Diffuse granulomatous necrotizing scleritis.
METHODS
A 34-year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiretinal neovascular membrane in the LE. Topical and systemic oral steroids and antiviral medication were prescribed. One year later, optic disc hyperaemia and swelling and macular oedema became apparent in the LE. Pulsed intravenous steroids were administered for 1 year, when a nasal septum perforation and vitreous haemorrhage in the LE were diagnosed. The eye was enucleated 3.5 years after the initial complaint. Necrotizing granulomatous tissue replacing the sclera and subconjunctival granulomatous tissue were observed. Six months later, oedema and neovascularization of the right optic disc were observed and cyclophosphamide was started, with regression of the clinical signs. No systemic abnormalities have so far become apparent.


CONCLUSIONS
Posterior scleritis is most often observed in patients with no signs of associated systemic autoimmune disease. The diagnosis in this case is most probably one of Wegener's granulomatosis (WG). In WG, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis. Eye involvement with WG has been reported in up to 58% of cases. Evaluation of the scleritis patient should include a detailed medical history, chest X-rays, blood tests, autoantibody serology and analysis of urinary sediment. Cyclophosphamide is the treatment of choice for patients with ocular manifestations of WG, polyarteritis nodosa or rheumatoid arthritis, either alone or in combination with systemic steroids. Visual loss is expected in 85% of individuals with severe necrotizing posterior scleritis.
Pecorella
Irene
I
Department of Experimental Medicine and Pathology, University of Rome, Rome, Italy. irene.pecorella@uniroma1.it
La Cava
Maurizio
M
Mannino
Giuseppe
G
Pinca
Marco
M
Pezzi
Paola Pivetti
PP
D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D015353:Eye Enucleation; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000008:administration & dosage; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D020551:Pulse Therapy, Drug; D015423:Scleritis / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D014463:Ultrasonography
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide