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      Ocular disease in patients with ANCA-positive vasculitis

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          Abstract

          Anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis—the term recently applied to Wegener's granulomatosis—is a rare multi-system inflammation characterized by necrotizing granulomas and vasculitis. We investigated the ocular manifestations of this disease in a group of patients drawn from five inflammatory eye disease clinics across the United States. Of 8,562 persons with ocular inflammation, 59 individuals were diagnosed with ANCA-positive vasculitis; 35 males and 21 females, aged 16 to 96 years, were included in this study. Ocular diagnoses were scleritis (75.0%), uveitis (17.9%), and other ocular inflammatory conditions (33.9%) including peripheral ulcerative keratitis and orbital pseudotumor. Mean duration of ocular disease was 4.6 years. Oral corticosteroids and other systemic immunosuppressive agents were used by 85.7% and 78.5% of patients, respectively. Over time, patients with ANCA-positive vasculitis experienced 2.75-fold higher mortality than other patients with inflammatory eye disease.

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          Wegener granulomatosis: an analysis of 158 patients.

          Gary Hoffman (1992)
          To prospectively study the clinical features, pathophysiology, treatment and prognosis of Wegener granulomatosis. Of the 180 patients with Wegener granulomatosis referred to the National Institute of Allergy and Infectious Diseases during the past 24 years, 158 have been followed for 6 months to 24 years (a total of 1229 patient-years). Characteristics of clinical presentation, surgical pathology, course of illness, laboratory and radiographic findings, and the results of medical and surgical treatment have been recorded in a computer-based information retrieval system. The Warren Magnuson Clinical Center of the National Institutes of Health. Men and women were equally represented; 97% of patients were white, and 85% were more than 19 years of age. The mean period of follow-up was 8 years. One hundred and thirty-three patients (84%) received "standard" therapy with daily low-dose cyclophosphamide and glucocorticoids. Eight (5.0%) received only low-dose cyclophosphamide. Six (4.0%) never received cyclophosphamide and were treated with other cytotoxic agents and glucocorticoids. Ten patients (6.0%) were treated with only glucocorticoids. Ninety-one percent of patients experienced marked improvement, and 75% achieved complete remission. Fifty percent of remissions were associated with one or more relapses. Of 99 patients followed for greater than 5 years, 44% had remissions of greater than 5 years duration. Thirteen percent of patients died of Wegener granulomatosis, treatment-related causes, or both. Almost all patients had serious morbidity from irreversible features of their disease (86%) or side effects of treatment (42%). The course of Wegener granulomatosis has been dramatically improved by daily treatment with cyclophosphamide and glucocorticoids. Nonetheless, disease- and treatment-related morbidity is often profound. Alternative forms of therapy have not yet achieved the high rates of remission induction and successful maintenance that have been reported with daily cyclophosphamide treatment. Despite continued therapeutic success with cyclophosphamide, our long-term follow-up of patients with Wegener granulomatosis has led to increasing concerns about toxicity resulting from prolonged cyclophosphamide therapy and has encouraged investigation of other therapeutic regimens.
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            The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis.

            Ronald L Calabrese, Blair Leavitt, A Fauci … (1990)
            Criteria for the classification of Wegener's granulomatosis (WG) were developed by comparing 85 patients who had this disease with 722 control patients with other forms of vasculitis. For the traditional format classification, 4 criteria were selected: abnormal urinary sediment (red cell casts or greater than 5 red blood cells per high power field), abnormal findings on chest radiograph (nodules, cavities, or fixed infiltrates), oral ulcers or nasal discharge, and granulomatous inflammation on biopsy. The presence of 2 or more of these 4 criteria was associated with a sensitivity of 88.2% and a specificity of 92.0%. A classification tree was also constructed with 5 criteria being selected. These criteria were the same as for the traditional format, but included hemoptysis. The classification tree was associated with a sensitivity of 87.1% and a specificity of 93.6%. We describe criteria which distinguish patients with WG from patients with other forms of vasculitis with a high level of sensitivity and specificity. This distinction is important because WG requires cyclophosphamide therapy, whereas many other forms of vasculitis can be treated with corticosteroids alone.
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              Etanercept plus standard therapy for Wegener's granulomatosis.

