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波斯纳-施洛斯曼综合征

Posner-Schlossman Syndrome

作者信息

Okonkwo Ogugua N., Zeppieri Marco, Tripathy Koushik

机构信息

Eye Foundation Hospital

University Hospital of Udine, Italy

Abstract

In 1948, Posner and Schlossman published a series of 9 case reports of patients with the characteristic presentation of unique glaucoma, with open anterior angles of the anterior chamber, associated with unilateral acute elevations in intraocular pressure (IOP). This combination of symptoms later became known as Posner-Schlossman syndrome (PSS), a rare disease also called the glaucomatocyclitic crisis.  PSS is a unilateral ocular disease characterized by recurrent episodes of acute nongranulomatous anterior uveitis and increased intraocular pressure (IOP). This unique eye ailment manifests as a recurrent syndrome involving periodic rises in IOP. It differs from other glaucomas because it frequently involves mild-to-moderate anterior chamber inflammation; therefore, PSS is classified as inflammatory glaucoma. These episodes of IOP can eventually result in secondary chronic glaucoma. Initial attacks may go undetected because of the mild nature of the uveitis. PSS can be misdiagnosed as an acute angle-closure glaucoma (ACG) episode early in the disease because of the acute marked elevation in IOP and unnoticed inflammatory reaction in the anterior chamber.  During acute episodes, patients with PSS frequently report abrupt and intense eye pain, redness, blurred vision, and halos around lights. Between bouts, some people may also experience slight discomfort or a gritty sensation in their eyes. The majority of the time, these symptoms affect only one eye at a time. The exact prevalence of PSS is unclear. Although it can affect anyone of any age or gender, PSS mainly affects people between the ages of 20 and 50. It can be difficult to correctly quantify the syndrome's true incidence because of its sporadic nature. A thorough eye examination, which is primarily clinical, is necessary for diagnosing PSS. Ophthalmologists will measure the patient's IOP, look for inflammation in the anterior chamber, and rule out other possible causes of glaucoma. Diagnostic procedures like gonioscopy and imaging may be carried out to assess the angle of the eye's drainage system and rule out other types of glaucoma. The outlook for PSS over the long term is usually positive. If the illness is treated quickly, vision loss is uncommon, and increased IOP episodes typically go away on their own or without medical intervention. Repeated episodes of elevated IOP, however, have the potential to harm the optic nerve and cause permanent vision loss if they are not well managed. People with PSS must get continual monitoring and follow-up care to ensure their eye health is preserved. The clinical course of the disease tends to exhibit a variable pattern. Patients can have frequent attacks with shorter symptom-free intervals or less frequent attacks with more prolonged symptom-free intervals; in between episodes, patients tend to have a normal IOP. In either case, the symptoms are self-limiting, with spontaneous resolution occurring within days or weeks, even without treatment.  Management during an episode is aimed at controlling the IOP and minimizing intraocular inflammation. The main objective of PSS therapy is to control the acute increase in IOP and inflammation that occurs during episodes. Topical and systemic drugs, like oral glaucoma medications and anti-inflammatory eye drops, are frequently used as part of treatment. IOP management may occasionally require more intrusive techniques, such as laser therapy or surgery. Even though PSS is a well-known illness, further research is needed to grasp its underlying causes and risk factors fully. Prospects could involve the creation of more specialized treatments and enhanced diagnostic equipment to help with the early identification and treatment of PSS. Researching the genetic and immunological causes of the disease may also provide potential preventative measures. This review aims to summarize information on the diagnosis and treatment of PSS.

摘要

1948年,波斯纳(Posner)和施洛斯曼(Schlossman)发表了一系列9例具有独特青光眼特征表现患者的病例报告,这些患者前房角开放,伴有单侧眼压(IOP)急性升高。这种症状组合后来被称为波斯纳 - 施洛斯曼综合征(PSS),这是一种罕见疾病,也被称为青光眼睫状体炎危象。PSS是一种单侧眼病,其特征为急性非肉芽肿性前葡萄膜炎反复发作和眼压升高。这种独特的眼部疾病表现为一种反复发作的综合征,涉及眼压周期性升高。它与其他青光眼不同,因为它经常伴有轻度至中度的前房炎症;因此,PSS被归类为炎症性青光眼。这些眼压升高的发作最终可能导致继发性慢性青光眼。由于葡萄膜炎症状较轻,初始发作可能未被察觉。在疾病早期,PSS可能会被误诊为急性闭角型青光眼(ACG)发作,因为眼压急性显著升高且前房炎症未被注意到。在急性发作期间,PSS患者经常报告突然且强烈的眼痛、眼红、视力模糊以及灯光周围出现光晕。在发作间歇期,有些人眼睛可能也会感到轻微不适或有沙砾感。大多数时候,这些症状一次仅影响一只眼睛。PSS的确切患病率尚不清楚。尽管它可影响任何年龄或性别的任何人,但PSS主要影响20至50岁的人群。由于其散发性,很难正确量化该综合征的真实发病率。全面的眼部检查(主要是临床检查)对于诊断PSS是必要的。眼科医生会测量患者的眼压,检查前房是否有炎症,并排除其他可能导致青光眼的原因。可能会进行如前房角镜检查和影像学等诊断程序,以评估眼睛引流系统的角度并排除其他类型的青光眼。PSS的长期预后通常是良好的。如果疾病得到及时治疗,视力丧失并不常见,眼压升高发作通常会自行缓解或无需药物干预即可缓解。然而,如果眼压升高发作反复出现且未得到妥善控制,有可能损害视神经并导致永久性视力丧失。患有PSS的人必须接受持续监测和后续护理,以确保其眼部健康得到维护。该疾病的临床病程往往呈现出可变模式。患者可能发作频繁,无症状间隔较短,或者发作较少,无症状间隔较长;在发作间歇期,患者眼压往往正常。无论哪种情况,症状都是自限性的,即使未经治疗,也会在数天或数周内自行缓解。发作期间的治疗旨在控制眼压并尽量减少眼内炎症。PSS治疗的主要目标是控制发作期间出现的眼压急性升高和炎症。局部和全身药物,如口服青光眼药物和抗炎眼药水,经常被用作治疗的一部分。眼压管理偶尔可能需要更具侵入性的技术,如激光治疗或手术。尽管PSS是一种已知疾病,但仍需要进一步研究以全面了解其潜在病因和危险因素。未来可能涉及开发更专门的治疗方法和改进诊断设备,以帮助早期识别和治疗PSS。研究该疾病的遗传和免疫原因也可能提供潜在的预防措施。本综述旨在总结有关PSS诊断和治疗的信息。

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Posner-Schlossman glaucomatocyclitic crisis.波斯纳-施洛斯曼青光眼睫状体炎危象
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