Bilateral Anterior Ischaemic Optic Neuropathy as First Manifestation of Henoch-Schönlein Purpura (IgA Vasculitis)

Abstract

A 46-year-old man was referred to our department complaining of a bilateral progressive decrease in his visual acuity. Fundus examination revealed bilateral optic disc oedema, indicative of anterior ischaemic neuropathy (AION), and a macular star in the right eye. Laboratory analysis showed low haematocrit and haemoglobin, elevated creatinine, and increased erythrocyte segmentation rate and C-reactive protein level. Physical examination revealed the presence of purpuric rash on the trunk and the extremities. During the investigation we performed a complete laboratory and imaging examination for autoimmune collagen diseases, vasculitides and infectious diseases, which were all negative. Histologic findings of renal biopsy were compatible with IgA glomerulonephritis and thus Henoch-Schönlein purpura (HSP) diagnosis was established. The patient was treated with methylprednisolone and cyclophosphamide. Six months later, his renal function and his visual acuity had improved, and the rash had subsided. This is a rare case of AION in a patient with HSP.

Systemic Vasculitis and Its Association with the Eye

“…Cases of ischemic optic neuropathy and retinal vasculitis have been described in IgAV [87][88][89][90][91]. But, the most common ocular manifestation of IgAN is episcleritis and scleritis [92,93].…”