Anti-TNF alpha therapy in chronic necrotizing scleritis resistant to standard immunomodulatory therapy in a patient with Wegener's granulomatosis

Sir,

Wegener's granulomatosis is characterized by a necrotizing granulomatous vasculitis. Ocular complications may include uveitis, scleritis, and retinal vasculitis and occur in up to 45% of patients.^{1, 2}

Case report

A 75-year-old man was referred to our clinic for peripheral ulcerative keratitis and anterior uveitis in both eyes. High serum proteinase-3 antibody levels in addition to a biopsy of the nasal mucosa revealing a necrotizing granulomatous vasculitis confirmed the diagnosis of Wegener's granulomatosis (WG). His visual acuity (VA) was 20/50 in the right eye (OD) and 20/100 in the left eye (OS). Systemic immunomodulatory therapy with oral cyclophosphamide (3 mg/kg/BW) with 75 mg glucocorticoids was initiated; however, despite adjustment of cyclophosphamide (4 mg/kg/BW), the scleritis remained active over the following 5 months (Figure 1a). His VA dropped to 20/200 OD and counting fingers OS. Owing to ongoing inflammation and positive reports on infliximab in systemic Wegener's granulomatosis,^{3, 4, 5} infliximab was started at 5 mg/kg/BW on day 0, and repeated at weeks 2, 6, thereafter every 8 weeks. Cyclophosphamide was discontinued after week 6. After the second infliximab infusion, the anterior chamber (a/c) cell counts as well as the necrotizing scleritis decreased. At week 8, his scleritis was in remission. His VA increased to 20/60 OD and remained at counting fingers OS. Prednisolone was tapered to 12.5 mg/day (Figure 1b). After 5 months, infliximab was discontinued due to an acute herpes zoster infection, resulting in a flare-up 6 weeks later with 4+ a/c cells OD (Figure 1c) and an almost total melting of the sclera OS. His VA was counting fingers OD and light perception OS. Therefore, infliximab (5 mg/kg/BW) was restarted and azathioprine at 1.5 mg/kg BW and prednisolone 50 mg/day were added. For the last 8 months, infliximab has been administered every 4–5 weeks keeping the scleritis under remission (Figure 1a). His VA increased to 20/400 in the right eye. The corticosteroid dose was tapered to 12.5 mg/day.

Figure 1

(a) Photograph demonstrates the active necrotizing scleritis with inflammation of the eye. (b) At 5 months after infliximab therapy has been started. The scleritis is almost completely in remission. (c) Reactivation of the scleritis after discontinuation of the scleritis. (d) At 3 months into reinstallation of combined infliximab and azathioprine therapy.

The classic regimen consists of cyclophosphamide and prednisone.^{1, 2} However, as not all patients sufficiently respond to standard therapy, and due to its toxicity alternative treatment should be considered. This is just a single case report. Still the fact that our case, in addition to previous reports, shows the effectiveness of specific TNFα-blockers in systemic WG^{3, 4, 5} suggests that selective TNFα inhibition is an option in ocular manifestations of this disease.