与巩膜炎相关的边缘性角膜病变的眼部特征及疾病相关因素

目的：评价与巩膜炎相关的边缘部角膜病变的眼部病变特点及与全身性疾病的相关性、病变分型以及巩膜炎同时合并边缘部角膜病变患者的眼部及全身愈后。方法：回顾单纯巩膜炎患者125例及合并周边部角膜病变的巩膜炎患者47例；回顾合并周边部角膜病变的巩膜炎患者的角膜病变分型：周边变薄、基质性角膜炎及周边部溃疡性角膜炎型（PUK）；回顾及比较巩膜炎患者合并及不合并周边部角膜病变对眼部及全身愈后的影响。结果：合并周边部角膜病变的患者比单纯性巩膜炎的患者更多地表现为坏死性巩膜炎（57％，P＜0．001），视力减退（81％，P＜0．001），前部葡萄膜炎（62％，P＜0．002), 角膜濒于穿孔（62％，P＜0．001），合并严重的特异性全身性疾病（87％，P＜0．001）。合并PUK患者的眼部及全身性愈后最差。在24例合并PUK的患者中，有16例（67％）为坏死性巩膜炎（P＝0．02），这些患者均有致死性全身疾病，而且全部患者角膜濒于穿孔。结论：合并周边部角膜病变尤其是PUK的巩膜炎患者，预示其愈合不良。     

玻璃体切除术后白内障发生或进展的机制及其影响因素

玻璃体切除术(pars plana vitrectomy，PPV)自1972年由O''Malley提出后已成为眼科治疗史的一大革命，打破了既往的手术禁区，为无数玻璃体视网膜疾病的患者带去了希望。随着玻璃体切除术的不断发展，其安全性及有效性增加，手术适应证扩大，目前玻璃体切除术已成为治疗眼后段疾病最常用的手术方式。虽然玻璃体切除术后并发症越来越少，但白内障的发生或进展仍为PPV术后最常见的高发并发症，严重影响患者术后视力及眼底的观察，且目前尚无有效办法预防，白内障摘除手术为其主要治疗方法，严重增加了患者的负担。PPV术后白内障进展的影响因素众多，对其发病机制有多种假说，包括晶状体周围氧分压增高、玻璃体正常结构的破坏、光毒性等，但尚无定论。本文将从PPV术后白内障发生或进展的发生率、发生机制、影响因素几方面对玻璃体切除术后白内障发生或进展进行综述，以期为其后续的研究及临床预防和治疗提供参考。     

眼科住院患者全身系统性危重疾病的临床分析

目的：通过分析眼科住院患者中全身系统性疾病的发生情况,掌握眼病患者全身危重疾病的特点,为医疗安全提供保障。方法：对2007-06/2010-12在西安北方医院眼科住院的2 016例眼病患者中发生全身系统性危重病变（包括急性新发病变和慢性疾病急性发作）的病历资料进行回顾性分析。结果：既往有全身系统性疾病史者1644例（81.55%）。住院期间发生全身危重病变者36例（1.79%）,包括循环系统疾病24例（高血压急性发病致高血压危象19例、急性心肌梗塞3例、急性低血压2例）、神经系统疾病4例（脑出血2例、急性脑梗塞2例）、消化系统疾病3例（急性胃肠炎2例、急性消化道出血1例）、泌尿系统疾病2例（尿路结石致急腹症2例）以及重症药物过敏3例。所有患者经及时治疗后全身病情好转,眼病病情稳定。结论：眼科住院患者中老年人居多,既往有全身系统性疾病史者比例高,易发生以循环系统和神经系统为主的全身危重疾病。只有高度重视对系统性危重疾病全面评估,早期诊断,妥善治疗,才能安全有效地完成眼病治疗。     

干燥综合征相关型干眼症的临床诊疗进展

干燥综合征(SS)是一种系统性自身免疫类疾病,其主要特征是免疫细胞浸润并破坏各外分泌腺体,影响机体其他各器官的系统功能。当该类疾病累及眼部时,极易引发干眼症,出现眼干、畏光等眼部不适的症状。目前,SS相关型干眼症的发病机制尚不明确,也无统一的诊断及治疗标准,至今仍未找到能够彻底根治此类疾病的治疗办法。此类疾病的治疗目标主要是控制病情、缓解症状及减少并发症的发生。近年来,随着免疫系统疾病和干眼症治疗等相关研究的不断深入,SS相关型干眼症的治疗也有了很大进展。本文中笔者重点回顾目前能够应用于SS相关型干眼症的临床诊疗方法,整理近年来有关此类疾病诊断及治疗取得的研究进展,以期为SS相关型干眼症的临床诊疗提供参考。     

