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      Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis: A Meta-Analysis of 62 Case Reports

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          Abstract

          Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-systemic vasculitis, with cardiac involvement being one of its most serious manifestations. We aimed to systematically review and analyze the limited case reports of EGPA with cardiac involvement.

          Methods: Based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we performed a systematic literature search for the case reports of EGPA with cardiac involvement in the MEDLINE database from 2011 until 2018. For each case, clinical data including sex, age, clinical presentation, electrocardiographic and cardiac imaging findings, the type of cardiac involvement, the available laboratory data (cardiac biomarkers, white blood cell count, eosinophilic count, erythrocyte sedimentation rate, C-reactive protein, and antineutrophil cytoplasmic antibody positivity), therapeutic regimen, and the outcome of the patients were collected and analyzed.

          Results: A total number of 62 cases were included. The mean age was 48.29±15.60 years, and 51.6% were male. All the cases were in the active disease state. Cardiac symptoms, electrocardiographic abnormalities, abnormal biomarkers, and abnormal echocardiography were detected in 82.3%, 68.5%, 77.4%, and 96.8%, respectively. Cardiac magnetic resonance was done in 46.8% of the patients, and it was abnormal in all. The most common abnormal findings in echocardiography were systolic left ventricular dysfunction (83.9%) and pericardial effusion (37.1%). The most common type of clinical presentation was clinical heart failure (51.6%). Only 6.5% of the patients presented with tamponade. The overall prognosis was good.

          Conclusion: Any part of the heart could be involved by EGPA. The results emphasize the necessity of in-depth cardiac evaluation in these patients.

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          Most cited references71

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          The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).

          Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia greater than 10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.
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            Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients.

            Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.
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              Churg-Strauss syndrome.

              Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative.
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                Author and article information

                Journal
                J Tehran Heart Cent
                J Tehran Heart Cent
                JTHC
                The Journal of Tehran University Heart Center
                Tehran University of Medical Sciences, 2006- (Tehran, Iran )
                1735-5370
                2008-2371
                January 2020
                : 15
                : 1
                : 18-26
                Affiliations
                [1 ]School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
                [2 ]Department of Cardiovascular Diseases, Hazrat‐e Rasool General Hospital, Iran University of Medical Sciences, Tehran, Iran.
                Author notes
                [* ]Corresponding Author: Marziyeh Pakbaz, Department of Cardiovascular Diseases, Hazrat‐e Rasool General Hospital, Iran University of Medical Sciences, Sattar-Khan Avenue, Niyayesh Street, Tehran, Iran. 1445613131. Tel: +98 21 64352463. Fax: +98 21 64352466. E-mail: marzi.pakbaz@ 123456gmail.com.
                Article
                JTHC-15-18
                10.18502/jthc.v15i1.3334
                7360870
                32742288
                533b24fb-6e56-4e53-8f5a-c31a664bc438
                Copyright © 2015 Tehran Heart Center, Tehran University of Medical Sciences

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 1 August 2018
                : 10 November 2019
                Categories
                Original Article

                Cardiovascular Medicine
                churg-strauss syndrome,anti-neutrophil cytoplasmic antibody-associated vasculitis,cardiovascular diseases

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