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      Behçet’s Disease and AA-Type Amyloidosis

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          Abstract

          Behçet’s disease (BD) is a multisystem disorder characterized by vasculitis. To our knowledge, 52 patients with BD and amyloidosis have previously been described in the literature. Nephrotic syndrome was the most common type of presentation of amyloidosis in these patients. The prognosis of patients with BD and amyloidosis has not been reported before. In this report, we present a patient with BD and AA-type amyloidosis and analyze the prognosis in these patients. Follow-up and prognosis have been reported in 23 patients. Ten of these 23 patients died and most of these deaths occurred within 3 months after the diagnosis of amyloidosis. End-stage renal disease developed shortly in 4 patients. BD should be considered in the differential diagnosis of AA amyloidosis. In conclusion, the cases with BD and amyloidosis carry poor prognosis.

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          Criteria for diagnosis of Behcet's disease

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            End-Stage Renal Failure due to Crescentic Glomerulonephritis in a Patient with Behçet’s Syndrome

            We report a rare case of a patient with Behçet’s syndrome who developed end-stage renal failure due to crescentic glomerulonephritis (GN). A 20-year-old male patient had suffered from uveitis, aphthous mouth ulcers and genital ulceration for the past 7 years. His renal function rapidly deteriorated and renal biopsy specimens obtained when his serum creatinine level was 3 mg/dl showed diffuse proliferative GN with fibrous crescent formation in 75% of glomeruli excluding totally sclerotic glomeruli. Immune complexes were identified by demonstration of complement and immunoglobulins in the glomeruli. He developed end-stage renal failure during a 1-year course and received maintenance hemodialysis. We reviewed the literature on severe forms of GN in patients with Behçet’s syndrome.
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              Author and article information

              Journal
              AJN
              Am J Nephrol
              10.1159/issn.0250-8095
              American Journal of Nephrology
              S. Karger AG
              0250-8095
              1421-9670
              2000
              February 2000
              13 January 2000
              : 20
              : 1
              : 68-70
              Affiliations
              Departments of aNephrology and bPathology, School of Medicine, Ondokuz Mayıs University, Samsun, Turkey
              Article
              13559 Am J Nephrol 2000;20:68–70
              10.1159/000013559
              10644872
              © 2000 S. Karger AG, Basel

              Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

              Page count
              Figures: 1, References: 24, Pages: 3
              Product
              Self URI (application/pdf): https://www.karger.com/Article/Pdf/13559
              Categories
              Case Report

              Cardiovascular Medicine, Nephrology

              Amyloidosis, Behçet’s disease, Prognosis

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