Eosinophilic Granulomatous Polyangiitis Research Articles

Effectiveness of mepolizumab in patients with severe eosinophilic asthma: In a real life study

We analyzed the effects of mepolizumab treatment on symptoms, asthma attacks, pulmonary function test parameters peripheral blood eosinophil level, and percentage in patients with severe eosinophilic asthma receiving mepolizumab treatment as the baseline, sixth and twelfthmonth data. The medical records of patients diagnosed with severe eosinophilic asthma and treated with mepolizumab at our clinic were retrospectively reviewed for the period between January 2018 and December 2021. Demographic data of the patients, duration of asthma disease, comorbidities such as a nasal polyp, eosinophilic granulomatous polyangiitis, and nonsteroidal anti-inflammatory drug exacerbated respiratory disease were investigated. A comparison was made of various factors before initiating mepolizumab treatment, as well as at the sixth and twelfth month after treatment initiation. These factors include asthma control test scores, frequency of asthma attacks (including emergency admissions, hospitalizations, and intensive care admissions), peripheral blood eosinophil levels and percentages, and pulmonary function test parameters. Clinic and laboratory parameters that provide a prediction of being a responder and super responder were evaluated. A total of 21 patients were included in the study. Their mean age was 50.7 ± 11.9 years, and four (19%) were males. The mean duration of asthma diagnosis was 17.5 ±13.7 years. 14 patients (66.7%) were atopic. 4 patients (19%) had nasal polyps and four patients (19%) had NERD. Before mepolizumab, 13 (61.9%) patients had received omalizumab. The duration of receiving mepolizumab treatment was 29.2 ± 9.9 months. A statistically significant decrease was observed in both the number and percentage of eosinophils at months six and 12 (p<0.01). There was a statistically significant increase in FEV1 values both as a percentage and in milliliters at month 12. There was an increase in both percentage and milliliters in FEF25-75 values, but this increase did not reach statistical significance. There was a decrease in service admissions, intensive care admissions, and emergency admissions due to asthma exacerbations. Out of 21 patients, 11 (52.4%) were classified as responders, while 10 (47.6%) were classified as super responders. Although the number of patients in our study was limited, mepolizumab improved symptom scores in severe eosinophilic asthma, reduced the number of attacks, and improved pulmonary function test values.

Experience with the use of immunobiological drugs in the treatment of patients with severe bronchial asthma and comorbidities

Introduction. The accumulation of clinical experience in the use of biological therapy in patients with severe bronchial asthma (SBA) in real-world clinical practice with the possible identification of additional, previously undescribed clinical results is of practical interest.Aim. The goal of the study is to present results of observational study that reflects experience of using benralizumab in SBA in real-world clinical practice in city of Saint Petersburg.Materials and methods. We presents results of more than 1 year observation of 18 patients with eosinophilic SBA (12 females, 6 males, age from 28 to 74, average age 55 ± 11.9 years). Patients received benralizumab in addition to standard medical management. Conducted clinical, laboratory, functional examination of patients, assessment of the frequency of exacerbations and additional clinical effects.Results. It was shown that the inclusion of benralizumab in complex therapy led to a significant improvement in disease control, improved bronchial conduction, a significant decrease in blood eosinophilia and a reduction in the number of exacerbations. Clinical examples are given that evidence to the stabilizing effect of this therapy on the course of recurrent nasal polyposis and the positive effects on the course of eosinophilic granulomatous polyangiitis, manifested by SBA.Conclusions. In real clinical practice, the possibility of a significant improvement in control in patients with SBA with the use of biological therapy has been confirmed. At the same time, data have been obtained that will allow expanding the indications for the use of benralizumab in the future.

