Acute Myocarditis in Patients with Antineutrophil Cytoplasmic Antibody–positive Microscopic Polyangiitis and Receiving Rituximab Therapy

Wang, Chrong‐Reen; Tsai, Yi Shan; Tsai, Hung Wen

doi:10.3899/jrheum.190569

Cited by 6 publications

(8 citation statements)

References 10 publications

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“…Under the approval of the Institutional Review Board, patients admitted to Departments of Internal Medicine and Pediatrics and ful lling 1990 American College of Rheumatology (ACR) criteria for the EGPA classi cation [17], were analyzed from January 1, 2008 to December 31, 2019. Myocarditis was diagnosed according to the following criteria: (1) presenting symptoms consistent with heart failure, (2) raised concentrations of cardiac biomarkers, and 3new or worsening changes including impaired left ventricle ejection fraction (LVEF, mild 46 to 55%, moderate 30 to 45%, severe below 30%) or plus wall motion abnormality on transthoracic echocardiography (ECG) or cardiac magnetic resonance imaging (cMRI) [15,16,18]. The additional endocardial involvement, i.e., endomyocarditis, was detected by cMRI.…”

Section: Patients Enrollmentmentioning

confidence: 99%

“…Cardiac rhythm was examined by 24-hour Holter monitor. Exclusion criterion was coronary artery disease with characterized ndings in coronary angiography or cMRI, viral myocarditis with a con rmed history of infection, or preexisting heart diseases under medical therapy [15,16].…”

Section: Patients Enrollmentmentioning

confidence: 99%

“…Demographical, clinical, laboratory, imaging and pathological data were analyzed, including age/sex, EGPA manifestations, disease activity (Birmingham Vasculitis Activity Score, BVAS) [19], prognosis assessment [5], myocarditis symptoms [15,16], New York Heart Association Functional Classi cation (NYHAFC), ANCA titers (immuno uorescence, enzyme-linked immunosorbent assay), eosinophil counts, C-reactive protein (CRP) values, IgE levels, circulating B-cell numbers (CD19-positive lymphocytes), cardiac biomarker concentrations (cardiac troponin I, creatine kinase-MB, N-terminal pro-brain natriuretic peptide), cardiac image and rhythm ndings, and pathological results. There was a comprehensive review in medication pro les including CS, immunosuppressive agents, biologics and cardiac medications with antiarrhythmic drugs for cardiac dysrhythmia and cardiac supportive agents (CSA) (angiotensin converting enzyme inhibitor, angiotensin-receptor blocker, β blocker, diuretic, inotrope) for heart failure.…”

Section: Data Collectionmentioning

confidence: 99%

“…Therapeutic responses to rituximab (RTX), a B celldepleting mAb, have been observed in EGPA, and it has been recommended at induction for refractory activity or relapsing disease with a potential bene t in ANCA negativity [13]. Interestingly, MEP has been suggested to treat eosinophilic myocarditis in hypereosinophilic syndrome (HES) [14], and therapeutic effects of RTX on autoimmune myocarditis have been observed in microscopic polyangiitis and systemic lupus erythematosus [15,16].…”

Section: Introductionmentioning

confidence: 99%

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Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

