Cogan综合征

Cogan综合征是一种罕见的以非梅毒性角膜基质炎及听觉-前庭功能障碍为特点的综合征,病因尚不明确,可能为自身免疫性疾病.多在青年人发病,主要表现为非梅毒性角膜基质炎和听觉前庭功能障碍.分为典型性和非典型性.需与肉芽肿性多血管炎、类风湿性关节炎、系统性红斑狼疮等鉴别.治疗以糖皮质激素、免疫抑制剂为主.     

视网膜血管炎

视网膜血管炎是一复杂眼底病,眼底表现为血管外白鞘,常伴玻璃体炎或视网膜脉络膜炎、病因除视网膜局部血管炎症外可伴有全身血管炎,甚至恶性肿瘤,因而要求眼科医师具有更广泛的知识,从眼部病变进而分析出全身疾病,及时进行治疗及转诊,不仅挽救视力,还可能拯救生命。本文就我国常见的感染性、非感染性、眼部特发性血管炎的诊治做一简要描述,并对可能伴有全身血管炎的病例就如何从病史、体格检查、实验室及影像学检查着手,逐步深入分析,找出诊治方向。     

自体角膜瓣翻转联合板层角膜移植治疗边缘性角膜穿孔

目的探讨用自体角膜瓣翻转封闭角膜穿孔重建前房，并联合板层角膜移植治疗边缘性角膜溃疡穿孔的临床疗效。方法对11例11眼不同病因引起的边缘性角膜溃疡穿孔患者行自体角膜瓣翻转封闭角膜穿孔口重建前房后，再行板层角膜移植术(lamellar keratoplasty，LK)治疗，术后对并发症如层间积液、虹膜前粘连、继发青光眼以及视力和免疫排斥反应等进行观察。结果平均随访12个月(范围6～18)，所有患者植片透明，未发现层间积液，前房深度正常，无虹膜前粘连和继发青光眼。大于6个月随访，裸眼视力较术前平均提高4行，最佳矫正视力提高7行。平均散光4．3D。1例患者术后8个月出现基质型免疫排斥反应，抗排斥治疗后排斥反应控制。结论自体角膜瓣翻转联合LK是治疗边缘性角膜穿孔的有效方法。     

中国免疫相关性边缘性角膜病临床诊疗专家共识(2022年)

免疫相关性角膜病是由于全身或眼局部免疫功能异常,对自身正常角膜组织或变性角膜组织产生异常免疫应答反应,造成角膜和结膜结构和功能破坏,导致视力损伤的一类疾病,主要包括蚕蚀性角膜溃疡、睑缘炎相关角膜结膜病、类风湿性关节炎等全身免疫性疾病相关的边缘性角膜溃疡等。免疫相关性角膜病临床表现多样,病程反复迁延,误诊和漏诊率高,导致临床治疗预后不佳。为此,中华眼科学会眼科学分会角膜病学组针对边缘性免疫相关性角膜病的诊断、鉴别诊断及治疗原则进行充分讨论,结合国内外研究成果,达成共识性意见,以期对该类疾病的早期发现、早期干预和正确治疗发挥临床指导作用。     

板层角膜移植联合自体角膜瓣填垫治疗边缘性角膜穿孔

目的:探讨用自体角膜瓣封闭角膜穿孔,联合板层角膜移植治疗边缘性角膜溃疡穿孔的临床疗效。方法:对17例(17眼)不同病因引起的边缘性角膜溃疡穿孔患者行自体角膜瓣填垫及板层角膜移植,术后对植片愈合、虹膜前粘连、继发青光眼、视力、免疫排斥反应进行观察。结果:随访3～12(平均6)mo,所有患者植片透明,未见排斥反应,层间积液2例至术后1mo吸收,前房轴深正常,虹膜粘连于角膜穿孔处3例,无继发青光眼。随访>3mo,视力较术前提高12眼,无进步5眼。结论:板层角膜移植联合自体角膜瓣填垫是治疗边缘性角膜穿孔的有效方法。     

中国免疫相关性边缘性角膜病临床诊疗专家共识(2022年)