Efficacy of omalizumab in the treatment of eosinophilic granulomatous polyangiitis with asthma as the first symptom

Objective: To investigate the efficacy, and safety of omalizumab in the treatment of eosinophilic granulomatous with polyangiitis (EGPA) with asthma as the first symptom. Method: The clinical characteristics of 22 EGPA patients with asthma as the first symptom treated with omalizumab in the First Affiliated Hospital of Guangzhou Medical University from March 2018 to December 2020 were retrospectively analyzed. The asthma control test (ACT) score, the frequency of asthma exacerbation (AE), the Birmingham Vasculitis Activity Score (BVAS), the variation rate of peak expiratory flow (PEF), the percentage of PEF to predicted value of PEF (PEFpred%), the percentage of forced expiratory volume in first second (FEV1) to predicted value of FEV1 (FEV1pred%), the dosage of oral corticosteroid (OCS) and other clinical data [M(Q1, Q3)] were collected before and after treatment, to observe the efficacy and adverse reactions of omalizumab. Results: There were 22 subjects recruited in this study. The median age was 42 (22-70) years. Eleven of the patients were males. After treated with omalizumab for 4 months, there were 68.2%(15/21) of patients who responded to the treatment. In the response group (n=15), the patients' ACT score increased from 19.0 (16.5, 21.0) to 23.0 (21.5, 24.0) (P=0.001). The frequency of AE decreased from 0.7 (0.3, 1.0) to 0 (0, 0.7) per four mouths (P<0.001). The BVAS decreased from 4.0 (2.0, 6.0) to 2.0 (2.0, 4.0) (P=0.007). The variation rate of PEF decreased from 18.8% (14.0%, 27.7%) to 9.2% (6.8%, 11.9%) (P=0.007). The PEFpred% increased from 80.8% (73.5%, 90.7%) to 100.5% (79.4%, 114.0%) (P=0.005). The maintenance dosage of OCS reduced from 15.0 (10.0, 20.0) mg/d to 8.8 (5.0, 10.0) mg/d (P=0.005). The level of baseline eosinophil in peripheral blood of patients in non-response group was higher than that in response group [11.4% (9.2%, 22.6%) vs 3.4% (1.1%, 6.5%), P<0.05]. A total of 190 injections were performed in 22 patients, and only 4 patients (2.1%) had adverse reactions after a single injection of omalizumab, such as dizziness, swelling of injection site and pruritus. The adverse reactions were tolerable. Conclusions: Omalizumab has certain curative effect on EGPA, can reduce asthmatic symptoms and OCS maintenance dosage, and has a good safety profile. The rate of response to the treatment is higher in patients with mild eosinophilic inflammation.

Relationship Between Familial Mediterranean Fever and Other Rheumatic Diseases: Coincidence or Coexistence?

Background: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease affecting mainly the ethnic groups of the Mediterranean basin. It has been reported that it can coexist with various systemic inflammatory diseases. This study aimed to obtain information on rheumatic diseases that accompany FMF and evaluate the relation between FMF and such diseases.&#x0D; Methods: Eighty-four patients diagnosed with FMF and have rheumatic disease comorbidity in the rheumatology clinic between January 2018 - March 2020 were included in this study. &#x0D; Results: The most common accompanying rheumatic disease was spondyloarthritis with 36 patients. Vasculitides was the second common disease accompanying FMF with 22, followed by connective tissue disease in 13, rheumatoid arthritis in 5, juvenile idiopathic arthritis in 4, gout in 3, and hidradenitis suppurativa in 1 patients. The most common MEFV mutation observed was M694V. There was no significant difference in demographic characteristics and clinical manifestations between M694V mutation types. &#x0D; Conclusions: In this study, the relation between FMF and various rheumatic diseases was determined. Two new conditions, eosinophilic granulomatous polyangiitis, and scleroderma were detected. The associations may be just coincidental or an extension of the common underlying pathology. To be aware of this association is important to early diagnosis and appropriate treatment.

Clinical case of eosinophilic granulomatous polyangiitis debuted by myocardial infarction

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).

