Necrotizing Scleritis: Clinical Outcomes at a Tertiary Academic Center
Purpose
Necrotizing scleritis is the most severe form of anterior scleritis and can result in scleral perforation. We evaluated a cohort of patients with necrotizing scleritis to determine risk factors for perforation.
Methods
Retrospective study of 35 patients seen at the Wilmer Eye Institute from January 2006 to September 2022. Data collected demographics, immunomodulatory therapy (IMT), whether perforation occurred, and surgical management.
Results
Median age at presentation was 69 years. 57% of patients were male, and 63% were Caucasian. Infectious scleritis was diagnosed in 6 patients (17%). A systemic illness was present in 18 patients (51%); ANCA associated vasculitis was the most common, followed by rheumatoid arthritis. At baseline, 39 eyes (57%) had scleral thinning, and 4 eyes (6%) had perforated prior to presentation. During follow-up 2 eyes perforated in a median time of 10 days. Surgical management consisted of three patch grafts in one eye and no surgery performed in the other eye. At the time of perforation both patients were on oral prednisone at a median dose of 47.5 mg. Only one of the patients was on other IMT (100 mg/daily oral Cytoxan). In patients who experienced perforation in at least one eye prior, 33% (N=2) had an underlying systemic illness.
Conclusion
The percent of patients with necrotizing scleritis requiring surgical management decreases with aggressive initiation of prednisone with combination immunosuppressive therapy. Infectious scleritis is common and must be appropriately ruled out and treated prior to beginning immunosuppressive therapy. Long term immunosuppressive therapy can further reduce the risk for perforation, especially in those with an underlying autoimmune disorder.
Conflict of interest
No
Authors 1
Last name
MALLEM
Initials of first name(s)
K
Department
Division of Ocular Immunology, Wilmer Eye Institute, Johns Hopkins School of Medicine
City
Baltimore, MD
Country
United States
Authors 2
Last name
Nguyen
Initials of first name(s)
J
Department
Drexel University College of Medicine
City
Philadelphia, PA
Country
United States
Authors 3
Last name
Chaon, MD
Initials of first name(s)
B
Department
Division of Ocular Immunology, Wilmer Eye Institute, Johns Hopkins School of Medicine
City
Baltimore, MD
Country
United States
Authors 4
Last name
Burkholder, MD
Initials of first name(s)
B
Department
Division of Ocular Immunology, Wilmer Eye Institute, Johns Hopkins School of Medicine
City
Baltimore, MD
Country
United States
Authors 5
Last name
Thorne, MD, PhD
Initials of first name(s)
J
Department
Division of Ocular Immunology, Wilmer Eye Institute, Johns Hopkins School of Medicine
City
Baltimore, MD
Country
United States
Authors 6
Last name
Berkenstock, MD
Initials of first name(s)
M
Department
Division of Ocular Immunology, Wilmer Eye Institute, Johns Hopkins School of Medicine
City
Baltimore, MD
Country
United States
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