系统性硬化症患者视神经萎缩一例

进行性系统性硬化症以往称为硬皮病，是一种全身性结缔组织疾病，眼部常受累，可有不同的临床表现，但出现视神经萎缩者罕见。我们曾于2003年收治1例系统性硬化症患者，在病变进展过程中，出现进行性双眼视力下降和视神经萎缩。现报告如下。     

玻璃体形态的影像学研究进展

了解玻璃体形态在生理和病理状态下的影像特点，对研究玻璃体视网膜病变发病机制及相关疾病的早期诊断治疗有重要意义。本着重介绍了近年来国外有关玻璃体影像学研究的进展及应用前景。     

基因治疗在神经眼科中的应用

目的应用基因技术治疗眼病的可行性备受国内外学者关注，并且通过利用动物模型进行了一系列实验取得很大进展，在治疗神经眼科疾病方面有临床应用前景。本文就应用该技术治疗视神经疾病的可行性及其研究现状进行综述。     

纳米给药系统在眼底疾病治疗中的研究进展

眼底疾病是全球导致患者视力严重受损甚至丧失的最主要原因，由于各种生理屏障使治疗药物难以进入眼底，严重影响了眼底疾病的治疗。纳米给药系统具有纳米级尺寸、表面积/体积比值大的特点，可以负载不同理化性质的治疗药物，同时可以修饰各种表面活性物质，能够提高药物的溶解度以及生理屏障的穿透性、保护生物药物不被降解而提高药物安全性与生物利用度、并将治疗药物递送到特定的眼部靶点，具有巨大的治疗潜力。目前越来越多围绕利用纳米材料的优势治疗眼底疾病的研究已经展开并引起广泛关注，包括神经退行性病变、眼底新生血管、眼内炎及眼底肿瘤等。本综述通过分析不同给药途径在眼底疾病治疗中面临的挑战及障碍，对近年开展的相关研究中常见纳米药物递送系统的理化性质、在常见眼底疾病治疗研究的进展、优势、局限性及未来发展方向进行了总结。     

眼部组织细胞增生症诊疗进展

眼部组织细胞增生症是一组罕见的成人及儿童均可发病的异质性疾病，其临床及影像学表现无特异性，临床上该类疾病的诊疗存在巨大困难。随着对组织细胞增生症分子机制的研究，BRAF^V600E突变基因的发现使人们对组织细胞增生症有了进一步的认识，也使多数患者能够得到针对性的治疗。诊断的金标准发展为组织病理活检联合突变基因的检测，治疗方式不再是单一的手术切除治疗，靶向治疗对MAPK-ERK通路中突变基因阳性的患者也有极好的疗效。但是眼部组织细胞增生症类型众多且常常涉及多个系统，临床表现多样，对其早期、正确地诊断仍面临挑战。因此本文就近年眼部组织细胞增生症的诊疗进展进行文献复习，总结眼部组织细胞增生症的临床病理特征并对最新的治疗进展进行综述，旨在提高临床医生对该疾病的诊疗水平。     

系统性红斑狼疮眼底病变发展的临床观察

目的 探讨系统性红斑狼疮(SLE)眼底病变发展的临床特征.方法 回顾分析我院就诊随访的SLE视网膜病变患者26例,总结该病眼底病变发展转归的临床特点.结果 SLE视网膜病变的发展与全身病情的进展密切相关.初期多表现为程度不同的棉绒斑,血管迂曲扩张,小片出血.随着病情的进展,眼底表现为血管炎性反应、广泛出血、血管阻塞.病变进展到晚期,视网膜大片血管闭塞呈白线状、视神经萎缩苍白,视功能严重受损.结论 眼底检查不但能检测SLE眼底损害的进展,也为全身疾病的治疗及预后评估提供参考依据.     

风湿病相关的边缘性角膜溃疡

边缘性角膜溃疡是指近角膜缘处角膜基质的半月形破坏性病变,伴随角膜上皮缺损、基质变性,以及基质炎性细胞浸润。风湿病常累及眼部,类风湿关节炎、Wegener’s肉芽肿、结节性多动脉炎、复发性多软骨炎、系统性红斑狼疮等可发生边缘性角膜溃疡。边缘性角膜溃疡可能是系统性血管炎的首发表现,也可能预示着某些风湿病进入了严重的血管炎阶段。眼科医师了解这些风湿病的全身及眼部表现,做出正确及时的诊断,并协同风湿科医师进行全身性治疗,对于挽救患者的生命和眼睛都是非常重要的。就边缘性角膜溃疡的病因、临床表现及治疗的研究进展进行综述。     

