Diffuse granulomatous necrotizing scleritis
| Autor: | Maurizio La Cava, Marco Pinca, Irene Pecorella, Giuseppe Mannino, Paola Pivetti Pezzi |
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| Rok vydání: | 2005 |
| Předmět: |
Adult
Male medicine.medical_specialty Histology genetic structures Wegener's grannulomatosis Fundus (eye) Posterior scleritis Visual loss Eye Enucleation Necrotizing Vasculitis medicine Humans Fluorescein Angiography Cyclophosphamide Glucocorticoids Ultrasonography Nasal septum perforation Polyarteritis nodosa business.industry Granulomatosis with Polyangiitis medicine.disease Dermatology eye diseases Sclera Surgery Ophthalmology medicine.anatomical_structure Pulse Therapy Drug sense organs Vasculitis business Immunosuppressive Agents Scleritis Optic disc |
| Zdroj: | Acta Ophthalmologica Scandinavica. 84:263-265 |
| ISSN: | 1395-3907 |
| Popis: | Case report: 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. Discussion: 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. |
| Databáze: | OpenAIRE |
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