Wegener's granulomatosis: the relationship between ocular and systemic disease

OBJECTIVE: Wegener's granulomatosis (WG) is an etiologically obscure entity with multiple systemic manifestations. Ocular involvement is present in up to 58% of patients with WG. We describe a series of patients with ocular manifestations of WG to evaluate the presence of ocular lesions in the setting of systemic WG and to determine the value of ocular inflammation in the diagnosis of WG. METHODS: A computerized database was used to generate a list of patients cared for in the Ocular Immunology Service of the Massachusetts Eye and Ear Infirmary during the 10 year period 1988-98 with a diagnosis of Wegener's granulomatosis. A detailed chart review was undertaken to determine demographic characteristics, history, initial manifestation of WG, initial ocular presentation, biopsy results, laboratory testing results, treatment, total followup period, and final outcome. RESULTS: Forty-seven patients diagnosed with WG were identified. Twenty-eight were women (59.6%), 19 were men (40.4%). The average age was 53 years (range 18-90). Patients were divided into 4 groups. Group I included 27 patients (57.4%) who had systemic disease first and who subsequently developed an ocular lesion. Group II included 3 patients (6.3%) who had ocular inflammation first and who then subsequently developed systemic manifestations of WG. Group III included 3 patients (6.3%) who presented due to ocular symptoms but, on initial evaluation by us, were found to have occult systemic manifestations consistent with WG or biopsy evidence of WG. Group IV included 14 patients (30%) with ocular lesions and no history or presence of systemic disease at their last followup visit. CONCLUSION: Ocular inflammation can occur with or without obvious systemic manifestations of WG. It may represent the first sign of WG that enables the knowledgeable physician to diagnose this potentially lethal disease.     

炎症性肠病眼部表现的研究进展

炎症性肠病(inflammatory bowel disease,IBD)是一种胃肠道的慢性非特异性炎性病症,近年来,我国的发病率逐渐升高,已成为消化科的主要疾病之一.IBD的肠外表现复杂多样,涉及多个系统.眼部表现作为其中的一个类型较少见,可严重影响患者的生活质量,由于发病率低、缺乏特异性表现,在临床工作中常被忽略....     

梅毒的眼部表现

梅毒螺旋体可侵犯眼的各层组织,其临床表现可因梅毒螺旋体累及眼的不同组分而异,其中以葡萄膜炎最为常见。该文就各期梅毒中主要的眼部表现做一综述。     

Ocular manifestations of primary systemic vasculitis

A variety of ophthalmic manifestations can occur in patients who have systemic vasculitides and may be the presenting feature. Ocular involvement is frequently found and can result in significant morbidity, even in blindness. Early diagnosis and treatment may improve visual outcome. The management is challenging and requires a multidisciplinary approach. Treatment of ocular manifestations and systemic disease usually follows the same path. The mainstay of treatment is the use of systemic corticosteroids, usually combined with steroid-sparing immunosuppressive drugs. New, promising, emerging therapies rely on the development of biologic agents, which seem useful in the ocular manifestations of systemic vasculitides.     

Necrotizing retinitis in a patient with syphilis: A case report

Rationale:Ocular syphilis varies widely in presentation and should be considered in all patients with posterior uveitis. Necrotizing retinitis is a rare manifestation of ocular syphilis and mimics ARN.Patient concerns:We report a male patient who presented with bilateral dense vitritis obscuring fundus details similar to ARN, as a rare reported manifestation of syphilis, who was initially given intravitreal ganciclovir.Diagnosis:After the results for herpes viral PCR disclosed negative, the diagnosis of syphilitic necrotizing retinitis was made based on positive RPR.Intervention and Outcomes:With the clinical diagnosis of ocular syphilis, treatment with intravenous penicillin was promptly initiated. His visual acuity improved to 20/100 in the right eye and still light perception in the left. Pars plana vitrectomy with silicon oil tamponade was performed in his left eye.Lessons:Ocular syphilis varies widely in presentation and should be considered in all patients with posterior uveitis. However, whenever ARN is clinically suspected, empiric treatment against herpetic viruses should be promptly administered while awaiting further infectious disease study results. Recognition of syphilitic retinitis and prompt initiation of intravenous penicillin is of critical important for clinicians.     

