scholarly journals Successful treatment with benralizumab in a patient with eosinophilic granulomatosis with polyangiitis refractory to mepolizumab

2021 ◽  
Vol 16 ◽  
Author(s):  
Francesco Menzella ◽  
Carla Galeone ◽  
Giulia Ghidoni ◽  
Patrizia Ruggiero ◽  
Silvia Capobelli ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulmonary Function Tests ◽  
Treatment Options ◽  
Granulomatous Inflammation ◽  
Skin Lesions ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Clinical Effects ◽  
Frequent Relapses ◽  
Eosinophilic Granulomatosis

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing eosinophilic granulomatous inflammation that frequently involves the respiratory tract (90% of cases). Asthma in EGPA is systematically severe and often refractory to common treatment, it is corticosteroid resistant and can often anticipate the onset of systemic vasculitis by many years. A release of cytokines necessary for the activation, maturation and survival of eosinophils, such as IL-4, IL-5 and IL-13 occurs in the activated Th-2 phenotype. In particular, IL-5 level is high in active EGPA and its inhibition has become a key therapeutic target. Oral glucocorticoids (OCS) are effective treatment options but unfortunately, frequent relapses occur in many patients and they lead to frequent side effects. As for now, there are currently no official recommendations on doses and treatment schedules in the management of EGPA.Case presentation: In this article, we describe the case of a man with EGPA, severe asthma and chronic rhinosinusitis with nasal polyps (CRSwNP), with poor asthma and CRSwNP control despite OCS and mepolizumab treatment. Respiratory and vasculitis symptoms improved markedly after therapeutic switch to benralizumab. During the treatment, in addition to clinical effects, we witnessed a depletion of blood eosinophils, as well as an improvement in both pulmonary function tests, CT scan and skin lesions present initially. Conclusions: While there are many studies confirming the efficacy of benralizumab in EGPA, the most interesting aspect of our report is that efficacy was confirmed in a patient previously unresponsive to mepolizumab, known to be effective in EGPA.

scholarly journals A Progressive Case of Eosinophilic Myocarditis Due to Eosinophilic Granulomatosis With Polyangiitis in a Caucasian Male

2020 ◽  
Vol 8 ◽  
pp. 232470962096685
Author(s):  
Valerie F. Civelli ◽  
Vishal K. Narang ◽  
Rupam Sharma ◽  
Ritika Sharma ◽  
Jessica Kim ◽  
...  
Keyword(s):  
Allergic Rhinitis ◽  
Inflammatory Process ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Small Vessels ◽  
History Of ◽  
Eosinophilic Myocarditis ◽  
Eosinophilic Granulomatosis ◽  
Large Vessels

Vasculitis is an inflammatory process involving blood vessels of various sizes, including the small vessels in the kidneys to the large vessels, such as the aorta. This inflammatory condition is usually autoimmune in nature and is associated with involvement of many locations, such as the sinuses, lungs, kidneys, and even the heart. Specifically, eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that may initially hide as asthma, allergic rhinitis, and/or sinusitis. However, it is known to become a lethal disease once progressed to include cardiovascular manifestations. It is important to remember EGPA as a differential for any patient with a history of asthma, allergic rhinitis, and/or sinusitis who also presents with cardiovascular complaints and eosinophilia. Treatment recommendations focus on immunosuppression in such cases. In this article, we discuss the case of a 62-year-old male, with a known history of asthma, who presented to the emergency department with concern for his chest pain and right-sided weakness. He was later diagnosed with EGPA with eosinophilic myocarditis. Diagnosis and treatment are described.

scholarly journals Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

2019 ◽  
Vol 6 (6) ◽  
pp. e615 ◽  
Author(s):  
Antje Bischof ◽  
Veronika K. Jaeger ◽  
Robert D. M. Hadden ◽  
Raashid A. Luqmani ◽  
Anne-Katrin Pröbstel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vasculitic Neuropathy ◽  
Diagnostic Certainty ◽  
Primary Systemic Vasculitis ◽  
Organ Manifestations ◽  
Eosinophilic Granulomatosis

ObjectiveReported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.MethodsData of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.ResultsNine hundred fifty-five patients (mean age 57 years, range 18–91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement.ConclusionsOur study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis: Diagnostic Dilemmas

