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      Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

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          Abstract

          Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

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          Most cited references12

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          The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis

          Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of pauci-immune small vessel vasculitides that often affect the kidneys manifesting as rapidly progressive glomerulonephritis. Although the exact pathogenesis of AAV is not fully known, evidence from in vitro, in vivo and clinical studies all point to the involvement of ANCA in the pathogenesis of AAV. In this review, we highlight the contributory roles played by various factors (e.g. genetics, environment, B and T-regulatory cells, toll-like receptors, etc.) in the pathogenesis of AAV. Furthermore, we discuss renal involvement in AAV in terms of clinical features and the various histopathological classification patterns, which are also known to be of prognostic importance. We also present information on useful imaging techniques for localizing kidney and other organ system involvement in AAV, and also on novel laboratory methods and assays useful for rapid and more specific determination of patients' ANCA status. Finally, we demonstrate evidence on novel serum biomarkers that have been shown to correlate with disease activity in AAV.
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            Pregnancy in women diagnosed with antineutrophil cytoplasmic antibody-associated vasculitis: outcome for the mother and the child.

            Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is infrequently seen in women of childbearing age. Only a limited number of pregnancies in women with AAV have been reported, and often they were associated with complications.
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              Role of therapeutic plasmapheresis in ANCA-associated vasculitis.

              Plasma exchange, or plasmapheresis, is a treatment method that developed over a period of two decades and involves the removal and replacement of a patient's circulating plasma. The aim of treatment is to remove disease-associated molecules and therefore interrupt disease progression. This article summarizes the developmental history of this treatment and then looks in more detail at data on the use of plasma exchange in treating antineutrophil antibody (ANCA)-associated vasculitis. The eight randomized trials and the Cochrane Systematic Review on treating renal vasculitis are summarized to show that plasma exchange may be effective in this disease, specifically in reducing the development of end-stage kidney disease (ESKD) by approximately 40%. The plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS) study is a currently enrolling study aiming to answer some of the outstanding questions relating to the use of this treatment in ANCA-associated vasculitis.
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                Author and article information

                Journal
                Case Rep Nephrol
                Case Rep Nephrol
                CRIN
                Case Reports in Nephrology
                Hindawi Publishing Corporation
                2090-6641
                2090-665X
                2016
                11 May 2016
                : 2016
                : 1075659
                Affiliations
                1Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, FL 32209, USA
                2Department of Pathology, University of Florida College of Medicine, Jacksonville, FL 32209, USA
                3Department of Nephrology, University of Florida College of Medicine, Jacksonville, FL 32209, USA
                Author notes

                Academic Editor: Kouichi Hirayama

                Article
                10.1155/2016/1075659
                4879222
                27293925
                5be0f451-4eeb-4103-af8d-b7114ca45b3c
                Copyright © 2016 Ryan Kunjal 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.

                History
                : 17 February 2016
                : 11 April 2016
                : 24 April 2016
                Categories
                Case Report

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