Ocular infections in sub‐Saharan Africa in the context of high HIV prevalence

Healthy eyes and good vision are important determinants of populations' health across the globe. Sub‐Saharan Africa is affected by simultaneous epidemics of ocular infections and human immunodeficiency virus (HIV). Ocular infection and its complications, along with cataract and ocular trauma, are common conditions in this region with great impact on daily life. In this review, we discuss the epidemiology, clinical manifestations and microbial aetiology of the most important infectious ocular conditions in sub‐Saharan Africa: conjunctivitis, keratitis and uveitis. We focus specifically on the potential association of these infections with HIV infection, including immune recovery uveitis. Finally, challenges and opportunities for clinical management are discussed, and recommendations made to improve care in this neglected but very important clinical field.     

The eye as a window to rare endocrine disorders

The human eye, as an organ, can offer critical clues to the diagnosis of various systemic illnesses. Ocular changes are common in various endocrine disorders such as diabetes mellitus and Graves' disease. However there exist a large number of lesser known endocrine disorders where ocular involvement is significant. Awareness of these associations is the first step in the diagnosis and management of these complex patients. The rare syndromes involving the pituitary hypothalamic axis with significant ocular involvement include Septo-optic dysplasia, Kallman's syndrome, and Empty Sella syndrome all affecting the optic nerve at the optic chiasa. The syndromes involving the thyroid and parathyroid glands that have ocular manifestations and are rare include Mc Cune Albright syndrome wherein optic nerve decompression may occur due to fibrous dysplasia, primary hyperparathyroidism that may present as red eye due to scleritis and Ascher syndrome wherein ptosis occurs. Allgrove's syndrome, Cushing's disease, and Addison's disease are the rare endocrine syndromes discussed involving the adrenals and eye. Ocular involvement is also seen in gonadal syndromes such as Bardet Biedl, Turner's, Rothmund's, and Klinefelter's syndrome. This review also highlights the ocular manifestation of miscellaneous syndromes such as Werner's, Cockayne's, Wolfram's, Kearns Sayre's, and Autoimmune polyendocrine syndrome. The knowledge of these relatively uncommon endocrine disorders and their ocular manifestations will help an endocrinologist reach a diagnosis and will alert an ophthalmologist to seek specialty consultation of an endocrinologist when encountered with such cases.     

Ocular manifestations of vasculitis.

Systemic necrotizing vasculitis is characterized by inflammation of blood vessels and often affects the eyes. Ocular manifestations of vasculitis may involve any part of the eye or orbit. The frequency of ocular involvement generally is dependent on the size and type of blood vessels affected by the vasculitis. This article reviews the major types of ocular inflammation and then addresses the ocular manifestations of specific systemic vasculitides.     

Syphilis iridocyclitis in a patient with type 1 diabetes

We present a rare cause of iridocyclitis in a patient with vitiligo and type 1 diabetes who showed poor metabolic control, and suffered from remitting fever, weight loss, fatigue, diffuse arthralgias and reduced visual acuity. Mild systemic symptoms coupled with increased cholestasis enzymes, insulin resistance, mild inflammation and a functioning adrenal gland focused our clinical work‐up on granulomatous causes of iridocyclitis. Specific tests confirmed syphilis, with no involvement of the central nervous system. Ocular syphilis, despite being unusual, can be the only manifestation of the disease. The work‐up of any unexplained ocular inflammation should include testing for syphilis so as to not delay the diagnosis.     

Ocular manifestations in adult T-cell leukemia/lymphoma

 Ocular manifestations of adult T-cell leukemia/lymphoma (ATL) are rare events. However, several ocular lesions which resulted from human T-cell leukemia virus type I (HTLV-I) infection have been reported, including direct infiltration of ATL cells, cytomegalovirus retinitis, and HTLV-I-associated uveitis (HAU). The aim of this study was to characterize ocular involvement in ATL and to correlate these lesions with HTLV-I proviral DNA integration. Three patients with acute-type ATL and ocular lesions were evaluated hematologically and ophthalmologically. Analysis of HTLV-I proviral DNA was carried out with a standard Southern blot technique using DNA from abnormal lymphocytes in peripheral blood. Two patients developed intraocular lesions located within intermediate and/or posterior segments which were caused by infiltration of ATL cells. Ocular lesions in one patient, which were localized to the anterior-intermediate segment, closely resembled those of HAU. Analysis of HTLV-I proviral DNA revealed multiple integrations in all three patients. The present study indicated heterogeneity in ocular manifestations of ATL. Multiple HTLV-I proviral DNA integrations may be associated with intraocular involvement in this disease. Received: 30 October 1996 / Accepted: 23 January 1997     