2018 ◽  
Vol 15 (1) ◽  
pp. 63-68
Author(s):  
Delia Tulbă ◽  
Răzvan Adrian Ionescu ◽  
Daniela Gologanu ◽  
Marius Ioan Balea ◽  
Simona Caraiola
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Histopathological Examination ◽  
Systemic Vasculitis ◽  
Transbronchial Biopsy ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Imaging Features ◽  
Necrotizing Vasculitis ◽  
Tissue Eosinophilia ◽  
Lung Abnormalities ◽  
Eosinophilic Granulomatosis

AbstractIntroduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a systemic vasculitis of the small vessels that often associates asthma and blood/tissue eosinophilia.Case presentation. A 58-year-old woman was admitted to our hospital for progressive exertional dyspnea. She had been diagnosed with asthma three years earlier. Recent multiple thoracic computed tomography scans displayed non-fixed interstitial lung abnormalities, whereas the infectious workup (HIV, parasites) was negative.On admission, the clinical examination noted prolonged expiratory phase. Paraclinical tests revealed biological inflammatory syndrome, eosinophilia, polyclonal hypergammaglobulinemia, elevated total IgE level, negative anti-neutrophil cytoplasmic antibodies and proteinuria of nephritic pattern.The pulmonary evaluation reconfirmed the obstructive ventilatory dysfunction and interstitial lung abnormalities - interlobular septal thickening and diffuse ground-glass opacification. The histopathological examination of a transbronchial biopsy specimen identified leukocytoclastic necrotizing vasculitis and tissue eosinophilia.Provided the clinical and paraclinical setting, specifically the asthma, blood and extravascular eosinophilia, paranasal sinus abnormalities, non-fixed pulmonary infiltrates and the histopathologically confirmed necrotizing vasculitis, the diagnosis of EGPA seemed appropriate.Conclusion. In the absence of diagnostic criteria, EGPA diagnosis is often challenging. Although certain clinical and imaging features could assist the diagnosis, biopsy remains the diagnostic gold standard.In the setting of lung involvement, open lung biopsy is usually required for EGPA histopathological proof, but few cases diagnosed by transbronchial biopsy have been reported. This method often identifies discrete, nonspecific lesions or an incomplete spectrum of pathognomonic abnormalities but has the advantage of minimal invasivity that justifies its use as an alternative diagnostic technique.

Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

2017 ◽  
Vol 16 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Vincent Cottin ◽  
Elisabeth Bel ◽  
Paolo Bottero ◽  
Klaus Dalhoff ◽  
Marc Humbert ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

scholarly journals Safety and Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Ravi Ranjan Pradhan ◽  
Gaurav Nepal ◽  
Shobha Mandal
Keyword(s):  
Side Effects ◽  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Sustained Remission ◽  
Organ Systems ◽  
Frequent Relapses ◽  
Systemic Glucocorticoids ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis disorder which involves multiple organ systems and is characterized by asthma, pulmonary infiltrates, sinusitis, neuropathy, and peripheral eosinophilia. It also has an effect on the heart, skin, kidneys, and gastrointestinal tract. Interlukin-5 (IL-5) is involved in maturation and activation of eosinophil, the production of which is increased in the EGPA. Treatments of EGPA are limited to systemic corticosteroids and immunomodulators. These drugs are associated with significant side effects. Besides this, the response of patients to these drugs may be disappointing. Frequent relapses, the need for long-term medium-to-high-dose glucocorticoid therapy, and failure to achieve remission are not uncommon findings. There is a need for noble agents that could reduce frequent relapses and the dose of systemic glucocorticoids and maintain a sustained remission without significant side effects. Mepolizumab is IL-5 antagonist and may have value in treating patients with EGPA. Therefore, we did a systematic review to evaluate the efficacy and safety of mepolizumab in patients with EGPA.

scholarly journals GRANULOMATOSE EOSINOPHILIQUE AVEC POLYANGEITE: PRESENTATIONS INHABITUELLES. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: UNUSUAL PRESENTATIONS

2021 ◽  
Vol 9 (10) ◽  
pp. 1199-1203
Author(s):  
Fadwa Mekouar ◽  
◽  
Naoual Elomri ◽  
Mohamed Jira ◽  
Naoufal Assoufi ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pulmonary Involvement ◽  
Military Hospital ◽  
Unknown Etiology ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Peripheral Eosinophilia ◽  
Medicine Department ◽  
The Third ◽  
Eosinophilic Granulomatosis

