseminate in the first weeks of infection to the joints, heart, and the nervous system. Ocular involvement in Lyme disease has been reported at any stage of the disease and involve almost all ocular and adnexal structures, as well as the cranial nerves involved in oculomotor function.
Lyme disease rarely has been reported as a cause of scleritis in several case reports or as isolated cases included as part of a larger series of scleritis, mostly from endemic areas of the United States or Europe. We report a case series of Lyme disease scleritis collected over a 9-year period in a Lyme disease endemic region and estimate the incidence of Lyme scleritis.
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.
STATISTICS
The estimated population incidence of scleritis in the Baltimore metropolitan area over the years January 1, 2012 through December 31, 2019 was calculated as the number of cases of Lyme disease scleritis from the greater Baltimore metropolitan area divided by the average Baltimore metropolitan area population during this time divided by 8 years. The same calculation was repeated for the estimated population incidence of Lyme disease in the Baltimore metropolitan area using U.S. Center for Disease Control and Prevention (CDC) data. We also calculated 95% confidence intervals (CIs). The time interval for the estimated population incidences included January 1, 2012 through December 31, 2019, as the results of the 2020 CDC report on Lyme disease and the 2020 Census by the U.S. Census Bureau were not released at the time of this study. The greater Baltimore metropolitan area is defined by the U.S. Census Bureau as Baltimore City and the following 5 surrounding counties: Anne Arundel, Carroll, Baltimore, Harford, and Howard.
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.
| Characteristic | Value |
|---|---|
| Patients, N | 7 |
| Demographics | |
| Median age, y (range) | 43 (24-81) |
| Male, n (%) | 4 (57) |
| Race, n (%) | |
| White, non-Hispanic | 5 (72) |
| Black, non-Hispanic | 1 (14) |
| Hispanic | 1 (14) |
| Reside in Baltimore metropolitan area, n (%) | 6 (86) |
| Known tick bite, n (%) | 3 (43) |
| Presence of other ophthalmic manifestations, n (%) | 0 (0) |
| Characteristics of scleritis, n (%) | |
| Unilateral eye disease | 7 (100) |
| Scleritis type | |
| Sectoral anterior scleritis | 3 (43) |
| Nodular anterior scleritis | 3 (43) |
| Necrotizing scleritis | 0 (0) |
| Posterior scleritis | 0 (0) |
| Sclerokeratitis | 1 (14) |
| Characteristics of Lyme disease, n (%) | |
| Erythema migrans | 2 (28) |
| Cardiovascular disease a | 1 (14) |
| Arthritis | 1 (14) |
| Laboratory data, b n (%) | |
| Positive immunoglobulin M antibody | 1 (14) |
| Positive immunoglobulin G antibody | 5 (72) |
| Antibiotic treatment regimen, n (%) | |
| Intravenous ceftriaxone | 0 (0) |
| Oral doxycycline | 7 (100) |
| Oral cefuroxime c | 1 (14) |
| Mean days until resolution (range) | 41 (29-57) |
| Postantibiotic treatment recurrence, n (%) | 0 (0) |
| Antiinflammatory treatment regimen, n (%) | |
| Oral indomethacin | 2 (28) |
| Oral ibuprofen | 1 (14) |
| Oral flurbiprofen | 1 (14) |
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