Supplementary Material for: Working Under Pressure: Scleroderma Presenting with Bilateral Exudative Retinal Detachment in the Context of Scleroderma Renal Crisis – A Case Report

This is an observational case report to detail a novel case of scleroderma renal crisis presenting as bilateral exudative retinal detachments in a patient with newly diagnosed systemic sclerosis. An otherwise healthy 58-year-old female presented primarily with vision complaints and was found to have malignant hypertension (230/120 mmHg) and bilateral exudative retinal detachment on dilated fundus examination and macular OCT scan. Further history revealed sclerodactyly, mild dysphagia, and dyspnea. She was diagnosed with diffuse Systemic Sclerosis and Sjogren's Syndrome complicated by an episode of scleroderma renal crisis based on initial medical workup. She was admitted to intensive care for management of refractory hypertension with IV antihypertensive therapy. Three-months after treatment, her visual symptoms and ocular findings resolved. The presence of exudative retinal detachment among other signs of hypertensive retinopathy warrants thorough systemic screening for underlying causes of malignant hypertension, including systemic sclerosis. Treatment of the underlying disease with urgent antihypertensive therapy resolved the exudative retinal detachments and restored vision in the case of a scleroderma renal crisis.

本研究为一项观察性病例报告，旨在详细描述1例新发确诊系统性硬化症（Systemic Sclerosis）患者并发硬皮病肾危象，以双侧渗出性视网膜脱离为首发表现的罕见病例。患者为58岁既往体健女性，因视力不适为主诉就诊，散瞳眼底检查及黄斑光学相干断层扫描（OCT）结果显示其罹患恶性高血压（血压230/120 mmHg）伴双侧渗出性视网膜脱离。进一步问诊发现患者存在硬指症、轻度吞咽困难及呼吸困难。经初始医学检查评估，患者被诊断为弥漫性系统性硬化症合并干燥综合征（Sjogren's Syndrome），并发硬皮病肾危象。患者随后被收入重症监护病房，接受静脉降压治疗以控制难治性高血压。治疗3个月后，患者视力症状及眼部体征完全缓解。当渗出性视网膜脱离伴随高血压视网膜病变等其他体征出现时，需对恶性高血压的潜在病因开展全面系统筛查，其中包括系统性硬化症。本病例表明，针对硬皮病肾危象的基础疾病联合紧急降压治疗，可有效逆转渗出性视网膜脱离并恢复视力。