Marked neurological and immunological improvement in refractory eosinophilic granulomatous polyangiitis after treatment with mepolizumab, an anti‐interleukin‐5 antibody: A case report

AbstractBackgroundMepolizumab is an option for add‐on therapy in cases of refractory or relapsing eosinophilic granulomatous polyangiitis (EGPA). However, it has not been fully investigated if add‐on mepolizumab is effective for active refractory or active relapsing EGPA patients with life‐ and/or organ‐threatening manifestations, especially with severe neuropathy.Case presentationWe herein report on a 63‐year‐old man with severe progressive neuropathy secondary to active relapsing EGPA. In this case, the optimal dose of glucocorticoids and cyclophosphamide could not be used because of glaucoma and signet ring cell carcinoma. Add‐on therapy of mepolizumab showed prompt immunological remission and marked improvement in neurological manifestations.ConclusionsMepolizumab can be considered as an important induction therapy option for active relapsing or refractory EGPA. It has the potential to effectively improve neurological disturbances associated with severe neuropathy in EGPA.

Eosinophilic Myo-Pericarditis Presenting as Cardiac Tamponade

Eosinophilic myocarditis is a rare and acute life threatening condition often associated with drug reactions, eosinophilic granulomatous polyangiitis (formerly Churg-Strauss syndrome), Loeffler endomyocardial disease, parasite infections, and idiopathic hypereosinophilic syndrome. We describe a case of eosinophilic granulomatous polyangiitis presenting as progressive pericardial effusion and cardiac tamponade.

Relationship Between Immunosuppressive Therapy and the Development of Infectious Complications Among Patients with Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: A Single-center, Retrospective Observational Study.

IntroductionInfectious complications are the leading cause of death in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). However, the relationship between initial immunosuppressive therapy and the development of infectious complications and the details of infectious complications among patients with AAV are uncertain. We thus aimed to determine the association between initial immunosuppressive therapy and infectious complications.Material and methodsForty-seven patients with newly diagnosed AAV were enrolled in this retrospective observational study (patients with eosinophilic granulomatous polyangiitis were excluded). We statistically determined the association between types of initial immunosuppressive therapy (methylprednisolone pulse and/or cyclophosphamide therapy) and the development of infectious complications. In addition, we investigated the causes and timing of the onset of infectious complications.ResultsTwenty-one (21; 44.7%) patients required antibiotic, antimycotic, or antiviral therapy because of the development of infectious complications. Multiple logistic regression analyses adjusted for age and sex revealed that methylprednisolone pulse and cyclophosphamide therapy were significantly associated with the development of infectious complications (odds ratio (OR) 4.85, 95% confidence interval (CI) 1.09-21.5, p = 0.038; OR 5.32, 95% CI 1.28-22.2, p = 0.022, respectively). Bacterial pneumonia and sepsis occurred in 10 (47.6%) and 6 (28.6%) patients, respectively. Almost half of these infectious complications, including fungal infection, developed within six months from the start of initial treatment.ConclusionAmong patients with AAV, methylprednisolone pulse and cyclophosphamide therapy may increase the risk of developing infectious complications, such as pneumonia and sepsis, including fungal infection, particularly within six months from the initiation of treatment.

EOSINOPHILIC MYO-PERICARDITIS PRESENTING AS CARDIAC TEMPONADE

Eosinophilic myocarditis is a rare and acute life threatening condition often associated with drug reactions, eosinophilic granulomatous polyangiitis (formerly Churg-Strauss syndrome), Loeffler endomyocardial disease, parasite infections, and idiopathic hypereosinophilic syndrome. We describe a case

Last station in the eosinophilic asthma with chronic rhinosinusitis and/or nasal polyposis march: Eosinophilic asthma with radiological findings associated with blood eosinophilia

