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      Eosinophilic granulomatosis with polyangiitis: myocardial thickening reversed by corticosteroids

      case-report

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          Abstract

          Background

          In 1951 Churg and Strauss first described the clinical condition now known as eosinophilic granulomatosis with polyangiitis (EGPA), characterized by asthma, nasal polyposis, rhinosinusitis, hypereosinophilia with organ infiltration, and necrotizing vasculitis. It is classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, but ANCA negativity is common and more frequently encountered in EGPA with myocardial involvement. Long-term survival has substantially improved with corticosteroid treatment but myocardial involvement is still the leading cause of death in EGPA.

          Case presentation

          A 53-year old man with a history of asthma and nasal polyposis presented with acute chest pain and elevated troponin; a percutaneous coronary intervention was performed. The left ventricle was described as hypertrophic. After 20 days the myocardium had markedly increased in thickness of both the right and left ventricle. Evaluation revealed hypereosinophilia in the blood and nasal mucosal tissue, which confirmed the diagnosis of EGPA. He presented with signs of active vasculitis including weight loss, tiredness, intracerebral hemorrhage, and increasing serum creatinine. After 6 days of corticosteroid treatment, the myocardium returned to its initial thickness.

          Conclusion

          Rapid and marked thickening of the myocardium is not frequently reported but may occur in EGPA. Myocardial thickening in EGPA can be quickly reversed by corticosteroids, and is most likely caused by edema.

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          Most cited references15

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          Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.

          Matthieu Groh, Christian Pagnoux, Chiara Baldini … (2015)
          To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA).
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            Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.

            M Frassi, Carlo Buzio, R. A. Sinico … (2005)
            Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features. Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status. ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042). ANCAs are present in approximately 40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.
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              Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.

              Holly Lanham, C Pusey, G. Hughes … (1984)
              Drawing on our experience of 16 cases and a review of the English literature, we propose that CSS is under-diagnosed because of exclusive emphasis upon pathologic recognition of the disorder. The classical histological picture comprises a necrotizing vasculitis, eosinophilic tissue infiltration and extravascular granulomas, but it is only found in a minority of cases, and is not pathognomonic of the condition (69, 108). On the other hand, the clinical pattern of the disorder is most distinctive, and CSS can be readily identified on clinical grounds. Typically, it begins with allergic rhinitis, which is often complicated by nasal polyposis and sinusitis. Asthma and peripheral blood eosinophilia are essential features, often accompanied by pulmonary infiltrates. The systemic vasculitis of CSS resembles that of PAN, but severe renal disease is uncommon (the typical renal lesion is a focal segmental glomerulonephritis), and cardiac involvement accounts for 50% of deaths. Diagnostic difficulties arise from the close relationship of CSS to other granulomatous, vasculitic and eosinophilic disorders. CSS is usefully regarded as a point of overlap between these three disease spectrums (Fig. 5). Individual components of each spectrum can occur in the course of CSS; hence cases may be reported as PAN developing as a complication of Löffler syndrome or eosinophilic gastroenteritis (37, 57, 66). The hypereosinophilia of CSS tends to be less severe and more steroid-responsive than in HES, and evidence of eosinophil degranulation was not found in the patients we studied. Complement abnormalities are not a prominent feature of the disorder, and circulating immune complexes were detected in only two cases; both contained IgM. This may be of pathogenetic significance as IgM deposition was a dominant feature in four of the five cases with positive renal immunofluorescence. IgE levels were elevated in all patients studied during the vasculitic phase, and skin-prick tests were positive in 8 of 10 patients tested. CSS responds well to treatment with steroids, although some patients benefit from the addition of immunosuppressive agents. The vasculitic illness is usually of limited duration, but relapses can occur, and should be detected and treated early. Major problems in the post-vasculitic phase stem from hypertension and persisting peripheral nerve damage. Allergic upper and lower respiratory tract disease is an important cause of morbidity in the pre- and post-vasculitic periods.
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                Author and article information

                Contributors
                Gustav Mattsson:
                ORCID: http://orcid.org/0000-0002-4317-0443
                +46(0)703 122296 , gustav.mattsson@regiongavleborg.se
                Peter Magnusson: peter.magnusson@regiongavleborg.se
                Journal
                Journal ID (nlm-ta): BMC Cardiovasc Disord
                Journal ID (iso-abbrev): BMC Cardiovasc Disord
                Title: BMC Cardiovascular Disorders
                Publisher: BioMed Central (London )
                ISSN (Electronic): 1471-2261
                Publication date (Electronic): 20 December 2017
                Publication date PMC-release: 20 December 2017
                Publication date Collection: 2017
                Volume: 17
                Electronic Location Identifier: 299
                Affiliations
                [1 ]ISNI 0000 0004 1936 9457, GRID grid.8993.b, Centre for Research and Development, , Uppsala University/Region Gävleborg, ; SE-801 87 Gävle, Sweden
                [2 ]ISNI 0000 0004 1937 0626, GRID grid.4714.6, Cardiology Research Unit, Department of Medicine, , Karolinska Institutet, ; SE-171 76 Stockholm, Sweden
                Author information
                http://orcid.org/0000-0002-4317-0443
                Article
                Publisher ID: 734
                DOI: 10.1186/s12872-017-0734-8
                PMC ID: 5738832
                PubMed ID: 29262787
                SO-VID: 7e766462-54cf-4608-9271-c235aeb8dd5e
                Copyright © © The Author(s). 2017
                License:

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                Date received : 25 September 2017
                Date accepted : 12 December 2017
                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © The Author(s) 2017

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: cardiac imaging,cardiac magnetic resonance,cardiomyopathy,churg-strauss,corticosteroids,echocardiography,eosinophilic granulomatosis with polyangiitis,heart failure,hypertrophy,myocardial thickening
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: cardiac imaging, cardiac magnetic resonance, cardiomyopathy, churg-strauss, corticosteroids, echocardiography, eosinophilic granulomatosis with polyangiitis, heart failure, hypertrophy, myocardial thickening

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