Ocular cicatricial pemphigoid: Manifestations and management

Ocular cicatricial pemphigoid (OCP) is a chronic, progressive cicatrizing inflammatory disease of presumed autoimmune etiology affecting the mucous membranes and skin. It has sight-threatening ocular manifestations and potentially life-threatening extraocular manifestations. The ocular signs include: chronic cicatricial conjunctivitis, progressive conjunctival fibrosis, and corneal epitheliopathy. Systemic immunomodulatory therapy is the treatment of choice for controlling disease activity and limiting progression, given the systemic nature of the disease and the poor efficacy of current local or topical therapies. Systemic cyclophosphamide with short-term adjunctive high-dose prednisolone is the preferred treatment for severe and/or rapidly progressing OCP. Oral low-dose weekly methotrexate is a useful first-line treatment for mild-to-moderate OCP. The management of OCP requires a multidisciplinary approach to optimize the care of these patients.     

Ocular complications of childhood rheumatic diseases: Nonuveitic inflammatory eye diseases

Ocular involvement is common in pediatric rheumatologic diseases, supporting the concept that these conditions do not manifest in isolation but are components of a multisystem inflammatory process. It remains unclear why the eye and its adjacent tissues become a target during paninflammatory disease. Pediatric rheumatologists must recognize ocular disorders, as these conditions significantly concern the treatment team managing serious cases of inflammatory eye disease. Close collaboration between the treating rheumatologist and ophthalmologist is required to prevent potentially devastating outcomes. Therapeutic interventions, such as topical steroids, systemic immunosuppressants, and biologics, must balance the necessity of controlling ocular inflammation and treatment-related adverse effects. This article—the second in a series on ocular complications of childhood rheumatic diseases—reviews the presentation and management of the more common nonuveitic inflammatory ocular manifestations of childhood rheumatologic disease.     

Syphilitic panuveitis and asymptomatic neurosyphilis: a marker of HIV infection

Co-existing human immunodeficiency virus (HIV) infection can alter the course and presentation of syphilis. Severe ocular manifestations and accelerated natural course of syphilis along with neurosyphilis may be associated with HIV infection. A 30-year-old man is described in whom syphilitic panuveitis and asymptomatic neurosyphilis served as a marker for HIV infection.     

Ophthalmologic manifestations of systemic vasculitis: report of six cases and review of the literature

PURPOSE: Study of characteristics of ocular involvement in systemic vasculitis. METHODS: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. RESULTS: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. CONCLUSION: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.     

Ocular manifestation of systemic lupus erythematosus

The purpose of this study is to determine the ocular manifestations of systemic lupus erythematosus and its correlation with the disease activity. Fifty-two lupus patients and 20 healthy controls were included in this study. All patients have undergone complete rheumatological, neurological, and ophthalmic examination including visual acuity, slit-lamp examination of the anterior segment, and dry eye evaluation using Rose Bengal stain and Schirmer test. Fundus examination and fundus photography were done to the suspected cases. Eighteen patients (34.6%) had ocular lesion, from which only 13 (25%) patients were symptomatic. Keratoconjunctivitis was the most common ocular lesion. There was a highly statistically significant difference between patients and controls as regarding all ocular lesions (***P > 0.0001). There was good correlation between disease activity index and presence of ocular lesion. Ocular manifestations are common in SLE patients. Lupus retinopathy may reflect systemic, particularly CNS, vascular damage.     

眼部微孢子虫病研究进展

由微孢子虫所引起的眼部疾患具有两种不同的临床类型：一种是角膜基质型,常见于免疫功能正常的健康人;另一种为上皮型点状角膜结膜炎,多发生于免疫功能低下的患者。这两种不同的临床表现与病原体的种属及患者的免疫状况有关。本文就眼部微孢子虫病的研究进展进行综述。     

Ocular Syphilis: a Clinical Review

While ocular syphilis is not a new phenomenon, recent increased rates of new diagnoses, especially in human immunodeficiency virus (HIV)-infected persons and men who have sex with men, have sparked a new interest in an old disease. This article will review the clinical presentation, diagnosis, and treatment of ocular syphilis, and provide guidance on management.     