              Yuan-I Min (2005)
              The majority of patients with Wegener's granulomatosis have disease flares after conventional medications are tapered. There is no consistently safe, effective treatment for the maintenance of remission. We conducted a randomized, placebo-controlled trial at eight centers to evaluate etanercept for the maintenance of remission in 180 patients with Wegener's granulomatosis. The primary outcome was sustained remission, defined as a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis of 0 for at least six months (scores can range from 0 to 67, with higher scores indicating more active disease). In addition to etanercept or placebo, patients received standard therapy (glucocorticoids plus cyclophosphamide or methotrexate). After remission, standard medications were tapered according to the protocol. The mean follow-up for the overall cohort was 27 months. Of the 174 patients who could be evaluated, 126 (72.4 percent) had a sustained remission, but only 86 (49.4 percent) remained in remission for the remainder of the trial. There were no significant differences between the etanercept and control groups in the rates of sustained remission (69.7 percent vs. 75.3 percent, P=0.39), sustained periods of low-level disease activity (86.5 percent vs. 90.6 percent, P=0.32), or the time required to achieve those measures. Disease flares were common in both groups, with 118 flares in the etanercept group (23 severe and 95 limited) and 134 in the control group (25 severe and 109 limited). There was no significant difference between the etanercept and control groups in the relative risk of disease flares per 100 person-years of follow-up (0.89, P=0.54). During the study, 56.2 percent of patients in the etanercept group and 57.1 percent of those in the control group had at least one severe or life-threatening adverse event or died (P=0.90). Solid cancers developed in six patients in the etanercept group, as compared with none in the control group (P=0.01). Etanercept is not effective for the maintenance of remission in patients with Wegener's granulomatosis. Durable remissions were achieved in only a minority of the patients, and there was a high rate of treatment-related complications. Copyright 2005 Massachusetts Medical Society.
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                Author and article information

                Contributors
                : +1-503-4945023 , +1-503-4946875 , smithjus@ohsu.edu
                Journal
                Journal ID (nlm-ta): J Ocul Biol Dis Infor
                Title: Journal of Ocular Biology, Diseases, and Informatics
                Publisher: Humana Press Inc (New York )
                ISSN (Print): 1936-8437
                ISSN (Electronic): 1936-8445
                Publication date (Electronic): 12 December 2009
                Publication date PMC-release: 12 December 2009
                Publication date Collection: March 2010
                Volume: 3
                Issue: 1
                Pages: 12-19
                Affiliations
                [1 ]Casey Eye Institute, Oregon Health & Science University, Portland, OR USA
                [2 ]Ocular Inflammation Service, Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA USA
                [3 ]Center for Preventive Ophthalmology and Biostatistics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, PA USA
                [4 ]Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA USA
                [5 ]Department of Public Health & Preventive Medicine, Oregon Health & Science University, Portland, OR USA
                [6 ]Massachusetts Eye Research and Surgery Institution, Cambridge, MA USA
                [7 ]National Eye Institute, Bethesda, MD USA
                [8 ]Department of Internal Medicine, Oregon Health & Science University, Portland, OR USA
                [9 ]Department of Cell and Developmental Biology, Oregon Health & Science University, Portland, OR USA
                [10 ]Department of Ophthalmology, Johns Hopkins University School of Medicine, Baltimore, MD USA
                [11 ]Department of Epidemiology, Johns Hopkins University School of Medicine, Baltimore, MD USA
                [12 ]Department of Ophthalmology, Harvard Medical School, Boston, MA USA
                [13 ]Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD USA
                [14 ]Department of Ophthalmology, Mount Sinai School of Medicine, New York, NY USA
                [15 ]Department of Medicine, Mount Sinai School of Medicine, New York, NY USA
                [16 ]St. Luke’s Cataract and Laser Institute, Tarpon Springs, FL USA
                [17 ]Portland Veterans’ Affairs Medical Center, Portland, OR USA
                [18 ]Oregon Health & Science University, Mail Code: L467AD, Biomedical Research Building 3181 S.W. Sam Jackson Park Rd., Portland, OR 97239 USA
                Article
                Publisher ID: 9044
                DOI: 10.1007/s12177-009-9044-4
                PMC ID: 2933008
                PubMed ID: 20835396
                SO-VID: 6aa2c14d-112b-46f3-a484-509a51a0bedc
                Copyright © © The Author(s) 2009
                History
                Date received : 25 July 2009
                Date accepted : 9 November 2009
                Categories
                Subject: Article
                Custom metadata
                issue-copyright-statement © Humana Press 2010

                ScienceOpen disciplines: Vision sciences
                Keywords: scleritis,eye,anca-positive vasculitis,wegener's granulomatosis
                Data availability:
                ScienceOpen disciplines: Vision sciences
                Keywords: scleritis, eye, anca-positive vasculitis, wegener's granulomatosis

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