Necrotizing Scleritis and Peripheral Ulcerative Keratitis Associated with Wegener’s Granulomatosis

Abstract

IntroductionTo evaluate the complications, efficacy of medical and surgical treatment, and outcome in patients with necrotizing scleritis and peripheral ulcerative keratitis associated with Wegener’s granulomatosis.MethodsThe authors reviewed a series of seven patients with Wegener’s granulomatosis treated in the Corneal Department of Zhongshan Ophthalmic Center and the Department of Ophthalmology of Kashgar First People’s Hospital. A detailed chart review was performed to determine demographic characteristics, ocular presentation, biopsy and laboratory testing results, treatment, and final outcome.ResultsWegener’s granulomatosis was indicated by ocular and/or systemic findings; biopsy and immunohistochemistry results supported the diagnosis. Patients with necrotizing scleritis and/or peripheral ulcerative keratitis received cytotoxic immunosuppressive therapy; this, in conjunction with surgical treatment, halted the relentlessly progressive inflammation and preserved the integrity of the globe in 78% of eyes. Best-Corrected Visual Acuity remained stable in four of nine eyes, was improved in two of nine eyes, and decreased in three of nine eyes (secondary to cataract and/or stromal scarring). Although one patient died, treatment with corticosteroids and cytotoxic agents dramatically improved outcomes in these patients.ConclusionNecrotizing scleritis and peripheral ulcerative keratitis often have a poor visual outcome, and may herald an underlying systemic vasculitis. Wegener’s granulomatosis, with the associated necrotizing scleritis and peripheral ulcerative keratitis, should be managed with aggressive immunosuppression to avoid the associated morbidity and mortality. Thus, the ophthalmologist may play a significant role in its early diagnosis and treatment.

Scleritis in patients with granulomatosis with polyangiitis (Wegener)

“…Mean age at diagnosis was similar to that reported in other series 23. We found a discreet female preponderance (female-to-male ratio of 1.8:1) compared with 2:5 found in our previous series, while Gu et al 19 found a male preponderance. The disagreement observed might be due to the small number of cases found.…”

“…These findings differ from our previous series, where the proportions were 14% for diffuse anterior and 78% for necrotising scleritis in patients with GPA,2 but are in agreement with those reported in other series. Hoang et al 23 found that 71% had diffuse anterior scleritis, Watkins et al 22 described that 47.6% of patients showed diffuse anterior and 28% showed necrotising scleritis and Gu et al 19 reported that 86% of their patients suffered a necrotising disease. Again, the observed difference could be due to the low prevalence of the disease; this also likely explains why in this current series we did not find patients with associated posterior scleritis, while other series described a prevalence of 3.6% and 14%, as well as single case reports of posterior scleritis as the initial manifestation of PR3-ANCA-associated vasculitis 22–24.…”

Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis

“…Scleritis in GPA can be diffuse, nodular, or necrotizing. However, the presence of necrotizing scleritis is most suggestive of underlying GPA and it can significantly increase the risk of loss of vision and blindness [7, 8]. The clinical features of GPA-associated scleritis are distinct from other forms of scleritis.…”

Clinical findings, pathogenesis and treatment in non-infectious peripheral ulcerative keratitis

“…PUK pathogenesis in WG is explained through pathological B and T lymphocytes, and possibly antineutrophil cytoplasmic antibodies (ANCA) (91). The antineutrophil cytoplasmic antibody test in cases of PUK coexisting with WG is a specific and sensitive indicator (92). ANCA binds to receptors in neutrophils and monocytes stimulated by cytokines.…”

“…PUK associated with WG is severe and perforation is common. Therefore, surgical treatment is usually required in addition to systemic immunosuppressive treatment (92).…”