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      A Case Demonstrating the Pathological Relationship between Granulomatous Vasculitis and Glomerular Lesion in Renal Sarcoidosis


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          We experienced a rare case of tubulointerstitial angiocentric granulomatous vasculitis with focal segmental glomerulosclerosis (FSGS) and associated sarcoidosis. Our patient was an 18-year-old man who presented with exertional cough and dyspnea. He also had overt proteinuria (3.0 g/24 h), normal renal function (eGFR 95 mL/min/1.73 m<sup>2</sup>), heart failure, and hypertension. He had no previous episode of hypertension. These manifestations immediately improved after the administration of antihypertensive therapy that contained an angiotensin-converting enzyme inhibitor, calcium antagonists, beta antagonists, and diuretics. However, he, later on, developed renal dysfunction, with worsening of both proteinuria and hypertension. Renal biopsy was performed and showed epithelioid cells that were arranged concentrically around small blood vessels in tubulointerstitial granulomas. In the glomeruli, the segmental sclerotic lesions were classified as a perihilar variant of FSGS. There were no inflammatory changes, such as a mesangial lesion, inflammatory cell infiltration, fibrinoid necrosis, or crescent formation, and no glomerular granuloma. In the tubulointerstitial granulomas, the intimal elastic lamina of the interlobular arteries was reduplicated, and the intimal wall thickness of renal arterioles was remarkable. After receiving oral prednisolone therapy, the overt proteinuria resolved, the eGFR recovered from 39.4 to 60.6 mL/min/1.73 m<sup>2</sup>, and hypertension was managed more easily. Thereafter, he did not experience any recurrence. The concurrent improvement of renal function and proteinuria by steroid treatment suggested a relationship between the glomerular lesions and the tubulointerstitial granulomatous vasculitis with associated sarcoidosis.

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          Most cited references17

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            The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool.

            A Case Control Etiology of Sarcoidosis Study (ACCESS) sarcoidosis organ assessment instrument has been used for more than a decade to establish uniform standards for the probability of sarcoidosis organ involvement. The ACCESS instrument has become increasingly outdated as new technologies have been developed. Furthermore, the ACCESS instrument failed to address all possible organs involved with sarcoidosis. For these reasons, the World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) developed a new sarcoidosis organ assessment instrument.
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              Pathologic classification of focal segmental glomerulosclerosis: a working proposal.


                Author and article information

                Case Reports in Nephrology and Dialysis
                S. Karger AG
                September - December 2020
                12 October 2020
                : 10
                : 3
                : 109-116
                [_a] aKitamurayama Hospital, Higashine, Japan
                [_b] bJR Sendai Hospital, Sendai, Japan
                [_c] cDepartment of Nephrology and Endocrinology, Osaki Citizen Hospital, Osaki, Japan
                [_d] dRifuno Internal Medicine Clinic, Rifu, Japan
                [_e] eDivision of Nephrology, Endocrinology and Vascular Medicine, Tohoku University, Sendai, Japan
                [_f] fPulmonary Medicine, Nippon Medical School, Tokyo, Japan
                Author notes
                *Yoshinori Kamata, Kitamurayama Hospital, 2-15-1 Onsenmachi, Higashine, Yamagata 999-3792 (Japan), kp4y-kmt@asahi-net.or.jp
                509590 PMC7588675 Case Rep Nephrol Dial 2020;10:109–116
                © 2020 The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. 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.

                : 27 January 2020
                : 20 June 2020
                Page count
                Figures: 3, Pages: 8
                Case Report

                Cardiovascular Medicine,Nephrology
                Focal segmental glomerulosclerosis,Renal sarcoidosis,Perihilar variant,Vasculitis


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