Monov Simeon, Hristova Ruska, Dacheva Rositza, Toncheva Reni, Shumnalieva Russka, Shoumnalieva-Ivanova Viara, Monova Daniela
aClinic of Rheumatology, University Hospital "St. Iv. Rilski," Department of Internal Medicine bClinic of Ophthalmology, University Hospital "TsaritsaYoanna," cOphthalmology Outpatient Clinic, University Hospital "St. Iv. Rilski," dClinic of ophthalmology, University Hospital "Alexandrovska," eDepartment of Internal Medicine, Medical Institute-MID, Medical University, Sofia, Bulgaria.
Medicine (Baltimore). 2017 Jan;96(2):e5754. doi: 10.1097/MD.0000000000005754.
Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease. Due to its low frequency, we report a case of acute necrotizing retinal vasculitis as onset of SLE.
A 25-year-old white female was referred to the rheumatology clinic with gradually and rapid deterioration of the vision due to abnormal vessel permeability in the right fundus with edema along the vessels, occlusion of arterial branches in the middle periphery with leakage of the dye in these areas and indentical but less prominent changes with cotton wool spots in the papillomacular area and extensive hemorrhages in the left eye. The onset of malar rash, arthralgias and positive antinuclear, anti-double stranded DNA, anti-ribosomal P and anti-β2 glycoprotein I antibodies with decreased C4 complement levels, as well as the positive lupus-band test confirmed the diagnosis of SLE.
Aggressive immunomodulating therapy with high-dose methylprednisolone, intravenous immunoglobulin, and cyclophosphamide was used for suppression of the disease activity followed by azathioprine as maintaince therapy.
Substantial improvement and partial resorption of the vasculitic changes, including central retinal artery and vein, was achieved prominently in the left eye. The study was conducted in accordance with the Declaration of Helsinki and written informed consent was obtained from the patient. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary.
Inclusion of ocular manifestations among the classification criteria for SLE would enable earlier establishment of the diagnosis and therapeutic interventions in some instances of SLE.
系统性红斑狼疮（SLE）是一种复杂的自身免疫性疾病，其特征为自身抗体产生、补体激活以及免疫复合物在组织和器官中的沉积。SLE可累及视觉系统的任何部位。虽然眼部表现并非SLE分类标准的一部分，但在高达三分之一的SLE患者中可观察到。它们在疾病发作时很少被报道。视网膜血管炎通常与活动性全身性疾病相关。由于其发生率较低，我们报告一例以急性坏死性视网膜血管炎为首发表现的SLE病例。
一名25岁白人女性因右眼眼底血管通透性异常伴血管周围水肿、中周部动脉分支闭塞伴染料渗漏、乳头黄斑区棉絮斑及左眼广泛出血导致视力逐渐快速下降而被转诊至风湿科门诊。颧部皮疹、关节痛以及抗核抗体、抗双链DNA抗体、抗核糖体P抗体和抗β2糖蛋白I抗体阳性且C4补体水平降低，同时狼疮带试验阳性，确诊为SLE。
采用大剂量甲泼尼龙、静脉注射免疫球蛋白和环磷酰胺进行积极的免疫调节治疗以抑制疾病活动，随后使用硫唑嘌呤作为维持治疗。
左眼视网膜血管炎病变，包括视网膜中央动静脉，显著改善且部分吸收。本研究遵循赫尔辛基宣言，并获得了患者的书面知情同意。因此，无需进行特殊伦理审查，也无需伦理批准。
将眼部表现纳入SLE分类标准可使某些SLE病例更早确诊并进行治疗干预。