免疫相关性角膜病是由于全身或眼局部免疫功能异常,对自身正常角膜组织或变性角膜组织产生异常免疫应答反应,造成角膜和结膜结构和功能破坏,导致视力损伤的一类疾病,主要包括蚕蚀性角膜溃疡、睑缘炎相关角膜结膜病、类风湿性关节炎等全身免疫性疾病相关的边缘性角膜溃疡等。免疫相关性角膜病临床表现多样,病程反复迁延,误诊和漏诊率高,导致临床治疗预后不佳。为此,中华眼科学会眼科学分会角膜病学组针对边缘性免疫相关性角膜病的诊断、鉴别诊断及治疗原则进行充分讨论,结合国内外研究成果,达成共识性意见,以期对该类疾病的早期发现、早期干预和正确治疗发挥临床指导作用。     

新鲜多层羊膜移植治疗细菌性角膜溃疡的疗效观察

目的观察新鲜多层羊膜移植治疗细菌性角膜溃疡的临床疗效。方法对15例（15只眼）细菌性角膜溃疡进行新鲜多层羊膜移植术,术后观察病灶愈合情况及角膜透明程度。结果15只眼溃疡均达到临床治愈,角膜面残留不同程度的瘢痕。结论新鲜多层羊膜移植能促进角膜重新上皮化,抑制炎症和纤维化,抑制新生血管形成,是治疗角膜溃疡的有效方法。     

角膜溃疡多层羊膜移植术后早期组织病理学与临床分析

目的观察角膜溃疡多层羊膜移植术后羊膜与角膜早期组织病理学形态与临床特点。方法角膜溃疡患者23例(23眼),以多层羊膜填塞溃疡面,单层羊膜覆盖角膜表面,10-0线间断缝合于角膜缘。角膜组织行病理学检查,裂隙灯观察角膜溃疡变化,并观察术后并发症。结果 21例患者角膜溃疡愈合;2例患者溃疡穿孔再次行光学性角膜移植术,其中1眼继发青光眼。无其他并发症发生。HE染色羊膜呈染色均一结构,18d多层羊膜排列整齐,层次清晰,角膜基质内少量炎性粒细胞浸润,羊膜表面角膜上皮化;30d多层羊膜彼此融合,层次不清,羊膜与角膜基质界限模糊,少量新生血管形成,角膜厚度均一。结论多层羊膜移植后羊膜与角膜基质可有效整合,增加角膜厚度,阻止溃疡穿孔,抑制炎症,促进溃疡愈合。     

顽固性炎性眼病与风湿病的眼部受累

炎性眼病是一种顽固性、致盲性眼病。诸多风湿性疾病如脊柱关节炎、白塞病、类风湿关节炎、干燥综合征、复发性多软骨炎、抗中性粒细胞胞浆抗体相关血管炎可以导致眼部损害,而眼的各个部位均可受到风湿病的侵犯。炎性眼病与风湿病的眼部受累有诸多相似之处。以炎性眼病为首发表现或主要表现的风湿病眼病患者易被漏诊。对于反复发作的炎性眼病患者,要关注包括风湿性疾病及感染性疾病在内的全身疾病。基于炎性眼病与风湿病眼病的诸多共同点,应提高对风湿病眼部累及的认识,加强多学科协作诊治。（眼科,2020, 29： 90-92）     

深板层角膜移植术的临床应用及疗效

目的临床总结应用深板层角膜移植术治疗圆锥角膜,深及角膜后板层的角膜斑翳,角膜溃疡,边缘性角膜变性以及格子样角膜营养不良的治疗效果,探讨深板层角膜移植术的临床应用适应症。方法总结2001年1月至2005年5月深圳市眼科医院住院行深板层角膜移植的患者共14例(18只眼),其中圆锥角膜2例,(病毒性)角膜斑翳2例,(细菌性)角膜斑翳2例,真菌性角膜溃疡2例,细菌性角膜溃疡3例,边缘性角膜变性3例(5只眼),格子样角膜营养不良2例(4只眼)。记录术后半年复诊视力及角膜曲率,并统计角膜移植并发症的发生。综合评估该手术的临床适应症。结果有2例发生排斥反应,其中1例为病毒性角膜斑翳,1例为真菌性角膜溃疡。有3例原发病复发,2例真菌性角膜溃疡,1例病毒性角膜溃疡。术后视力半年时查0.5以上的有13例,0.1以上的有2例,0.1以下的有3例。结论深板层角膜移植对治疗未及角膜后弹力层的病变有效,但在治疗感染性角膜溃疡及病毒性角膜斑翳的治疗效果差,易发生原发病的复发及角膜移植排斥反应。     

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.     