系统性红斑狼疮相关性眼部损害

目的 了解系统性红斑狼疮(SLE)相关性眼部损害的发生率、表现形式以及与SLE疾病活动性、其他系统损害和自身抗体的相关性.方法 回顾性分析我科2008年1月至12月期间住院SLE患者152例,其中有眼部损害者34例为实验组,另选取其中无眼部损害的SLE 48例为对照组,详细记录患者主要临床症状、器官受累情况、眼部体征、外周血自身抗体以及SLE疾病活动指数(SLEDAI).结果 152例患者中34例出现眼部损害,发生率22.4%.其中女性31例,平均年龄35.6岁,平均SLEDAI评分(18±7)分.16例(47%)患者眼部损害出现在SLE病程1年以内.最常见的眼部表现是眼底病变23例(68%),其中13例视网膜病变(11例双眼,2例单眼),9例视网膜血管病变(7例双眼微血管病变,2例单眼视网膜中央动静脉血管栓塞),1例双眼脉络膜萎缩,其他眼部表现包括8例Schirmer's试验异常(6例双眼,2例单眼),1例视神经病变(单眼),4例视野缺损(2例双眼,2例单眼),1例巩膜炎(双眼),1例虹膜睫状体炎(单眼),1例青光眼(单眼),4例角膜炎(3例双眼,1例单眼),3例结膜炎(2例双眼,1例单眼),1例睑缘炎(双眼).实验组与对照组比较,前者皮疹发生率更高,其他系统损害、SLEDAI及自身抗体检测差异均无统计学意义.免疫抑制剂治疗后27例(79%)眼部症状或体征好转.结论 SLE患者眼部病变并不少见,其表现形式多种多样,重者视力下降甚至失明.SLE相关性眼部损害可出现在疾病早期,甚至先于疾病出现,免疫抑制剂治疗可能有效改善眼部症状.     

Ocular manifestations of Mycoplasma pneumoniae infection.

Ocular manifestations of Mycoplasma pneumoniae infection, other than conjunctivitis, are uncommon. Optic disk swelling, optic nerve atrophy, retinal exudates and hemorrhages, and cranial nerve palsies have been infrequently reported. We describe a 15-year-old patient who developed bilateral optic disk edema and iritis during an acute infection with M. pneumoniae and review the world literature on findings associated with ocular manifestations of infection with this pathogen. Although our patient experienced complete resolution of iritis and optic disk edema after 6 weeks, several patients described in the literature have experienced permanent sequelae as a result of optic neuropathy.     

Ocular surface involvement in patients with rheumatoid arthritis: Relation with disease activity and duration

Aim of the workThe aim of the present work was to present the frequency of ocular surface manifestations in rheumatoid arthritis (RA) patients and to determine its correlation with disease activity and duration.Patients and methodsThis cross-sectional study included 70 RA patients. Disease activity score (DAS-28) was scored. All patients underwent complete ophthalmic evaluation including assessment of visual acuity. Ocular surface assessment by Rose Bengal (RB) stain, and Schirmer test (ST) was carried out.ResultsSeventy patients (137 eyes) were studied; 3 eyes were excluded due to previous eye surgery. Patients were 67 women and 3 men with a mean age of 47.96 ± 10.8 years, and disease duration 9.19 ± 6.26 years. The mean DAS28 was 4.1 ± 0.77. Rheumatoid factor was positive in 39 (55.7%). Mean best-corrected visual acuity was 0.47 ± 0.33, ST was 10.6 ± 6.1 mm/5 min and RB score was 6.8 ± 3.6. 70% had ocular manifestations in the form of dry eye (54.7%), pterygium (16.1%), posterior blepharitis (5.8%), scleritis (1.3%), corneal thinning (13.9%), filamentary keratitis (8.7%) and maculopathy (11.7%). There was a significant correlation between ST and disease duration (r = −0.17, p = 0.04). Morning stiffness had a significant correlation with both ST (r = −0.2, p = 0.02) and RB (r = 0.22, p = 0.01) and remained significant on regression analysis. DAS-28 was not significantly associated with any of the ocular surface abnormalities.ConclusionOcular manifestations are significant in patients with RA. The most common ocular manifestation is dry eye. Ocular manifestations are significantly associated with the disease duration and should be expected regardless of disease activity in RA patients.