scholarly journals Case Report: Area of Focus of Myocardial Infarction With Non-obstructive Coronary Arteries in Eosinophilic Granulomatosis With Polyangiitis

2021 ◽  
Vol 8 ◽  
Author(s):  
Xiaoxian Cui ◽  
Yang Peng ◽  
Jun Liu ◽  
Yugang Dong ◽  
Zexuan Wu ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Coronary Arteries ◽  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Acute Chest Pain ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Inferior Myocardial Infarction ◽  
Gadolinium Enhancement ◽  
Eosinophilic Granulomatosis

Background: Eosinophilic granulomatosis with polyangitis manifested as myocardial infarction with non-obstructed coronary arteries (MINOCA) is rarely reported.Case: We report a 43-year-old male patient without any cardiovascular risk factors presenting with acute chest pain. Electrocardiogram was suggestive of acute anterior and inferior myocardial infarction. MINOCA was confirmed based on significant elevated cardiac troponin and normal coronary arteries. Cardiac magnetic resonance (CMR) imaging revealed extended late gadolinium enhancement (LGE). Further diagnosis of eosinophilic granulomatosis with polyangitis (EGPA) was based on clinical manifestations and auxiliary examination. Subsequent immunosuppressive therapy led to regression of symptoms and significant resolution of LGE on CMR.Conclusion: Our case highlights that EGPA can be a rare cause of MINOCA. CMR is useful for differentiation diagnosis and evaluation of cardiac involvement.

scholarly journals Clinical case of eosinophilic granulomatous polyangiitis debuted by myocardial infarction

2021 ◽  
Vol 40 (2) ◽  
pp. 41-48
Author(s):  
Evgeniy V. Kryukov ◽  
Dmitriy V. Cherkashin ◽  
Sergey L. Grishaev ◽  
Sayora A. Turdialieva ◽  
Elena A. Mozharovskaya ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Therapy Strategy ◽  
Granulomatous Polyangiitis ◽  
Diagnostic Biopsy ◽  
Eosinophilic Granulomatosis

The article presents clinical observations demonstrating the difficulties of diagnostic search in establishing the diagnosis of systemic vasculitis associated with antineutrophil cytoplasmic antibodies, which include a rare disease eosinophilic granulomatosis with polyangiitis (ChurgStrauss syndrome). Carefully collected anamnesis, participation of specialists of different profiles, retrospective analysis of laboratory and instrumental data allowed to verify the diagnosis, to prescribe adequate therapy. The aim of the publication is to discuss the need for early diagnosis of eosinophilic granulomatosis with polyangiitis, which can improve the effectiveness of therapy and improve the overall prognosis for this disease, taking into account modern approaches based on the main provisions of international recommendations that were prepared in 2015 with the participation of leading experts from Europe, USA and Canada and were called to become the basis for choosing a personalized patient therapy strategy. In some cases, the diagnosis of eosinophilic granulomatous polyangiitis is complicated by the diversity of the clinical picture, the non-simultaneous appearance of the main symptoms and the violation of the stages of the process. In the differential diagnosis of systemic vasculitis, assessment of initial clinical manifestations, testing for the presence of antibodies to the cytoplasm of neutrophils, multispiral computed tomography of the chest organs and diagnostic biopsy of the affected tissues are crucial. In the histological conclusion, a picture of focal ulcerative-necrotic lesions of the nasal mucosa with signs of vasculitis and a pronounced eosinophilic cell component of inflammation was noted. The distinctive features of this case include the onset of the disease with the development of myocardial infarction and early damage to the hearing organ in the form of cochlear neuritis, complicated by sensorineural hypoacusis. The combination of high doses of glucocorticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative (4 figs, bibliography: 3 refs).

scholarly journals CARDIAC INVOLVEMENT IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A CHALLENGING CASE REPORT

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 ◽  
...  
Keyword(s):  
Case Report ◽  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

scholarly journals Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

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

2020 ◽  
Author(s):  
Mojdeh Pakbaz ◽  
Marziyeh Pakbaz-
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Case Reports ◽  
Laboratory Data ◽  
Left Ventricular ◽  
Cardiac Biomarkers ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Cardiac Symptoms ◽  
Eosinophilic Granulomatosis

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.

Comparison of patient self-reported data to physician-reported data in eosinophilic granulomatosis with polyangiitis (Preprint)

2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
The United States ◽  
Research Network ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Patient Reported ◽  
Reported Data ◽  
Eosinophilic Granulomatosis

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305

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