Advertisement

Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): Prospective evaluation at a tertiary referral centre

Published:December 22, 2020DOI:https://doi.org/10.1016/j.ejim.2020.12.008

      Abstract

      Background

      Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. Cardiac specific involvement (CSI) is caused by coronary artery vasculitis, but also by myocardial eosinophilic infiltration. To date, the prevalence of CSI associated with EGPA is unresolved. Aim of this study was to systematically assess the prevalence and clinical impact of CSI in a consecutive outpatient EGPA population.

      Methods

      Between October 2018 and July 2019, we prospectively enrolled 52 consecutive EGPA patients. All underwent comprehensive evaluation including a standardized questionnaire, physical examination, 12-lead-ECG, echocardiography. Cardiac magnetic resonance and 24 h-Holter were performed as deemed clinically appropriate. Cardiac abnormalities were defined as CSI based on the likelihood of their relation to EGPA vasculitis, after exclusion of alternative diagnoses.

      Results

      52 enrolled patients, mean age 59±1 years. Thirteen of the 52 patients (25%) were classified as CSI+. CSI was characterized by myocarditis in four patients, non-scar-related regional wall motions abnormalities (RWMA) in three, apical thrombosis in two (one also had RWMA), pericarditis in three and non-atherosclerotic coronary disease (Prinzmetal angina and coronaritis) in 2. Five (38%) of the 13 CSI+ patients, presented an apical aneurysm. Peak eosinophil count at diagnosis was higher in CSI+: 8000 /μl vs CSI-: 3000 /μl, p = 0.017. Overall, 2 patients had severe LV dysfunction, 5 required urgent hospitalization and 8 required long-term cardioactive therapy.

      Conclusions

      CSI was present in one-quarter of patients, often associated with high peak eosinophils. Myocarditis, RWMA and apical aneurysms were the most common manifestations. Although rarely severe and life-threatening, CSI often required long-term cardioactive treatment.

      Keywords

      Abbreviations:

      ACR (American College of Rheumatology), ANCA (anti-neutrophil cytoplasmic antibodies), ASE (American Society of Echocardiography), CMR (cardiac magnetic resonance), CSI (cardiac specific involvement), ECG (electrocardiogram), EF (ejection fraction), EGPA (Eosinophilic granulomatosis with polyangiitis), ENT (ear nose throat), GLS (Global Longitudinal Strain), ICD (implantable cardioverter defibrillator), LGE (late gadolinium enhancement), LV (left ventricle), NIBP (non-invasive blood pressure), NSVT (non sustained ventricular tachycardia), NTproBNP (N-terminal pro-brain natriuretic peptide), PET (positron emission tomography), RWMA (Non scar-related regional wall motion abnormalities), SSFP (steady-state free precession), Tn (troponin), WMA (wall motion abnormalities)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to European Journal of Internal Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Jennette JC
        • Falk RJ
        • Bacon PA
        • et al.
        2012 revised international Chapel Hill consensus conference nomenclature of vasculitides.
        Arthritis Rheumatism. 2013; https://doi.org/10.1002/art.37715
        • Comarmond C
        • Pagnoux C
        • Khellaf M
        • et al.
        Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French vasculitis study group cohort.
        Arthritis Rheum. 2013; https://doi.org/10.1002/art.37721
        • Sinico RA
        • Di Toma L
        • Maggiore U
        • et al.
        Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.
        Arthritis Rheum. 2005; https://doi.org/10.1002/art.21250
        • Sablé-Fourtassou R
        • Cohen P
        • Mahr A
        • et al.
        Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.
        Ann Intern Med. 2005; https://doi.org/10.7326/0003-4819-143-9-200511010-00006
        • Healy B
        • Bibby S
        • Steele R
        • Weatherall M
        • Nelson H
        • Beasley R.
        Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome.
        J Allergy Clin Immunol. 2013; https://doi.org/10.1016/j.jaci.2012.05.058
        • Keogh KA
        • Specks U.
        Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists.
        Am J Med. 2003; https://doi.org/10.1016/S0002-9343(03)00359-0
        • Vaglio A
        • Buzio C
        • Zwerina J.
        Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art.
        Allergy. 2013; https://doi.org/10.1111/all.12088
        • Valent P
        • Klion AD
        • Horny HP
        • et al.
        Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes.
        J Allergy Clin Immunol. 2012; https://doi.org/10.1016/j.jaci.2012.02.019
        • Davies DJ
        • Moran JE
        • Niall JF
        • Ryan GB.
        Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?.
        Br Med J. 1982; https://doi.org/10.1136/bmj.285.6342.606
        • Falk RJ
        • Jennette JC.
        Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis.
        N Engl J Med. 1988; https://doi.org/10.1056/NEJM198806233182504
        • Hellemans S
        • Dens J
        • Knockaert D.
        Coronary involvement in the Churg-Strauss syndrome.
        1997https://doi.org/10.1136/hrt.77.6.576 (Heart)
        • Cohen A
        • Johnson N
        • Chauvel C
        • et al.
        Segmental myocarditis in Churg Strauss syndrome. A case report and review. La Rev Med interne.
        1995https://doi.org/10.1016/0248-8663(96)80665-5
        • Rothenberg ME.
        Eosinophilia.
        N Engl J Med. 1998; https://doi.org/10.1056/NEJM199805283382206
        • Mahr A
        • Guillevin L
        • Poissonnet M
        • Aymé S.
        Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate.
        Arthritis Care Res (Hoboken). 2004; https://doi.org/10.1002/art.20077
      1. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999. doi:10.1097/00005792-199901000-00003.