Introduction:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown etiology, affecting small and medium-sized vessels and associating asthma,pulmonary involvement, peripheral eosinophilia and extravascular eosinophilic granulomas. We report three GEPA sightings of unusual revelations. Methods:These are three cases of GEPA from the internal medicine department B, Mohammed V military hospital in Rabat. Results:Three patients aged between 27 and 70 years. The most frequently found manifestations were pulmonary, cutaneous, neurological and digestive. All patients presented with peripheral eosinophilia. The ANCA were negative. All patients were treated with corticosteroids in one case, immunosuppressive therapy were used. Conclusion:In this article, GEPA was revealed by an acute surgical abdomen (appendicitis) in one case, a febrile rash in the second and liver damage in the third patient. Our patients responded well to corticosteroid therapy but the treatment must be adapted depending on the case.

The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study

2016 ◽  
Vol 43 (6) ◽  
pp. 1072-1077 ◽  
Author(s):  
Gideon Nesher ◽  
Eli Ben-Chetrit ◽  
Bracha Mazal ◽  
Gabriel S. Breuer
Keyword(s):  
Jewish Population ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Pearson Correlation ◽  
Incidence Rates ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Medical Centers ◽  
Clinical Databases ◽  
Primary Systemic Vasculitis ◽  
Eosinophilic Granulomatosis

Objective.The incidence of primary systemic vasculitides varies among different geographic regions and ethnic origins. The aim of this study was to examine the incidence rates of vasculitides in the Jerusalem Jewish population, and to examine possible trends in incidence rates over a 20-year period.Methods.The clinical databases of inpatients at the 2 medical centers in Jerusalem were searched for patients with vasculitis diagnosed between 1990–2009. Individual records were then reviewed by one of the authors. The significance of trends in incidence rates throughout the study period was evaluated by Pearson correlation coefficient.Results.The average annual incidence rate of polyarteritis nodosa was 3.6/million adults (95% CI 1.6–4.7). Incidence rates did not change significantly during this period (r = 0.39, p = 0.088). The incidence of granulomatosis with polyangiitis (GPA) was 4.1 (2.2–5.9) for the whole period, during which it increased significantly (r = 0.53, p < 0.05). The incidence of microscopic polyangiitis (MPA) was lower: 2.3 (1.2–3.5)/million. It also increased significantly (r = 0.55, p < 0.05). The incidence of eosinophilic granulomatosis with polyangiitis was 1.2 (0.4–1.9), which remained stable throughout the study period. The incidence of Takayasu arteritis was 2.1/million (95% CI 1.2–2.9), and it also remained stable. Giant cell arteritis (GCA) incidence was 8.1 (5.7–10.6)/100,000 population aged 50 years or older. In sharp contrast with other vasculitides, its incidence decreased significantly throughout the study period (r = −0.61, p < 0.01).Conclusion.The incidence rates of vasculitides in the Jewish population of Jerusalem are in the lower range of global incidence rates. While GPA and MPA incidence are increasing, GCA incidence is decreasing.

Rheumatology

2016 ◽  
Keyword(s):  
Lupus Erythematosus ◽  
Basic Science ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Relapsing Polychondritis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Basic Training ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Chapter 19 covers the basic science and clinical topics relating to rheumatology which trainees are required to learn as part of their basic training and demonstrate in the MRCP. It covers basic science, the synovium, autoantibodies, osteoarthritis, rheumatoid arthritis, septic arthritis, crystal arthropathies, spondyloarthritides, psoriatic arthritis, low back pain, systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, Sjögren syndrome, giant cell arteritis/polymyalgia rheumatic, polyarteritis nodosa, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), granulomatosis with polyangiitis (Wegener), treating systemic vasculitis, relapsing polychondritis, and Behҫet disease.

scholarly journals Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Kenichiro Hira ◽  
Hideki Shimura ◽  
Riyu Kamata ◽  
Masashi Takanashi ◽  
Akane Hashizume ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Brain Infarction ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Consciousness Disturbance ◽  
The Right ◽  
The Brain ◽  
Eosinophilic Granulomatosis

Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. Case presentation A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma. Conclusions In this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.

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