Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Int J Rheum Dis. 2023 Jul;26(7):1373-1376. doi: 10.1111/1756-185X.14623. Epub 2023 Feb 20.
Purtscher-like retinopathy, an occlusive microvasculopathy, is a rare and severe ophthalmic complication of systemic lupus erythematosus (SLE) characterized by a sudden loss of vision with retinal whitening, cotton wool spots and minimal intraretinal hemorrhage. Recovery in visual acuity is usually poor even with prompt treatment. This case showed a patient with SLE concurrent Purtscher-like retinopathy treated with rituximab and interleukin-2 (IL-2) with good prognosis. A 16-year-old female with a 2-year history of SLE was admitted because of unrelieved disease activity of SLE when treated with a high dose of corticosteroids and immunosuppressants and she further suffered from reduced visual acuity in both eyes. She was diagnosed with Purtscher-like retinopathy secondary to SLE after ocular examination. Rituximab and low-dose IL-2 for systemic treatment and intravitreal injection of anti-vascular endothelial growth factor antibody to right eye were given. The SLE disease was completely relieved with the sight recovering and no recurrence of Purtscher-like retinopathy was reported during 6-year follow-up. Purtscher-like retinopathy associated with SLE should be treated early and promptly. Rituximab should be considered in SLE patients with Purtscher-like retinopathy who have an incomplete response to initial immunosuppressive therapy and low-dose IL-2 may help induction of clinical remission.
Purtscher 样视网膜病变是一种闭塞性微血管病,是系统性红斑狼疮(SLE)的一种罕见且严重的眼部并发症,其特征是突然视力丧失、视网膜苍白、棉絮斑和微量视网膜内出血。即使及时治疗,视力恢复通常也不佳。本病例报告了一例同时患有 SLE 和 Purtscher 样视网膜病变的患者,经利妥昔单抗和白细胞介素-2(IL-2)治疗后预后良好。一名 16 岁女性,患有 SLE 病史 2 年,因大剂量皮质类固醇和免疫抑制剂治疗后疾病活动未缓解而入院,且双眼视力进一步下降。眼部检查后诊断为 SLE 继发 Purtscher 样视网膜病变。给予利妥昔单抗和低剂量 IL-2 全身治疗,并对右眼进行抗血管内皮生长因子抗体眼内注射。SLE 疾病完全缓解,视力恢复,6 年随访期间未再发生 Purtscher 样视网膜病变。与 SLE 相关的 Purtscher 样视网膜病变应早期及时治疗。对于初始免疫抑制治疗反应不完全且低剂量 IL-2 可能有助于诱导临床缓解的 Purtscher 样视网膜病变的 SLE 患者,应考虑使用利妥昔单抗。