Il coinvolgimento cardiaco nella sindrome di Churg-Strauss

Translated title of the contribution: Cardiac involvement in Churg-Strauss syndrome

Antonio Brucato, Silvia Maestroni, Gabriella Masciocco, Enrico Ammirati, Edgardo Bonacina, Patrizia Pedrotti

Research output: Contribution to journalArticle

Abstract

Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and mediumsized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10[%] on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60[%] of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60[%] of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40[%] of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50[%] of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive agents, particularly cyclophosphamide in case of myocardial inflammation. Thus, early diagnosis of cardiac involvement and subsequent therapy may prevent progression of cardiac disease. At present, the role of troponin and brain natriuretic peptide in monitoring and therapy remains unclear. Orthotopic heart transplantation is feasible in case of severe disease, even if the experience is limited in EGPA, and optimal post-transplantation immunosuppressive strategy has yet to be defined.

Translated title of the contributionCardiac involvement in Churg-Strauss syndrome
Original languageItalian
Pages (from-to)493-500
Number of pages8
JournalGiornale Italiano di Cardiologia
Volume16
Issue number9
Publication statusPublished - Sep 1 2015

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Medicine(all)

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  • Cite this

    Brucato, A., Maestroni, S., Masciocco, G., Ammirati, E., Bonacina, E., & Pedrotti, P. (2015). Il coinvolgimento cardiaco nella sindrome di Churg-Strauss. Giornale Italiano di Cardiologia, 16(9), 493-500.