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      Pericardial Tamponade: An Uncommon Clinical Presentation in cANCA Related Vasculitis and Glomerulonephritis in Association with Very High Titres of ANA

      case-report
      1 , , 2 , 3 , 1
      Case Reports in Nephrology
      Hindawi

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          Abstract

          ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide.

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          Most cited references10

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          Pathogenesis of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis.

          Clinical, in vitro, and experimental animal observations indicate that antineutrophil cytoplasmic autoantibodies (ANCA) are pathogenic. The genesis of the ANCA autoimmune response is a multifactorial process that includes genetic predisposition, environmental adjuvant factors, an initiating antigen, and failure of T cell regulation. ANCA activate primed neutrophils (and monocytes) by binding to certain antigens expressed on the surface of neutrophils in specific inflammatory microenvironments. ANCA-activated neutrophils activate the alternative complement pathway, establishing an inflammatory amplification loop. The acute injury elicits an innate inflammatory response that recruits monocytes and T lymphocytes, which replace the neutrophils that have undergone karyorrhexis during acute inflammation. Extravascular granulomatous inflammation may be initiated by ANCA-induced activation of extravascular neutrophils, causing tissue necrosis and fibrin formation, which would elicit an influx of monocytes that transform into macrophages and multinucleated giant cells. Over time, the neutrophil-rich acute necrotizing lesions cause the accumulation of more lymphocytes, monocytes, and macrophages and produce typical granulomatous inflammation.
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            Management of pericardial effusion.

            Pericardial effusion is a common finding in clinical practice either as incidental finding or manifestation of a systemic or cardiac disease. The spectrum of pericardial effusions ranges from mild asymptomatic effusions to cardiac tamponade. The aetiology is varied (infectious, neoplastic, autoimmune, metabolic, and drug-related), being tuberculosis the leading cause of pericardial effusions in developing countries and all over the world, while concurrent HIV infection may have an important promoting role in this setting. Management is guided by the haemodynamic impact, size, presence of inflammation (i.e. pericarditis), associated medical conditions, and the aetiology whenever possible. Pericardiocentesis is mandatory for cardiac tamponade and when a bacterial or neoplastic aetiology is suspected. Pericardial biopsy is generally reserved for cases with recurrent cardiac tamponade or persistence without a defined aetiology, especially when a bacterial or neoplastic aetiology is suspected and cannot be assessed by other conventional and less invasive means. A true isolated effusion may not require a specific treatment if the patient is asymptomatic, but large ones are at risk of progression to cardiac tamponade (up to one third). Pericardiocentesis alone may be curative for large effusions, but recurrences are also common and pericardiectomy or less invasive options (i.e. pericardial window) should be considered with recurrent cardiac tamponade or symptomatic pericardial effusion (either circumferential or loculated). The aim of this paper was to summarize and critically evaluate current knowledge on the management of pericardial effusion.
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              Cardiac involvement in Churg-Strauss syndrome.

              Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients. Thirty-two consecutive patients with CSS in remission (mean +/- SD duration of disease between diagnosis and enrollment 6.1 +/- 5.8 years, mean +/- SD age 61 +/- 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age- and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI). Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P = 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI. These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement.
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                Author and article information

                Contributors
                Journal
                Case Rep Nephrol
                Case Rep Nephrol
                CRIN
                Case Reports in Nephrology
                Hindawi
                2090-6641
                2090-665X
                2019
                13 June 2019
                : 2019
                : 4983139
                Affiliations
                1Nephrology, Eastern Virginia Medical School, 855 W Brambelton Ave, Norfolk, VA 23510, USA
                2Eastern Virginia Medical School, Norfolk, VA, USA
                3Pathology, Eastern Virginia Medical School, 855 W Brambelton Ave, Norfolk, VA 23510, USA
                Author notes

                Academic Editor: Yoshihide Fujigaki

                Author information
                https://orcid.org/0000-0002-5040-3081
                Article
                10.1155/2019/4983139
                6595352
                4cd0622f-4415-45b3-874c-3d30275b5165
                Copyright © 2019 Amaresh Vanga 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
                : 26 January 2019
                : 12 May 2019
                : 19 May 2019
                Categories
                Case Report

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