RESUMO
PURPOSE: The authors describe a man undergoing autologous stem cell transplant, who developed granulomatosis with polyangiitis and Purtscher'sâlike retinopathy. METHODS: A 25-year-old man underwent fundus photography, optical coherence tomography, and fluorescein angiography to obtain a diagnosis and follow the treatment course. RESULTS: The initial ophthalmic presentation and imaging confirmed the findings of Purtscher'sâlike retinopathy. Throughout his course, he had progressive neovascularization and vitreous hemorrhages in both eyes, requiring vitrectomy and endolaser, and bevacizumab injections. CONCLUSION: The authors describe a patient who underwent autologous stem cell transplantation, and subsequently developed granulomatosis with polyangiitis. The presentation of Purtscher'sâlike retinopathy suggests that microvascular occlusion in the retina was likely the result of granulomatosis with polyangiitis-driven vasculitis of the precapillary arterioles.
Assuntos
Granulomatose com Poliangiite , Transplante de Células-Tronco Hematopoéticas , Doenças Retinianas , Adulto , Angiofluoresceinografia/métodos , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Masculino , Doenças Retinianas/complicações , Doenças Retinianas/etiologia , Transplante Autólogo/efeitos adversosRESUMO
Cataracts, a clouding of the eye lens, are a leading cause of visual impairment and are responsible for one of the most commonly performed surgical procedures worldwide. Although generally safe and effective, cataract surgery can lead to a secondary lens abnormality due to transition of lens epithelial cells to a mesenchymal phenotype (EMT) and opacification of the posterior lens capsular bag. Occurring in up to 40% of cataract cases over time, posterior capsule opacification (PCO) introduces additional treatment costs and reduced quality of life for patients. Studies have shown that PCO pathogenesis is driven in part by TGF-ß, signaling through the action of the family of Smad coactivators to effect changes in gene transcription. In the present study, we evaluated the ability of Smad-7, a well characterized inhibitor of TGF-ß -mediated Smad signaling, to suppress the EMT response in lens epithelial cells associated with PCO pathogenesis. Treatment of lens epithelial cells with a cell-permeable form of Smad7 variant resulted in suppressed expression of EMT markers such as alpha smooth muscle actin and fibronectin. A single application of cell-permeable Smad7 variant in the capsular bag of a mouse cataract surgery model resulted in suppression of gene transcripts encoding alpha smooth muscle actin and fibronectin. These results point to Smad7 as a promising biotherapeutic agent for prevention or substantial reduction in the incidence of PCO following cataract surgery.