Rituximab and Dupilumab Improve Eosinophilic Granulomatosis With Polyangiitis With Multiple Pulmonary Thrombi

2021 ◽  
Author(s):  
Sei Adachi ◽  
Chiyako Oshikata ◽  
Takeshi Kaneko ◽  
Naomi Tsurikisawa
Keyword(s):  
Heart Failure ◽  
Granulomatosis With Polyangiitis ◽  
Disease Onset ◽  
Gastrointestinal Symptoms ◽  
Patient Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Oxygen Inhalation ◽  
First Case ◽  
Venous Thromboembolic Events ◽  
Eosinophilic Granulomatosis

Abstract BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is characterized by a necrotizing vasculitis with tissue and peripheral blood eosinophilia affecting small and medium-sized arteries, capillaries, and veins. Venous thromboembolic events have occurred in 19 of 232 (8.2%) patients with EGPA. However, there are only a few reported cases of EGPA complicated by pulmonary embolism or infarction. Case presentationWe report the case of a 43-year-old woman with eosinophilic granulomatosis with polyangiitis patient with acute respiratory and heart failure due to bilateral pulmonary artery thrombosis and left femoral vein thrombosis in addition to cardiac involvement as myocarditis, pericardial effusion, and diastolic dysfunction, gastrointestinal symptoms and peripheral neuropathy 12 years after disease onset. The condition was refractory to treatment with systemic corticosteroids, intravenous cyclophosphamide, and mepolizumab, but the acute cardiac failure associated with the thrombosis, cardiac and gastrointestinal symptoms, and multiple polyneuropathy improved after a switch to rituximab. But her heart failure did not improve sufficiently, she continued to need oxygen inhalation at 1 L/min and asthma exacerbations occurred. We changed the patient’s treatment with mepolizumab to dupilumab. Not only did she have no asthma attacks after switching to dupilumab, but also her vasculitis symptoms improved. Oxygen therapy was discontinued as heart failure improved five months after starting the dupilumab. ConclusionsThis may be the first case report of the successful treatment of pulmonary thromboembolism associated with EGPA by rituximab. In addition, in this patient, treatment with dupilumab was effective not only for the asthma symptoms but also for the symptoms of vasculitis.

scholarly journals Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Dan Zhu ◽  
Yiming Luo ◽  
Xiangyuan Liu ◽  
Lingyun Zu
Keyword(s):  
Heart Failure ◽  
Granulomatosis With Polyangiitis ◽  
Thrombus Formation ◽  
Systemic Vasculitis ◽  
Anticoagulation Therapy ◽  
Cardiac Complications ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
First Case ◽  
Thrombogenic Potential ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases. We also hypothesize that the thrombogenic potential of eosinophils may play a central role in thrombogenesis in EGPA and aggressive immunosuppressive therapy remains the cornerstone of treatment, and the addition of anticoagulation therapy in the setting of thrombus formation and also very high risk of bleeding needs to be considered cautiously.

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

2021 ◽  
Vol 10 (19) ◽  
pp. 4577
Author(s):  
Chrong-Reen Wang ◽  
Yi-Shan Tsai ◽  
Hung-Wen Tsai ◽  
Cheng-Han Lee
Keyword(s):  
B Cell ◽  
Clinical Remission ◽  
Granulomatosis With Polyangiitis ◽  
Disease Onset ◽  
Cardiac Insufficiency ◽  
Induction Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Asthma Attack ◽  
Eosinophilic Granulomatosis ◽  
Eosinophil Counts

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.

scholarly journals Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis

2018 ◽  
Vol 45 (8) ◽  
pp. 1159-1166 ◽  
Author(s):  
Aiko Saku ◽  
Shunsuke Furuta ◽  
Masaki Hiraguri ◽  
Kei Ikeda ◽  
Yoshihisa Kobayashi ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Proportional Hazards ◽  
Proportional Hazards Model ◽  
Disease Onset ◽  
Japanese Patients ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Factors Associated ◽  
Hazards Model ◽  
Eosinophilic Granulomatosis ◽  
Eosinophil Counts

Objective.Patients with eosinophilic granulomatosis with polyangiitis (EGPA) frequently experience relapses, which lead to cumulative organ damage. In this retrospective observational study, we aimed to reveal the risk factors for relapse in EGPA.Methods.A total of 188 Japanese patients with EGPA diagnosed between 1996 and 2015 were identified from medical records in 10 hospitals. The diagnosis was based on the American College of Rheumatology 1990 criteria or Lanham’s criteria. Baseline characteristics, treatments, asthma exacerbation, and relapses were evaluated by retrospective chart review.Results.The median followup period was 56 months. The median age at disease onset was 59.7 years. At the disease onset, 95.2% of the patients had a history of bronchial asthma and 44.7% were positive for antineutrophil cytoplasmic antibodies. The cumulative survival and relapse-free survival rates at 5 years were 89.6% and 64.0%, respectively. Multivariate analysis with 2 models, proportional hazards, and competing risk models, was performed to identify the factors associated with relapse. The proportional hazards model identified azathioprine (AZA) maintenance therapy and high eosinophil counts at onset as independent factors with lower relapse risks, and high immunoglobulin E (IgE) levels at onset as a risk factor for relapse. The competing risk model identified no statistically significant factors.Conclusion.Although potential benefit of AZA maintenance therapy in preventing relapse of EGPA was suggested by the proportional hazards model, there was a discrepancy in the results between the models. Eosinophil counts and IgE levels at onset were also identified as candidates of factors associated with relapse in EGPA.

scholarly journals Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors

2021 ◽  
Vol 12 ◽  
pp. 204062232098705
Author(s):  
Suying Liu ◽  
Ling Guo ◽  
Zhaocui Zhang ◽  
Mengtao Li ◽  
Xiaofeng Zeng ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pericardial Effusion ◽  
Eosinophil Count ◽  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Single Center ◽  
Myocardial Involvement ◽  
Cardiac Manifestations ◽  
Eosinophilic Granulomatosis

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes. Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves. Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 109/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes ( p = 0.131) and survival ( p = 0.1972) were not significantly different between the groups. Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.

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