Wu Connie M, Dunn James P, Sergott Robert C
Wills Eye Hospital, Philadelphia, PA, USA.
Uveitis Unit/Retina Division, Wills Eye Hospital, USA.
Am J Ophthalmol Case Rep. 2020 Sep 10;20:100913. doi: 10.1016/j.ajoc.2020.100913. eCollection 2020 Dec.
To describe a case of pronounced papillitis with diminished venous outflow and vitreous inflammation in a 50-year-old man who was later found to have clinical and serological manifestations of dermatomyositis.
A 50-year-old man presented with papillitis associated with venous congestion and intraocular inflammation. He was normotensive, not on medications, and without known heritable hypercoagulable or inflammatory disease. Review of systems revealed axial and proximal muscle pain involving the lower back, hip, and thigh, and he developed a transient rash of the scalp. His evaluation including infectious, hypercoagulable, and inflammatory etiologies was negative except for a significantly elevated Mi-2 antibody titer. Treatment with intravenous and oral steroids improved the papillitis, visual acuity and visual field deficit.
Significant papillitis and retinal venous stasis with intraocular inflammation may be associated with dermatomyositis.
Rapidly progressive optic disc edema with associated inflammation and venous stasis requires a broad work up for infectious, hypercoagulable, and autoimmune etiologies for targeted therapy and visual preservation. To the best of our knowledge, this may be the first reported clinical presentation of dermatomyositis manifesting initially with papillitis and retinal venous congestion, based upon elevated Mi-2 antibodies and additional clinical features.
描述一例50岁男性患者,其患有明显视乳头炎,伴有静脉回流减少和玻璃体炎症,该患者后来被发现有皮肌炎的临床和血清学表现。
一名50岁男性患者出现与静脉充血和眼内炎症相关的视乳头炎。他血压正常,未服用药物,也没有已知的遗传性高凝或炎症性疾病。系统回顾显示轴向和近端肌肉疼痛,累及下背部、臀部和大腿,并且他头皮出现了一过性皮疹。除了Mi-2抗体滴度显著升高外,对其包括感染性、高凝性和炎症性病因的评估均为阴性。静脉和口服类固醇治疗改善了视乳头炎、视力和视野缺损。
明显的视乳头炎和视网膜静脉淤滞伴眼内炎症可能与皮肌炎有关。
快速进展的视盘水肿伴相关炎症和静脉淤滞需要对感染性、高凝性和自身免疫性病因进行广泛检查,以进行针对性治疗和保护视力。据我们所知,基于Mi-2抗体升高和其他临床特征,这可能是首例最初表现为视乳头炎和视网膜静脉充血的皮肌炎临床病例报告。