Efficacy of tumour necrosis factor inhibitors in peripheral ulcerative keratitis in Granulomatosis with polyangiitis

• Published in: Acta ophthalmologica (Oxford, England) England), 2017-09, Vol.95 (S259), p.n/a
• Main Authors: Verly, E., De Kock, J., Leroy, B.P., Sys, C., De Schryver, I.
• Format: Article
• Language: English
• Subjects: Antineutrophil cytoplasmic antibodies; Biopsy; Computed tomography; Cornea; Corticoids; Corticosteroids; Cyclophosphamide; Females; Gangrene; Granulomatosis; Immunomodulation; Inflammation; Inflammatory diseases; Infliximab; Keratitis; Lymphadenopathy; Monoclonal antibodies; Necrosis; Polymerase chain reaction; Septum; Serology; Tumor necrosis factor-TNF; Tumor necrosis factor-α; Tumors; Vasculitis; Vein & artery diseases
• ISSN: 1755-375X; 1755-3768
• DOI: 10.1111/j.1755-3768.2017.0T077

Purpose To report the efficacy of TNFi (tumour necrosis factor inhibitor), as a treatment of therapy‐resistant peripheral ulcerative keratitis (PUK) in granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis. Methods Observational report about 2 cases, known with the diagnosis of GPA, presenting with therapy‐resistant PUK. Results 2 middle‐aged females, known with GPA were referred to our department with a history of chronic redness resistant to local corticosteroids. Both patients showed a unilateral limbal vasculitis with necrotizing keratitis on slit lamp examination. In the first case, the diagnosis of GPA was confirmed by positive anti‐neutrophil cytoplasmic antibodies (ANCA) and nasal septum biopsy. In the second case, the clinical presentation was considered as a viral keratitis due to recurrent unilateral inflammation. Polymerase chain reaction (PCR) could not confirm the viral aetiology. Extensive systemic evaluation revealed a cervical lymphadenopathy on PET‐CT scan. Serology was positive for ANCA. In both cases, the PUK was nonresponsive to systemic corticosteroids. In the first case, cyclophosphamide failed to control the inflammation. Because the risk of corneal perforation, a treatment with TNFi, respectively Infliximab and Adalimumab, were successfully initiated. Conclusions Potentially lethal, it is important to diagnose and treat GPA urgently. Recently, TNFi were reported to be effective in the treatment of therapy‐resistant GPA. Both patients were refractory to conventional immunomodulatory therapy. Infliximab and adalimumab successfully controlled the inflammation with disappearance of necrotic foci. In our experience, TNFi are a safe and effective therapeutic strategy.