Dataset Information

Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.

ABSTRACT:

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by tissue and blood eosinophilia, vasculitis of small to medium-sized vessels, and allergy symptoms, and can cause various manifestations, including heart, lung, gastrointestinal, skin, and peripheral nerve disorders.

Case presentation

A 34-year-old woman with a history of asthma, nasal polyp, and sinusitis presented with ventricular fibrillation after severe chest pain. Emergent coronary angiography showed no coronary stenosis. After admission, she suffered from hypoxaemia and recurrent chest pain with ST-segment changes, suggesting vasospastic angina (VSA). Chest computed tomography (CT) showed centrilobular nodular shadows, suggesting bronchiolitis. Since she had hypereosinophilia, we administered oral prednisolone, which resulted in improvements of hypereosinophilia, hypoxaemia, and recurrent chest pains in 3 days. Right heart catheterization showed severe pulmonary hypertension (PH) with a mean pulmonary artery pressure (mPAP) of 48?mmHg and pulmonary vascular resistance (PVR) of 12 Wood units (WU). Ergonovine provocation test induced severe diffuse spasm of the left coronary artery including the left main trunk. Based on asthma, sinusitis, hypereosinophilia, and chest CT findings, the diagnosis of EGPA associated with VSA and PH was made. Thereafter, we started intravenous cyclophosphamide (IV-CY) pulse therapy in addition to prednisolone and pulmonary vasodilators. Six months after IV-CY therapy, mPAP and PVR decreased to 34?mmHg and 5.1 WU, respectively. Moreover, repeated ergonovine provocation test was negative without coronary spasm or electrocardiogram (ECG) changes.

Discussion

This case indicates that EGPA can cause severe PH, refractory VSA, and bronchiolitis, which could be markedly improved by treating underlying conditions with immunosuppressive therapy.

SUBMITTER: Konno R

PROVIDER: S-EPMC6177055 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature

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Publications

Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.

Konno Ryo R Tatebe Shunsuke S Shirai Tsuyoshi T Shimokawa Hiroaki H

European heart journal. Case reports 20180425 2

<h4>Introduction</h4>Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by tissue and blood eosinophilia, vasculitis of small to medium-sized vessels, and allergy symptoms, and can cause various manifestations, including heart, lung, gastrointestinal, skin, and peripheral nerve disorders.<h4>Case presentation</h4>A 34-year-old woman with a history of asthma, nasal polyp, and sinusitis presented with ventricular fibrillation after severe chest pain. Emergent coronary angiograph ...[more]

PMID: 31020129

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Project description:BackgroundEosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti-interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis.MethodsIn this multicenter, double-blind, parallel-group, phase 3 trial, we randomly assigned participants with relapsing or refractory eosinophilic granulomatosis with polyangiitis who had received treatment for at least 4 weeks and were taking a stable prednisolone or prednisone dose to receive 300 mg of mepolizumab or placebo, administered subcutaneously every 4 weeks, plus standard care, for 52 weeks. The two primary end points were the accrued weeks of remission over a 52-week period, according to categorical quantification, and the proportion of participants in remission at both week 36 and week 48. Secondary end points included the time to first relapse and the average daily glucocorticoid dose (during weeks 48 through 52). The annualized relapse rate and safety were assessed.ResultsA total of 136 participants underwent randomization, with 68 participants assigned to receive mepolizumab and 68 to receive placebo. Mepolizumab treatment led to significantly more accrued weeks of remission than placebo (28% vs. 3% of the participants had ≥24 weeks of accrued remission; odds ratio, 5.91; 95% confidence interval [CI], 2.68 to 13.03; P<0.001) and a higher percentage of participants in remission at both week 36 and week 48 (32% vs. 3%; odds ratio, 16.74; 95% CI, 3.61 to 77.56; P<0.001). Remission did not occur in 47% of the participants in the mepolizumab group versus 81% of those in the placebo group. The annualized relapse rate was 1.14 in the mepolizumab group, as compared with 2.27 in the placebo group (rate ratio, 0.50; 95% CI, 0.36 to 0.70; P<0.001). A total of 44% of the participants in the mepolizumab group, as compared with 7% of those in the placebo group, had an average daily dose of prednisolone or prednisone of 4.0 mg or less per day during weeks 48 through 52 (odds ratio, 0.20; 95% CI, 0.09 to 0.41; P<0.001). The safety profile of mepolizumab was similar to that observed in previous studies.ConclusionsIn participants with eosinophilic granulomatosis with polyangiitis, mepolizumab resulted in significantly more weeks in remission and a higher proportion of participants in remission than did placebo, thus allowing for reduced glucocorticoid use. Even so, only approximately half the participants treated with mepolizumab had protocol-defined remission. (Funded by GlaxoSmithKline and the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov number, NCT02020889 .).

Project description:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease characterized by eosinophil-rich granulomatous inflammation and necrotizing vasculitis, pre-dominantly affecting small-to-medium-sized vessels. It is categorized as a primary antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) but also shares features of hypereosinophilic syndrome (HES); therefore, both vessel inflammation and eosinophilic infiltration are suggested to cause organ damage. This dual nature of the disease causes variable clinical presentation. As a result, careful differentiation from mimicking conditions is needed, especially from HES, given the overlapping clinical, radiologic, and histologic features, and biomarker profile. EGPA also remains a diagnostic challenge, in part because of asthma, which may pre-dominate for years, and often requires chronic corticosteroids (CS), which can mask other disease features. The pathogenesis is still not fully understood, however, the interaction between eosinophils and lymphocytes B and T seems to play an important role. Furthermore, the role of ANCA is not clear, and only up to 40% of patients are ANCA-positive. Moreover, two ANCA-dependent clinically and genetically distinct subgroups have been identified. However, a gold standard test for establishing a diagnosis is not available. In practice, the disease is mainly diagnosed based on the clinical symptoms and results of non-invasive tests. The unmet needs include uniform diagnostic criteria and biomarkers to help distinguish EGPA from HESs. Despite its rarity, notable progress has been made in understanding the disease and in its management. A better understanding of the pathophysiology has provided new insights into the pathogenesis and therapeutic targets, which are reflected in novel biological agents. However, there remains an ongoing reliance on corticosteroid therapy. Therefore, there is a significant need for more effective and better-tolerated steroid-sparing treatment schemes.

Project description:ObjectiveMepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort.MethodsWe included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ≤4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations.ResultsTwo hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44).ConclusionMepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.

Dataset Information

Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.

Introduction

Case presentation

Discussion

Publications

Immunosuppressive therapy ameliorates refractory vasospastic angina, severe pulmonary hypertension, and bronchiolitis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.

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