引起周围溃疡性角膜炎的系统性疾病及眼局部治疗

周围溃疡性角膜炎（PUK）是一种罕见的周围角膜疾病，严重时可致失明，合并系统性疾病者甚至危及生命。因此，及时寻找病因进行早期诊断及干预性治疗有助于控制疾病进展，提高患者生活质量及延长寿命。近年来对于系统性疾病致PUK的研究取得了很多重要进展。本研究旨在阐述PUK相关的系统性疾病及眼局部治疗的研究进展。     

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.     

The cornea and systemic diseases

Multiple systemic diseases imply corneal involvement. Corneal pathology may also be a presenting sign or may herald a forthcoming relapse of chronic disease. The presence of ulcerative keratitis in patients with rheumatoid arthritis can be associated with systemic lethal vasculitis. Epithelial fragility of the cornea is correlated to the presence of diabetic retinopathy. Rarely, interstitial keratitis may be the presenting sign of sarcoidosis. Corneal involvement in oculomucocutaneous syndromes continue to present a therapeutic challenge. Infectious and possibly drug-induced corneal pathology is a recurrent problem in patients with AIDS.     

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.     

Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression

We performed a nonrandomized clinical trial comparing the ocular and systemic efficacy of cytotoxic immunosuppression with steroidal and nonsteroidal anti-inflammatory therapy in the care of 34 patients with rheumatoid arthritis who developed peripheral ulcerative keratitis and/or necrotizing scleritis. Nine of the 17 patients managed with conventional therapy died of a vascular-related event during the ten-year period of the study. In 13 of the 17 patients, the ocular inflammatory process progressed, and in 5 patients extraocular, although nonlethal, vasculitic lesions developed. One of 17 patients treated with long-term immunosuppressive therapy died during the ten-year follow-up period, and this death occurred after cytotoxic therapy was withdrawn. None of the patients on immunosuppressive regimens developed extraocular vasculitis while taking the drug, and none had progression of the ocular destructive lesion. The results of this study emphasize that the eye is a sensitive indicator for potentially lethal occult systemic vasculitis in patients with rheumatoid arthritis who develop peripheral ulcerative keratitis or necrotizing scleritis. Our mortality data strongly suggest that the use of cytotoxic drugs may alter favorably the general as well as the ocular prognosis in these patients.     

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.     

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.     

Peripheral ulcerative keratitis

Peripheral ulcerative keratitis (PUK) is an inflammatory condition of the peripheral cornea with hallmark features of epithelial defects and stromal destruction as a result of a complex interplay of factors including host autoimmunity and the peculiar anatomic and physiologic features of the peripheral cornea and environmental factors. PUK may be the result of local or systemic causes and infectious or noninfectious causes. Arriving at a specific etiological diagnosis requires a meticulous clinical workup that may include a battery of laboratory and radiological investigations. Management by a team of internists or rheumatologists and ophthalmologists and judicious use of immunosuppressive agents may yield favorable results minimizing adverse effects. We review current clinical knowledge on the diagnosis and management of PUK.     

Vasculitic peripheral ulcerative keratitis.

The onset of peripheral ulcerative keratitis in the course of a connective tissue disorder, such as rheumatoid arthritis, relapsing polychondritis, or systemic lupus erythematosus, may reflect the presence of potentially lethal systemic vasculitis. Moreover, peripheral ulcerative keratitis may be the first sign of systemic necrotizing vasculitis in patients with Wegener's granulomatosis, polyarteritis nodosa, microscopic polyangiitis, or Churg-Strauss syndrome. Although the exact pathogenesis of this severe corneal inflammation and destruction is not well understood, evidence points to a dysfunction in immunoregulation with immune complexes formed in response to autoantigens or to some unknown microbial antigen depositing in scleral and limbal vessels. These events lead to changes that are mainly responsible for the resulting tissue damage. In pauci-immune vasculitides positive for antineutrophil cytoplasmic antibodies, cell-mediated cytotoxicity may play an important role in the pathogenesis of peripheral ulcerative keratitis. Untreated systemic conditions such as those mentioned above may carry a grave prognosis for the eye and may also be life-threatening. Immunosuppressive therapy with corticosteroids and cytotoxic agents is, we believe, mandatory in the treatment of these multisystem disorders associated with vasculitic peripheral ulcerative keratitis.