■不同严重程度的眼科表现通常与全身性自身免疫性疾病有关。上眶裂综合征(SOFS)是一种罕见的颅神经病,影响通过上眶裂的神经,导致眼外和瞳孔表现的独特模式。我们报告了SOFS的共存,复发性多软骨炎(RP)和Sjögren综合征(SS)在52岁的女性谁提出了过去的病史的甲状腺功能减退,雷诺综合征,间歇性口干和1周化学恶化史,突增,复视,和疼痛的眼肌麻痹。
■经过全面的眼部检查,患者接受了对比CT检查,轨道的MRI,腰椎穿刺,和实验室调查。
■CT和MRI检查显示,在眶周皮下软组织和双侧眼球突出处有炎症,右眼内脂肪围绕神经的眶内和腔内节段,分别。腰椎穿刺和实验室检查显示炎症生物标志物升高,阴性传染性检查,并在考虑她的病史以及Schirmer阳性测试时在SS中统治。她开始服用高剂量类固醇,这导致了显著的改善;然而,治疗显示2型糖尿病,需要更快的类固醇锥度,在此期间,巩膜炎和眼肌麻痹的复发,导致利妥昔单抗开始输注。完成利妥昔单抗课程后,她转回类固醇治疗,成功减量,无事件发生.
■该病例以SOFS与RP/SS重叠综合征的罕见共存而著称,并强调了并发眼眶炎症综合征和自身免疫性疾病的管理。
UNASSIGNED: Ophthalmic manifestations of varying severity are often associated with systemic autoimmune conditions. Superior orbital fissure syndrome (SOFS) is a rare cranial neuropathy affecting nerves passing through the superior orbital fissure that causes a distinctive pattern of extraocular and pupillary findings. We report the coexistence of SOFS, relapsing polychondritis (RP) and Sjögren\'s syndrome (SS) in a 52-year-old female who presented with a past medical history of hypothyroidism, Raynaud\'s syndrome, and intermittent dry mouth and a 1-week history of worsening chemosis, proptosis, diplopia, and painful ophthalmoplegia.
UNASSIGNED: Following a comprehensive eye examination, the patient underwent a CT head with contrast, MRI of the orbit, lumbar puncture, and laboratory investigations.
UNASSIGNED: CT and MRI examination revealed inflammatory standing in periorbital subcutaneous soft tissues and bilateral exophthalmos with right intraconal fat stranding surrounding the intraorbital and intracanalicular segments of the nerve, respectively. Lumbar puncture and laboratory investigations revealed an elevation in inflammatory biomarkers, a negative infectious workup, and ruled in SS when considering her history alongside a positive Schirmer test. She was started on high-dose steroids, which led to significant improvement; however, treatment revealed type 2 diabetes, necessitating a faster steroid taper, during which there was a reoccurrence of scleritis and ophthalmoplegia, leading to the initiation of rituximab infusions. After completing rituximab course, she was transitioned back to steroid therapy and was successfully tapered without event.
UNASSIGNED: This
case is notable for the rare coexistence of SOFS with RP/SS overlap syndrome and highlights the management of concurrent orbital inflammatory syndrome and autoimmune diseases.