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      Upper Gastrointestinal Bleeding as the First Manifestation of Wegener’s Granulomatosis

      case-report

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          Abstract

          Wegener’s granulomatosis is an uncommon inflammatory disease that manifests as vasculitis, granulomatosis, and necrosis. It usually involves the upper and lower respiratory tracts and kidneys. Although it may essentially involve any organ, gastrointestinal (GI) involvement is notably uncommon.

          A 20-year-old male patient presented with epigastric pain, vomiting, hematemesis, and melena. On physical examination, he was pale. There was no abdominal tenderness or organomegaly. Upper GI endoscopy revealed dark blue-colored infiltrative lesions in prepyloric area. Evaluation of the biopsy sample showed mononuclear cell infiltration in the submucosal area, hyperplastic polyp, and chronic gastritis. High dose proton pump inhibitor and adjunctive supportive measures were given but no change in the follow-up endoscopy was detected. During hospital course, he developed intermittent fever and serum creatinine elevation. 12 days after admission, he developed dyspnea, tachypnea, and painful swelling of metacarpophalangeal joints, and maculopapular rash in extensor surface of the right forearm. Chest radiography showed pulmonary infiltration. Serum c-ANCA titer was strongly positive and skin biopsy revealed leukocytoclastic vasculitis. The patient received methylprednisolone pulse, which resulted in complete recovery of symptoms and gastric lesion.

          The present case indicates that GI bleeding may be the first manifestation of Wegener’s granulomatosis. Moreover, it should be emphasized that gastric biopsy is not characteristic or diagnostic in such patients.

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          Most cited references20

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          Wegener granulomatosis: an analysis of 158 patients.

          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.

            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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              Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis.

              Granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA) are subgroups of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) defined historically by clinical and histological features. GPA and MPA are heterogeneous entities with overlapping phenotypes. To identify novel subgroupings, cluster analysis was used to explore the phenotypic spectrum of AAV. This study used a dataset of patients newly diagnosed as having GPA and MPA enrolled in five clinical trials. One cluster model included nine clinical baseline variables as input variables, and a second cluster model additionally included ANCA specificities. The clustering process involved multiple correspondence analyses followed by hierarchical ascendant cluster analysis. The clinical relevance of the generated clusters was analysed by their summary characteristics and outcomes. The analyses involved data for 673 subjects: 396 (59%) with GPA and 277 (41%) with MPA. Both cluster models resulted in five partially redundant clusters of subjects, and the model including ANCA resulted in more pertinent separations. These clusters were named 'renal AAV with proteinase 3 (PR3)-ANCA' (40% of subjects), 'renal AAV without PR3-ANCA' (32%) and 'non-renal AAV' (12%), 'cardiovascular AAV' (9%) and 'gastrointestinal AAV' (7%). The five clusters had distinct death and relapse rates. On the basis of 4 variables, 651 subjects (97%) could be accurately allocated to 1 of the 5 classes. This analysis suggests that AAV encompasses five classes associated with different outcomes. As compared with the traditional GPA-MPA separation, this classification system may better reflect the phenotypic spectrum of AAV.
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                Author and article information

                Journal
                Middle East J Dig Dis
                Middle East J Dig Dis
                Middle East J Dig Dis
                MEJDD
                Middle East Journal of Digestive Diseases
                Iranian Association of Gastroerterology and Hepatology
                2008-5230
                2008-5249
                July 2016
                : 8
                : 3
                : 235-239
                Affiliations
                1 Department of Gastroenterology, Al- Zahra Hospital, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
                2 Department of Internal Medicine, Imam Reza Hospital, , School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran
                3 Department of Rheumatology, Al-Zahra Hospital, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
                4 Department of pathology, Al-Zahra Hospital, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
                Author notes
                [* ] Corresponding Author: Mehdi Zobeiri , MD Zacaria Razi Boulevard, Imam Reza Hospital, Kermanshah, Iran Post code: 6714415333 Tel: + 98 9188311726 Fax: + 98 8314276343 mzoberi@ 123456KUMS.ac.ir
                Article
                10.15171/mejdd.2016.27
                5045678
                69825769-d3d0-4407-be4f-0bacb8c3858e
                © 2016 Middle East Journal of Digestive Diseases

                This work is published by Middle East Journal of Digestive Diseases as an open access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by-nc-sa/4.0/). Non-commercial uses of the work are permitted, provided the original work is properly cited.

                History
                : 28 February 2016
                : 10 May 2016
                Page count
                Figures: 3, References: 24, Pages: 5
                Categories
                Case Report

                wegener’s granulomatosis,gastrointestinal symptoms,vasculitis,anca

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