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
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References
- 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides.Arthritis Rheumatism. 2013; https://doi.org/10.1002/art.37715
- 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
- Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.Arthritis Rheum. 2005; https://doi.org/10.1002/art.21250
- Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.Ann Intern Med. 2005; https://doi.org/10.7326/0003-4819-143-9-200511010-00006
- 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
- 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
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art.Allergy. 2013; https://doi.org/10.1111/all.12088
- 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
- Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?.Br Med J. 1982; https://doi.org/10.1136/bmj.285.6342.606
- 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
- Coronary involvement in the Churg-Strauss syndrome.1997https://doi.org/10.1136/hrt.77.6.576 (Heart)
- 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
- Eosinophilia.N Engl J Med. 1998; https://doi.org/10.1056/NEJM199805283382206
- 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
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.
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.
- 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
- 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
- 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
- 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
- 2015 ESC guidelines for the diagnosis and management of pericardial diseases.Eur Heart J. 2015; https://doi.org/10.1093/eurheartj/ehv318
- 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
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.
- Sudden death due to autoimmune vasculitis.Rom J Leg Med. 2004;
- Churg-Strauss syndrome. Sudden cardiac death of a young woman.Rechtsmedizin, 2005https://doi.org/10.1007/s00194-005-0316-4
- Sudden cardiac death associated with Churg-Strauss syndrome.Circ J. 2009; https://doi.org/10.1253/circj.CJ-08-0926
- Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.Am J Pathol. 1951;
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.
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.
- Cardiac Involvement in Churg-Strauss Syndrome. 2010; 62: 627-634https://doi.org/10.1002/art.27263
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.
- Cardiac involvement of churg-strauss syndrome as a reversible cause of dilated cardiomyopathy.2015
- Churg-Strauss syndrome complicated by eosinophilic endomyocarditis.Mayo Clin Proc. 2000; https://doi.org/10.4065/75.6.631
- 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
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.
- Churg-Strauss syndrome presenting with eosinophilic myocarditis: a diagnostic challenge.Rev Port Cardiol. 2013; https://doi.org/10.1016/j.repc.2012.10.017
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.
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.
- Vasospastic angina pectoris associated with Churg-Strauss syndrome.Nat Clin Pract Cardiovasc Med. 2005; https://doi.org/10.1038/ncpcardio0299
- Immunosuppressive therapy ameliorates severe pulmonary hypertension and refractory vasospastic angina due to eosinophilic granulomatosis with polyangiitis.Am J Respir Crit Care Med. 2018;
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.
- 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
Article info
Publication history
Published online: December 22, 2020
Accepted:
December 8,
2020
Received in revised form:
November 24,
2020
Received:
July 24,
2020
Identification
Copyright
© 2020 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