      2. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Med (United States). 1984. doi:10.1097/00005792-198403000-00001.

        • Moosig F
        • Bremer JP
        • Hellmich B
        • et al.
        A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients.
        Ann Rheum Dis. 2013; https://doi.org/10.1136/annrheumdis-2012-201531
        • Masi AT
        • Hunder GG
        • Lie JT
        • et al.
        The American college of rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
        Arthritis Rheum. 1990; https://doi.org/10.1002/art.1780330806
        • Groh M
        • Pagnoux C
        • Baldini C
        • et al.
        European journal of internal medicine eosinophilic granulomatosis with polyangiitis (Churg – Strauss) (EGPA) consensus task force recommendations for evaluation and management.
        Eur J Intern Med. 2015; 26: 545-553https://doi.org/10.1016/j.ejim.2015.04.022
        • Caforio ALP
        • Pankuweit S
        • Arbustini E
        • et al.
        Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European society of cardiology working group on myocardial and pericardial diseases.
        Eur Heart J. 2013; https://doi.org/10.1093/eurheartj/eht210
        • Adler Y
        • Charron P
        • Imazio M
        • et al.
        2015 ESC guidelines for the diagnosis and management of pericardial diseases.
        Eur Heart J. 2015; https://doi.org/10.1093/eurheartj/ehv318
        • HUNSAKER JC
        • WN O'CONNOR
        • JT LIE
        Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg-Strauss syndrome.
        Mayo Clin Proc. 1992; https://doi.org/10.1016/S0025-6196(12)60801-5
      3. Andre J, Val-bernal JF, Mayorga M, Garcı E. Churg – Strauss syndrome and sudden cardiac death. 2003;12:94-97. doi:10.1016/S1054-8807(02)00157-6.

