P TAN1, D LANGSFORD1, T PIANTA1, J SAVIGE1
1Northern Health, Epping, Australia
Background: Purtscher-like retinopathy is a rare and severe angiopathy mediated by retinal microembolisation and complement activation. We report two cases of Purtscher-like retinopathy in context of systemic vasculitis and lupus nephritis. The condition was demonstrated with retinal imaging using a Canon non-mydriatic retinal camera.
Case Report: A 68-year-old male presented with hypertension and acute kidney injury requiring dialysis. Lower limb palpable purpuric rash was noted. Renal biopsy demonstrated cortical infarction, which was proven to be bilateral despite patent renal arteries on angiogram. Autoimmune, vasculitic and pro-thrombotic screens found no abnormalities. Skin biopsy demonstrated leucocytoclastic vasculitis. Severe lower limb ischemia developed despite patent lower limb arteries on angiogram. He then developed increasing confusion, cardiac instability and painless visual loss. Retinal imaging found Purtscher-like retinopathy. He was treated with prednisolone, cyclophosphamide and plasma exchange. At 9 months, whilst dialysis dependent, he has made an otherwise good recovery including now normal retinal findings with restored vision.
A 47-year-old female presented with nephrotic syndrome, hypertension, a malar rash. She was found to have class IV lupus nephritis associated with low complement factors (C3 and C4). Antiphospholipid screen was normal. She was treated with prednisolone, hydroxychloroquine and mycophenolate. At 3 months she achieved partial remission but reported blurred vision. Retinal imaging demonstrated Purtscher-like retinopathy.
Conclusion: Purtscher-like retinopathy is a rare microvascular occlusive retinal disease that can cause painless visual loss. It is associated with complement-mediated disorders including lupus and atypical haemolytic uremic syndrome. The pathogenesis is believed to be microembolic or thrombotic with complement activation and reflects the underlying systemic disease. It may improve with successful treatment of the systemic disease.
Pek Ghe Tan is a renal advanced trainee in Victoria, Australia. She is currently in her final year training in the Northern Hospital.