scholarly journals Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis: A Meta-analysis of 62 Case Reports

Author(s):  
Mojdeh Pakbaz ◽  
Marziyeh Pakbaz-

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-systemic vasculitis, with cardiac involvement being one of its most serious manifestations. We aimed to systematically review and analyze the limited case reports of EGPA with cardiac involvement. f EGPA with cardiac involvement. Methods: Based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we performed a systematic literature search for the case reports of EGPA with cardiac involvement in the MEDLINE database from 2011 until 2018. For each case, clinical data including sex, age, clinical presentation, electrocardiographic and cardiac imaging findings, the type of cardiac involvement, the available laboratory data (cardiac biomarkers, white blood cell count, eosinophilic count, erythrocyte sedimentation rate, C-reactive protein, and antineutrophil cytoplasmic antibody positivity), therapeutic regimen, and the outcome of the patients were collected and analyzed. Results: A total number of 62 cases were included. The mean age was 48.29±15.60 years, and 51.6% were male. All the cases were in the active disease state. Cardiac symptoms, electrocardiographic abnormalities, abnormal biomarkers, and abnormal echocardiography were detected in 82.3%, 68.5%, 77.4%, and 96.8%, respectively. Cardiac magnetic resonance was done in 46.8% of the patients, and it was abnormal in all. The most common abnormal findings in echocardiography were systolic left ventricular dysfunction (83.9%) and pericardial effusion (37.1%). The most common type of clinical presentation was clinical heart failure (51.6%). Only 6.5% of the patients presented with tamponade. The overall prognosis was good. Conclusion: Any part of the heart could be involved by EGPA. The results emphasize the necessity of in-depth cardiac evaluation in these patients.

2021 ◽  
Author(s):  
Larissa Maria Oliveira Gonzaga ◽  
Ana Luisa Bagno de Almeida ◽  
Aurivan Essado Dantas ◽  
Carolina Ruas Freire Santos ◽  
Anna Carolina Faria Moreira Gomes Tavares ◽  
...  

Rheumatology ◽  
2021 ◽  
Author(s):  
Vincenzo G Menditto ◽  
Giulia Rossetti ◽  
Diletta Olivari ◽  
Alessia Angeletti ◽  
Marco Rocchi ◽  
...  

Abstract Objective to analyze the available evidence about the use of rituximab (RTX) and other biologic agents in Eosinophilic Granulomatosis with Polyangiitis (EGPA) patients and to provide useful findings to inform the design of future, reliable clinical trials. Methods A systematic review was performed. A systematic search was conducted in PubMed/MEDLINE, Scopus, Web of Science and the Cochrane library databases and an extensive literature search on other biologic agents. Results 45 papers pertinent to our questions were found: 16 retrospective cohort studies, 8 case series, 3 prospective cohort studies and 18 single case reports, for a total of 368 EGPA patients. More than 80% of evaluable patients achieved complete or partial remission with a tendency towards a higher rate of complete response in pANCA positive subgroup. Conclusion Although the majority of the evaluable EGPA patients treated with RTX appears to achieve complete remission, we strongly believe that a number of sources of heterogeneity impairs a clear interpretation of results and limits their transferability in clinical practice. Differences in design, enrollment criteria, outcome definition and measurement make a comparison among data obtained from studies on RTX and other biologic agents unreliable.


Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii84-iii94 ◽  
Author(s):  
Giorgio Trivioli ◽  
Benjamin Terrier ◽  
Augusto Vaglio

Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e14011-e14011 ◽  
Author(s):  
Dana Ionescu ◽  
Danielle Stone ◽  
James Stone ◽  
Jean-Bernard Durand ◽  
Juan Lopez-Mattei ◽  
...  

e14011 Background:Variants of the classic Tako-tsubo syndrome or stress induced cardiomyopathy (SC) includes mid ventricular or basal left ventricular wall motion abnormalities. Midcavitary dyskinesia and ballooning is considered a unique presentation, and there is no published data showing midcavitary involvement in cancer patients. Methods: All cancer patients who fulfilled the diagnostic criteria for SC at MD Anderson Cancer Center over a 6–year period were included in the study. We selected and retrospectively reviewed the medical records of 8 patients who had midcavitary SC. Clinical presentation, ECG, laboratory data, transthoracic echocardiogram and left ventriculography results were reviewed. Results: Out of30 cancer patients who fulfilled the diagnostic criteria for SC, 8 patients (26.7%) (4 females, 4 men, mean age 57.37 yo) had midcavitary SC. 62,5 % patients were diagnosed with a solid malignancy. Trigger factors for midcavitary SC were: systemic infection (3 patients with neutropenia), emotional stress (2 patients), chemotherapy (1 patient undergoing treatment with Ibrutinib), and surgical interventions (3 patients). Clinical presentation included chest pain (37.5%), shortness of breath (50%) and non specific symptoms (12.5%). T wave inversion was the most common electrocardiographic presentation (37.5 %), followed by ST elevation (25 %). All patients had changes of the cardiac biomarkers (BNP mean 2224. 4 pg/dl, TN I mean 2. 8 ng/dl, CK-MB mean 14 ng/dl) and significant LV dysfunction (LVEF < 50%). All patients underwent coronary angiography which showed no obstructive CAD; left ventriculography identified basilar and apical hyperkinesis and midventricular hypokinesia. Cancer therapy was interrupted; aspirin and beta blockers were initiated in all patients. The most common complications of midcavitary SC were: respiratory failure requiring intubation (37, 5%), pulmonary edema (25%), and hypotension (25%). No cardiac deaths were registered. None of the patients experienced recurrence of SC. Conclusions: Mid cavitary SC remains a rare entity, and raises further questions about the causal association between the mid cavitary involvement and cancer, and its impact on cancer therapy and overall survival in this cohort of patients.


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