[Peripheral ulcerative keratitis with necrotizing scleritis, manifestation of granulomatosis with polyangiitis]

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune disorder that typically affects small and/or medium size vessels. A collaborative management form ophthalmologist and rheumatologist could led to long term remission of the disease despite being yet a life threatening pathology.

A 61 years old masculine complied of recurrent cases of ocular discomfort managed as a bacterial conjunctivitis for 2 years, also patient with loss of visual acuity and weight loss mainly in the three previous months. After discard infectious diseases such as tuberculosis and syphilis being in a high incidence country GPA investigation were carried out and later diagnosed. He was to start proper treatment with rituximab but as systemic complications advance he could not managed to achieve disease control. Ocular involvement has been show to occur in around 30% percent of cases in several studies. It could cause scleritis as well as conjuntivitis, episcleritis, necrotizing keratitis, corneoscleral perforation, posterior uveitis, and optic neuritis. C-ANCA and high positive PR3 autoantibody have strong association and sensitivity with GPA. Rituximab has been prove to be the most accurate treatment for this disease.

Necrotizing scleritis and peripheral ulcerative keratitis can be manifestation of GPA. An ophthalmic check up may be the key to reach a promptly diagnosis of a tough systemic case.