Monitoring therapy in anterior necrotizing scleritis with inflammation with anterior segment optical coherence tomography

Introduction Early diagnosis and initiation of immunosuppression can prevent the necessity of surgical intervention in necrotizing scleritis with inflammation and lowers the risk of perforation and loss of vision. However, clinical signs for early diagnosis and methods for monitoring response to immunosuppressive therapy are missing. Methods Here, we present a case of necrotizing scleritis with inflammation where avascular plaques precede scleral defects. We use slit lamp imaging and anterior segment optical coherence tomography to evaluate evolution lesions depth and impact on scleral structure. Results The patient presented 5 months after detection of avascular plaques with a new scleral ulcer of the left eye. After 3-day-administration of i.v. corticosteroids anterior segment optical coherence tomography showed progressive scleral thickening. The patient was therefore spared surgical intervention and discharged resulting in complete remission under decreasing doses of oral corticosteroids. Conclusions Avascular plaques can precede necrotizing scleritis with inflammation by several months and may therefore qualify as early clinical signs. Anterior segment optical coherence tomography enables objective evaluation of scleral structure for making rational decisions about surgical intervention.

Granulomatosis with Polyangiitis with Ocular Manifestations

Granulomatosis with polyangiitis (GPA) is a granulomatous-necrotic systemic vasculitis with a lesion of predominantly the upper and lower respiratory tracts at the onset of the disease (vasculitis, accompanied by granulomatous inflammation), and subsequently renal (glomerulonephritis). In addition, GPA may manifest as inflammation of small arteries and veins. Despite many years of study of this disease, the etiology of GPA remains unknown. The present case is about a 47-year-old female, who had been suffering from necrotizing scleritis, corneal ulcer, and secondary glaucoma in both eyes for 3 months, and she was treated with anti-inflammatory and antimicrobial therapy that showed no effect; the patient’s general condition became worse. In the second week of treatment, multiple abscess ruptures exposed the sclera. Sampling of the affected conjunctival tissue and positive HLA B8 haplotype and ANCA (PR3-ANCA) testings make it clear that GPA was the main reason of necrotizing scleritis with inflammation. The targeted treatment of the underlying disease allows to stabilize an inflammation of corneal and scleral lesions.