Role of ocular matrix metalloproteinases in peripheral ulcerative keratitis

AIM: Peripheral ulcerative keratitis (PUK) is an ocular manifestation of rheumatoid arthritis and other similar systemic diseases. The purpose of this inquiry was to investigate the involvement of matrix metalloproteinases (MMPs) in the induction and/or maintenance of PUK. METHODS: Substrate gel electrophoresis was used to characterise the MMP activities secreted by primary cultures of keratocytes derived from normal and perforated pathological corneal specimens, and those present in tears of normal subjects and patients with PUK. Substrate specificity and the in vivo activity status of the secreted MMPs was assessed by SDS-polyacrylamide gel electrophoresis of standard collagens incubated in the presence or absence of the various enzyme preparations. RESULTS: In addition to MMP-2 of M(r) 66,000, cultured keratocytes derived from perforated corneas of patients with PUK abnormally produce the MMP-2 of apparent M(r) 62,000. Other MMPs and in particular MMP-9 of M(r) 92,000, also occur in the tears of these patients. Their visualisation on substrate polyacrylamide gels correlated with clinical manifestations of disease activity; during periods of disease quiescence they were barely detectable. The steroid prednisolone, frequently used in systemic therapy, had no effect on the in vitro activity of MMP-2, or on its production by cultured corneal keratocytes. Although the in vitro activity of MMP-2 was inhibited by both Cu(2+) and Zn(2+), Cu(2+) apparently induced the keratocytes to produce activated enzyme and Zn(2+) irreversibly inhibited their production of MMP-2. CONCLUSION: Overexpression of corneal MMP-2 and tear film MMP-9 are characteristic features of patients with PUK and their activation may be a crucial facet of disease initiation or progression. Although effective in systemic therapy for PUK, prednisolone had no direct control over corneal MMP-2 production or activity. Zn(2+) on the other hand inhibited both MMP-2 production and MMP-2 activity and may, therefore, be of therapeutic value if suitably formulated and used in conjunction with systemic steroid treatment.     

A rare case of peripheral ulcerative keratitis associated with Behçet’s disease

We report a rare case of peripheral ulcerative keratitis (PUK) associated with Behçet’s disease. A 34-year-old female presented with right ocular pain persisting for 8 months. The patient had been treated for previously diagnosed Behçet’s disease. The corrected visual acuity was 20/32 in the right eye. Ocular examination revealed mild conjunctival hyperemia and a deep ulcerative lesion with perilesional haziness in the peripheral cornea. Autoimmune screening and corneal culture were negative. One month following treatment with topical and systemic immunosuppressants, the symptom had resolved. Slit-lamp examination showed a healed corneal lesion, and visual acuity improved to 20/20. Despite its rare occurrence, PUK can develop in patients with Behçet’s disease. Therefore, patients presenting PUK require examination for Behçet’s disease using a systemic evaluation.     

An analysis of therapeutic decision making regarding immunosuppressive chemotherapy for peripheral ulcerative keratitis

We reviewed our experience in the management of 47 patients (61 eyes) with peripheral ulcerative keratitis (PUK) to establish guidelines for appropriate indications to consider institution of systemic chemotherapy. Fifty-three percent of patients had a systemic disease as the etiology of PUK; one fourth of these were newly diagnosed as a result of meticulous history taking. The histologic demonstration of vasculitis in ocular tissue was the crucial step in deciding on chemotherapy in more than half of our patients. The presence of scleritis was highly associated with active vasculitis. Twelve of 14 patients with bilateral PUK required chemotherapy. Recommendations for an approach to therapy of PUK are presented.     

Tear film MMP accumulation and corneal disease

BACKGROUND/AIMS: Matrix metalloproteinases (MMPs) accumulate in the tears of patients with active peripheral ulcerative keratitis (PUK) but it is unknown whether these enzymes have a central role in disease progression. The aims of the present investigation were to determine the source of these enzymes and to ascertain whether their accumulation in tears is a phenomenon specific to PUK or a general feature of other anterior segment diseases. METHODS: The experimental samples were obtained from the culture media of conjunctival and corneal epithelial cells, from fractionated blood plasma and leucocytes of healthy subjects and patients with rheumatoid arthritis, and from the tears of healthy subjects and patients with a variety of anterior segment diseases. The MMPs of all samples were visualised by zymography and tear samples were assayed using nitrophenol acetate and an MMP-9 susceptible quenched fluorescent peptide as substrate. RESULTS: The major MMPs that accumulate in the tears of patients with rheumatoid arthritis with active ocular disease are MMP-9 and a species of M(r) 116,000. By comparing the zymographic activity profiles of the gelatinases present in the samples obtained, it was deduced that the main source of these MMPs was granulocytes. Their accumulation in tears was not unique to patients with PUK; detectable amounts of the enzymes also occurred in the tears of patients with keratoconus with associated atopic disease, patients undergoing treatment for herpetic eye disease, and patients with systemic and non-systemic dry eye disease. CONCLUSION: The MMPs that accumulate in tears are mainly derived from granulocytes. This may be effected by autoimmune diseases that involve ocular tissue or by ocular diseases that induce an inflammatory response.     