ABSTRACTObjective: Eosinophilic asthma with chronic rhinosinusitis and/or nasal polyposis (EA-CRS/NP) is a subphenotype of adult-onset eosinophilic asthma. Blood eosinophil levels are shown to be highly elevated in patients with EA-CRS/NP and have potential for tissue infiltration. We aimed to demonstrate the clinical features of the patients who have a blood eosinophil level above 10% and have thorax computed tomography findings due to blood eosinophilia. Methods: Patients who were followed up in our clinic between 2012 and 2017 were retrospectively evaluated. Inclusion criteria were as follows: 1) Eosinophilic severe asthma, 2) eosinophilia >10%, 3) chronic sinusitis and/or nasal polyps, 4) patients with pathologic findings on thorax computed tomography, 5) regular follow-up for at least 1 year. Results: We identified 36 patients who met the above criteria. We defined this group as “Eosinophilic Asthma with chronic Rhinosinusitis and/or nasal polyposis with Radiological findings related to blood eosinophilia” (EARR). The mean age was 44.9 ± 11 years and 64% were females. Nasal polyps, aspirin exacerbated respiratory disease, and atopy, were present in 81%, 47%, and 25% of the patients, respectively. The mean blood eosinophil count was 1828.6 cells/mm3 (19%). The majority of EARR patients had upper lobe dominant ground-glass opacities. The mean follow-up period was 3.2 ± 2.5 years. EARR patients did not evolve into eosinophilic granulomatous polyangiitis in the follow-up. Conclusions: This phenotype is the first eosinophilic asthma sub-phenotype reported in the literature. EARR is the final stage of the allergic march of EA-CRS/NP.

Eosinophilic granulomatous polyangiitis with lung involvement: clinical analysis of 8 cases

Clinical data, imaging finding, laboratory and pathology results of 8 patients with eosinophilic granulomatous polyangiitis (EGPA) admitted in our hospital from April 2014 to September 2016 were retrospectively analyzed. There were 7 cases with sinus involvement; 8 cases with increased eosinophil counts in peripheral blood, bronchoalveolar lavage fluid (BALF) and induced sputum, and with negative antineutrophil cytoplasmic antibodies (ANCA). Lung function tests showed mild obstructive ventilation dysfunction in 7 cases, positive relaxation test or excitation test in 4 cases, and mild dispersion function decline in 4 cases. Chest CT revealed multiple ground glass shadows and peripheral pulmonary nodules in both lungs in 4 cases, and bronchial wall thickening in 1 case. Transbrochial lung biopsy (TBLB) indicated extravascular infiltration of acidophilic granulocytes. Four cases were treated by glucocorticoid monotherapy, the other 4 cases were treated by glucocorticoids combined with cyclophosphamide. All patients achieved clinical relief after treatment, but 3 cases recurred. For suspicious EGPA patients in addition to paranasal sinus CT, ANCA vasculitis index, urine routine and renal function tests, it is necessary to perform nasal mucosa, bronchial mucosa, transbrochial lung biopsy and BLAF, induced sputum cytology to obtain timely diagnosis and treatment. Key words: Churg-Strauss syndrome; Bronchial alveolar lavage fluid

Anti-neutrophil cytoplasmic antibodies negative eosinophilic granulomatous polyangiitis glomerulonephritis in children - A diagnostic dilemma

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) refers to small-to-medium vessel vasculitis with multisystemic involvement and is characterized by the presence of ANCA to specifically either proteinase-3 (PR3) or myeloperoxidase (MPO). Eosinophilic granulomatous polyangiitis earlier called as Churg-Strauss vasculitis is a clinicopathological variant of AAV or AAV with granulomatous necrotizing small vessel vasculitis primarily affecting individuals with severe asthma or allergies and rarely reported in the pediatric population. We report a 9-year-old child with pauci-immune crescentic eosinophilic granulomatousglomerulonephritis with heavy eosinophilic infiltration of skin and subcutaneous tissue and negative ANCA. Although he had a recurrent history of cough, there was no history of use of oral or inhalational bronchodilator therapy indicating asthma and no history suggestive history of allergy. He showed significant and rapid clinical as well as biochemical improvement on aggressive immunosuppressive therapy along with plasma exchanges and is under regular follow-up. Diagnosing Eosinophilic granulomatous Polyangiitis glomerulonephritis in children is difficult due to a varied clinical presentation at onset which may evolve over a period of time and due to lack of specific diagnostic tests. High index of suspicion is the key to early diagnosis and successful management.