Wang

Tsai

Wang

et al. 2020

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Background Cardiac insufficiency is a major cause of mortality in eosinophilic granulomatosis with polyangiitis (EGPA). Despite the dosages-related cardiotoxicity, cyclophosphamide is usually prescribed to induce disease remission in the presence of myocarditis with heart involvement. There is an imperative need of novel medications to efficiently control disease activity and spare the use of cyclophosphamide. Methods A retrospective study was carried out in hospitalized EGPA patients from January 1, 2008 to December 31, 2019, focusing on the use of biologics including benralizumab (BEN, anti-IL-5 receptor), mepolizumab (MEP, anti-IL-5), omalizumab (OMA, anti-IgE) and rituximab (RTX, anti-CD20). Results Sixteen admitted patients, 8 females aged 10 to 70 years (40.4 ± 15.5), had higher disease activities (Birmingham Vasculitis Activity Score 16 to 39, 26.8 ± 6.9), poorer prognostic factors (five-factor score 1 or 2, 1.4 ± 0.5) and elevated eosinophil counts (2,314 to 26,781/µL, 11,108 ± 7,060). BEN, MEP, OMA and RTX were prescribed in one, 2, one and 6 patients, respectively. Ten patients (63%) had myocarditis with impaired left ventricle ejection fraction and cardiac arrhythmia, and 7 received biological therapy without a combined use of cyclophosphamide. One patient obtained MEP with a 100 mg quadri-weekly × 13 regimen at induction for disease relapse. Six patients acquired RTX with a 375 mg/m2 weekly × 4 regimen at induction for refractory activity or relapsing disease, or plus a yearly maintenance schedule in 5. All patients received serial cardiac magnetic resonance imaging, transthoracic echocardiography and 24-hour Holter monitor to evaluate the therapeutic responses in heart involvement. After biological therapy, there were improved cardiac dysfunction, lower eosinophil counts and clinical remission (4 complete, 3 partial) with a relapse-free follow-up (13 to 61 months, 39.1 ± 16.0) after induction. Conclusions In this single-center retrospective study, we observed improved cardiac dysfunction and disease activity after biological therapy in EGPA patients with myocarditis.

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Section: Patients Enrollmentmentioning

confidence: 99%

Section: Patients Enrollmentmentioning

confidence: 99%

Section: Data Collectionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

Wang

Tsai

Wang

et al. 2020

Preprint

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“…Current evidence supports the use of an anti-IL-5 mAb, mepolizumab (MEP), for the induction treatment of active or relapsing EGPA with eosinophilia [ 8 , 11 , 14 ]. Moreover, the therapeutic efficacy of RTX in overt myocarditis was observed in microscopic polyangiitis, another seropositive AAV disorder [ 15 , 16 ]. In hypereosinophilic syndrome (HES), MEP is recommended to be used in the cardiac manifestation, eosinophilic myocarditis [ 17 ].…”

Section: Introductionmentioning

confidence: 99%

B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis

Wang

Tsai

et al. 2021

JCM

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Cardiac involvement is a major mortality cause in eosinophilic granulomatosis with polyangiitis (EGPA), requiring novel therapeutics to spare the use of cyclophosphamide with known cardiotoxicity. Despite the observed efficacy of B-cell-depleting therapy in myocarditis of seropositive microscopic polyangiitis, it remains to be elucidated in seronegative EGPA. A retrospective study was performed in 21 hospitalized active patients aged 20 to 70 years with five-factor score 1 or 2, eosinophil counts 10,034 ± 6641/μL and vasculitis scores 27 ± 6. Overt myocarditis was identified in 10 cases, at disease onset in 6 and relapse in 4, with endomyocarditis in 4 and myopericarditis in 4. Five seronegative and one seropositive patient received rituximab with an induction regimen 375 mg/m2 weekly × 4 for refractory or relapse disease, and the same regimen for annual maintenance therapy. All cases had lower eosinophil counts, improved cardiac dysfunction and clinical remission with a relapse-free follow-up, 48 ± 15 months after the induction treatment. One seronegative endomyocarditis patient had eosinophilia and disease relapse with asthma attack and worsening cardiac insufficiency 24 months after induction, achieving clinical remission under anti-IL-5 therapy. Our findings suggest the suppression of IL-5-mediated eosinophilia as an action mechanism of B-cell-depleting therapy in seronegative EGPA myocarditis.

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New Advances and Ongoing Challenges in the Use of Biologic Agents in Cardiac Sarcoidosis and Other Inflammatory Cardiomyopathies

Taimeh

Tang

2021

Curr Treat Options Cardio Med

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Acute Myocarditis in Patients with Antineutrophil Cytoplasmic Antibody–positive Microscopic Polyangiitis and Receiving Rituximab Therapy

Cited by 6 publications

References 10 publications

Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis

New Advances and Ongoing Challenges in the Use of Biologic Agents in Cardiac Sarcoidosis and Other Inflammatory Cardiomyopathies

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