Bilateral anterior ischaemic optic neuropathy with macular star as first manifestation of Henoch‐Schönlein purpura (IgA vasculitis)

Abstract

AbstractPurposeTo report a case of bilateral anterior ischaemic optic neuropathy with macular star in a patient with Henoch–Schönlein purpura (HSP).MethodsA 46‐year‐old man was referred to our clinic complaining of progressive decrease in visual acuity of the right eye (OD) since the last 10 days, and since 3 days in his left eye (OS). His visual acuity was ‘hand motion’ OD and 4/10 OS. Anterior segment examination was insignificant, while fundus examination revealed optic disc edema, and bilateral macular star. A relative afferent pupillary defect was present in his right eye with normal pupillary reflexes in the left eye. Laboratory analysis showed: Hematocrit: 22.1%, ESR: 140 mm/hr, CRP: 64.80 mg/dl, Creatinine: 4.4 mg/dl. Physical examination revealed presence of erythematous rash on the trunk and the extremities. The patient was admitted to the Nephrology department for treatment of his azotemia.ResultsDuring his hospital course we performed complete laboratory and imaging examinations for autoimmune diseases, vasculitis and infectious diseases, which were negative. Histologic findings of renal biopsy were compatible with IgA glomerulonephritis and thus HSP was diagnosed. The patient received oral methylprednisolone and intravenous cyclophosphamide therapy. Six months later his renal function had improved, the rash had subsided and his visual acuity was ‘counting fingers’ OD and 10/10 OS. Standard automated perimetry showed peripheral visual field depression bilaterally.ConclusionsHenoch–Schönlein purpura (HSP) is a small vessel vasculitis which is more common in children. Based on literature, ophthalmic manifestations of HSP usually involve the anterior segment and less frequently the posterior segment. Systemic vasculitis should be included in the differential diagnosis of bilateral optic disc edema.