RESUMO
INTRODUCTION: Necrotizing scleritis (NS) presents 30%-40% as having a systemic autoimmune condition. OBJECTIVE: To present a clinical case report and a systematic review of necrotizing scleritis with ocular manifestation as the first sign of rheumatologic disease. METHODS: The present study was elaborated according to the rules of CARE. CASE REPORT: A female patient, 63 years old, a white, administrative assistant, presented irritation, low visual acuity (LVA) in the left eye (LE), and headache. Biomicroscopy (BIO) in the right eye (RE) was normal, and the LE showed hyperemia and scleral thinning. After 1 month, the patient returns without signs of infectious diseases in the exams, and after a rheumatological evaluation with a diagnosis of rheumatoid arthritis, methotrexate and prednisone are prescribed. After 2 months, she relapsed and started treatment with anti-TNF, with remission after the 4th dose. After 1 year, she evolved with LVA in LE. RESULTS: A total of 244 articles were found, 104 articles were evaluated and 10 were included in the brief review. The symmetrical Funnel Plot does not suggest a risk of bias. CONCLUSION: Both in the present case report and the literary findings, it was evidenced that the ophthalmologic findings may precede the systemic changes of the disease for the early diagnosis of rheumatoid arthritis.