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      Renal Solid Mass as a Rare Presentation of Wagener's Granulomatosis: A Case Report

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          Abstract

          Wagener's granulomatosis (WG) is a rheumatologic disease with unknown etiology which renal and pulmonary involvement is commonly seen. Renal involvement in Wagener's granulomatosis represents as a segmental necrotizing glomerulonephritis which is not visible with imaging techniques and usually presents with proteinuria, microhematuria, and hypertension. A rare presentation of the disease is a renal mass which can be mistaken as renal tumors, abscess, or lymphoma. We report a 22-year-old female with flank pain and fever who was admitted in our hospital. The patient underwent renal tumor biopsy and diagnosed with Wagener's granulomatosis in pathologic staining. The aim of this work is introduction of Wagener's granulomatosis as a differential diagnosis of renal tumors, to prevent unnecessary interventions and delayed treatment.

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          Most cited references 7

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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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            Unusual radiographic features of Wegener's granulomatosis.

            The classical radiologic manifestations of Wegener's granulomatosis are paranasal sinus disease and pulmonary nodules. A review of 31 clinicopathologically proven cases of Wegener's granulomatosis was undertaken to delineate some of the atypical radiologic presentations in this disorder. In 16 of the 31 patients a total of 18 atypical radiographic findings were demonstrated. These included lesions of the airways, parenchyma, pleura, and kidneys. We feel the "typical radiologic findings" of Wegener's granulomatosis should be expanded to include (1) endobronchial disease resulting in atelectasis of either a lobe or a complete lung, and (2) pleural disease with thickening or effusion which may be massive. While some of the rarer presentations are still not included, such a redefinition would cover more than 75% of our 31 cases.
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              Wegener's granulomatosis presenting as a renal mass.

              Wegener's granulomatosis is a systemic necrotizing vasculitis that usually involves the kidneys, typically causing segmental necrotizing glomerulonephritis. An association between Wegener's granulomatosis and renal cell carcinoma was recently reported. We describe a case of Wegener's granulomatosis presenting as a renal mass in a 72-year-old woman. Histologic examination of the mass revealed granulomatous inflammation, an extremely rare manifestation of this disease. We also review the incidence of renal mass in Wegener's granulomatosis and highlight the importance of excluding the coexistence of renal cell carcinoma.
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                Author and article information

                Journal
                Case Rep Urol
                Case Rep Urol
                CRIM.UROLOGY
                Case Reports in Urology
                Hindawi Publishing Corporation
                2090-696X
                2090-6978
                2012
                5 August 2012
                : 2012
                Affiliations
                1Department of Urology, Isfahan University of Medical Sciences, Isfahan 81746-75731, Iran
                2Department of Pathology, Isfahan University of Medical Sciences, Isfahan 81746-75731, Iran
                3School of Medicine, Isfahan University of Medical Sciences, Isfahan 81746-75731, Iran
                Author notes

                Academic Editors: G. L. Gravina, H.-L. Lee, and T. Nomura

                Article
                10.1155/2012/793014
                3420076
                22919535
                Copyright © 2012 Mehrdad Mohammadi Sichani et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Urology

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