        • D. S, A. S, F. S, S.A. Z, D. D
        Sudden death due to autoimmune vasculitis.
        Rom J Leg Med. 2004;
        • Schön C
        • Ramaswamy A
        • Barth PJ.
        Churg-Strauss syndrome. Sudden cardiac death of a young woman.
        Rechtsmedizin, 2005https://doi.org/10.1007/s00194-005-0316-4
        • Setoguchi M
        • Okishige K
        • Sugiyama K
        • et al.
        Sudden cardiac death associated with Churg-Strauss syndrome.
        Circ J. 2009; https://doi.org/10.1253/circj.CJ-08-0926
        • CHURG J
        • STRAUSS L.
        Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.
        Am J Pathol. 1951;
      4. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis Nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore). 1996. doi:10.1097/00005792-199601000-00003.

      5. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin P Le. The five-factor score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French vasculitis study group (FVSG) cohort. Medicine (Baltimore). 2011. doi:10.1097/MD.0b013e318205a4c6.

        • Dennert RM
        • Paassen P
        • Van
        • Schalla S
        • et al.
        Cardiac Involvement in Churg-Strauss Syndrome. 2010; 62: 627-634https://doi.org/10.1002/art.27263
      6. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in churg-strauss syndrome impact of endomyocarditis. 2009;88(4):236-243. doi:10.1097/MD.0b013e3181af35a5.

        • Jeong HC
        • Kim KH
        • Cho JY
        • Song JE.
        Cardiac involvement of churg-strauss syndrome as a reversible cause of dilated cardiomyopathy.
        2015
        • Ramakrishna G
        • Connolly HM
        • Tazelaar HD
        • Mullany CJ
        • Midthun DE.
        Churg-Strauss syndrome complicated by eosinophilic endomyocarditis.
        Mayo Clin Proc. 2000; https://doi.org/10.4065/75.6.631
        • Francesco A
        • Pedrotti P
        • De Capitani L
        • Giannattasio C
        • Roghi A
        European journal of internal medicine comprehensive evaluation of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) with cardiac magnetic resonance.
        Eur J Intern Med. 2016; : 1-6https://doi.org/10.1016/j.ejim.2016.09.014
      7. Masaki N, Issiki A, Kirimura M, Kamiyama T, Sasaki O. Echocardiographic changes in eosinophilic endocarditis induced by Churg-Strauss syndrome. 2016:2819-2823. doi:10.2169/internalmedicine.55.7150.

        • Sofia Correia A
        • Gonçalves A
        • Araújo V
        • et al.
        Churg-Strauss syndrome presenting with eosinophilic myocarditis: a diagnostic challenge.
        Rev Port Cardiol. 2013; https://doi.org/10.1016/j.repc.2012.10.017
      8. Cottin V, Bel E, Bottero P, et al. Autoimmunity Reviews Revisiting the systemic vasculitis in eosinophilic granulomatosis with A study of 157 patients by the Groupe d ’ Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinoph. 2017;16:1-9.

      9. Vinit J, Bielefeld P, Muller G, et al. European journal of internal medicine heart involvement in Churg – Strauss syndrome : retrospective study in French Burgundy population in past 10 years. 2010;21:341-346. doi:10.1016/j.ejim.2010.05.004.

        • Petrakopoulou P
        • Franz WM
        • Boekstegers P
        • Weis M.
        Vasospastic angina pectoris associated with Churg-Strauss syndrome.
        Nat Clin Pract Cardiovasc Med. 2005; https://doi.org/10.1038/ncpcardio0299
        • S. T, R. K, K. S
        Immunosuppressive therapy ameliorates severe pulmonary hypertension and refractory vasospastic angina due to eosinophilic granulomatosis with polyangiitis.
        Am J Respir Crit Care Med. 2018;
      10. Buhaescu I, Williams A, Yood R. Rare manifestations of Churg – Strauss syndrome : coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness — a case report. 2009:231-233. doi:10.1007/s10067-008-1052-4.

        • Greulich S
        • Mayr A
        • Kitterer D
        • et al.
        T1 and T2 mapping for evaluation of myocardial involvement in patients with ANCA-associated vasculitides.
        J Cardiovasc Magn Reson. 2017; : 1-12https://doi.org/10.1186/s12968-016-0315-5