Corneal perforation due to severe peripheral ulcerative keratitis in Crohn disease

PURPOSE: To describe corneal perforation secondary to severe peripheral ulcerative keratitis (PUK) in a patient with Crohn disease. METHODS: Interventional case report. RESULTS: A 72-year-old male with biopsy-proven Crohn disease presented with reduced vision, PUK, and corneal perforation in the right eye. Despite initial treatment with intravenous methylprednisolone and a conjunctival flap, a tectonic sectorial penetrating keratoplasty was required to preserve the globe and achieve a good visual result. CONCLUSIONS: Crohn disease may be associated with severe PUK leading to corneal perforation. Tectonic corneal grafting combined with treatment of the underlying systemic disease was associated with a favorable outcome.     

Rituximab in rheumatoid arthritis-associated peripheral ulcerative keratitis

CLINICAL CASE: We report two cases of patients affected by longstanding rheumatoid arthritis who developed a severe form of peripheral ulcerative keratitis (PUK). Neither of them had an optimal biological and clinical control of their systemic illness despite being treated with several disease-modifying antirheumatic drugs (DMARDs) and biologic therapy. High-dose systemic corticosteroids were given to treat the PUK without any success. Rituximab resulted in a favourable response with resolution of the corneal lesions and optimal control of their systemic illness. DISCUSSION: Rituximab may be an additional tool to arrest progressive rheumatoid arthritis-associated PUK that is refractory to other drugs.     

Peripheral ulcerative keratitis due to a 'long lost' hard contact lens

Peripheral ulcerative keratitis (PUK) is a disorder consisting of a crescent-shaped destructive inflammation of the perilimbal corneal stroma. PUK can occur in a variety of ocular and systemic conditions including infections, lid abnormalities, dermatological disorders and connective tissue disorders. We present a case of PUK associated with a hard contact lens (CL) retained in the superior fornix for over 16 years. After removal of the embedded CL, a superior forniceal conjunctival pedicle graft was performed to prevent corneal perforation. The patient was managed postoperatively with a combination of topical steroids and antibiotics. The use of systemic immunosuppressive therapy was not necessary. Micro-trauma and micro-keratitis may have occurred as a result of the mechanical effect of the CL but if this was the sole mechanism, one would expect presentation at a much earlier date. We discuss the pathogenetic mechanisms which may have contributed to the development of this ulceration. This report highlights the importance of lid eversion when examining patients with anterior segment pathology.     

Peripheral ulcerative keratitis associated with hideradenitis suppurativa

Peripheral ulcerative keratitis (PUK) is a noninfectious inflammation and ulceration of the peripheral cornea. It may be idiopathic--i.e., Mooren's ulcer--or it may be associated with a variety of systemic disorders, including many collagen vascular diseases. We present a patient who had bilateral peripheral infiltrative and ulcerative keratitis in association with severe hideradenitis suppurativa (HS), a suppurative and cicatricial disease of the apocrine glands of the skin. The peripheral ulcerative keratitis was unresponsive to all routine treatment modalities, but it finally responded to immunosuppression by cyclophosphamide, given orally. To the best of our knowledge, there is only one report in the dermatology literature citing the association of hideradenitis suppurativa and interstitial keratitis.     

Tectonic keratoplasty for peripheral ulcerative keratitis

Peripheral ulcerative keratitis (PUK) is a destructive, inflammatory process that can lead to corneal perforation and visual loss. Successful control of PUK has been reported with conjunctival resection, cyanoacrylate adhesive, and systemic immunosuppression. Cases with impending or actual corneal perforation may require more extensive surgery, including lamellar or penetrating keratoplasty, to maintain the integrity of the globe. We report on 17 eyes of 14 patients with PUK that required tectonic keratoplasty because of progressive ulceration. Surgery with concomitant immunosuppression preserved the eyes in all but two cases, and 8 of 17 eyes maintained or improved preoperative visual acuity. Six eyes had final visual acuities of 20/200 or better. This therapeutic strategy can preserve eyes that might otherwise be lost to progressive inflammation.