P167 17 year-old asthmatic male with eosinophilic granulomatous polyangiitis and allergic fungal rhinosinusitis treated with omalizumab

P167 17 year-old asthmatic male with eosinophilic granulomatous polyangiitis and allergic fungal rhinosinusitis treated with omalizumab

Antineutrophilic cytoplasmic antibody-associated vasculitis and malignant hemopathies, a retrospective study of 16 cases

ObjectivesANCA-associated vasculitis are severe autoimmune pathologies that are characterized by inflammation and necrosis of the small vessels. The physiopathological mechanisms are complex and have yet to be totally elucidated. Several environmental factors have been described as being associated: medications, infectious agents… and rarely, neoplasms. MethodsWe performed a retrospective multicenter study over a period of 12 years with a view to describing the association of ANCA-associated vasculitis and malignant hemopathies, excluding hemopathies secondary to vasculitis treatment. ResultsSixteen patients with ANCA-associated vasculitis with an hemopathy were identified. The gender ratio was 7 and the mean age was 65 years. The frequency of this association is estimated at 1%. The ANCA-associated vasculitis were micropolyangiitis (n=7), followed by granulomatous polyangiitis (n=4), vasculitis limited to the kidney (n=3), and eosinophilic granulomatous polyangiitis (n=2). The associated malignant hemopathies were mainly non-Hodgkin's lymphoma in seven cases and myelodysplasia in five cases. The other hemopathies were: Hodgkin's disease, hypereosinophilic syndrome, and Waldenström's macroglobulinemia. Hemopathy treatment was associated with vasculitis treatment in seven cases. ConclusionThe association of ANCA-associated vasculitis and malignant hemopathy is rare but must nevertheless be recognized because: (i) the clinical signs of both pathologies are not specific, (ii) the survival scores that are used for ANCA-associated vasculitis do not appear to be applicable, (iii) both pathologies must be taken into account in order to implement an effective therapeutic strategy that limits the inherent risks.

Clinical Outcomes of Patients with Dual Positivity for Proteinase 3 and Myeloperoxidase Specific Antineutrophil Cytoplasmic Antibodies

Objective: Granulomatous polyangiitis patients generally have ANCA targeting Proteinase 3 (PR3), while microscopic polyangiitis and eosinophilic granulomatous polyangiitis patients generally have ANCA targeting myeloperoxidase (MPO). Patients positive for both PR3-ANCA and MPO-ANCA are uncommon and any diagnostic value of this situation has not been well described. The aim of this study was to determine whether there were any patterns of clinical presentations or outcomes of patients who tested positive for both PR3-ANCA and MPO-ANCA. Methods: A retrospective clinical audit was carried out. A list of patients who had tested positive for both PR3- ANCA and MPO-ANCA was obtained from ACT Pathology from 2003-2013. Medical records were then used to determine their clinical outcomes. Results: 3 of the 15 study patients were found to have ANCA-associated vasculitis (AAV), two with MPA and the other EGPA along with a non-AAV autoimmune disease. 1 patient had drug-induced vasculitis and 4 had a non-AAV autoimmune disease. An additional three patients had a non-AVV autoimmune disease, but one concurrently had a malignancy while the other two had recurrent infections. Two patients had a malignancy, another one only had recurrent infections, and the remaining patient was lost to follow up with an undiagnosed inflammatory condition Conclusion: Pathology results showing dual positivity for PR3-ANCA and MPO-ANCA in itself does not appear to indicate any particular pattern of clinical outcome and so cannot be used to draw any clinical conclusions at the time of presentation. The 3 cases of AAV demonstrated MPO-predominance which fitted with their final diagnosis.

The Role of Environmental Factors in the Pathogenesis of Anti-Neutrophil Antibody Associated Vasculitis

This review seeks to critically examine the environmental factors reported to be associated with anti-neutrophil antibody associated systemic vasculitis. The published literature was searched in a systematic fashion. From this emerges strong evidence for a latitude gradient in the case of granulomatous polyangiitis and eosinophilic granulomatous polyangiitis; both appear more common at high latitudes. Microscopic polyangiitis does not demonstrate this. Latitude affects ultraviolet radiation exposure and the relationship of increased incidence of granulomatous polyangiitis and eosinophilic polyangiitis with decreased ultraviolet radiation exposure is much stronger. The most plausible explanation is vitamin D levels, a hormone known to be vital for normal immune function and largely synthesised in humans secondary to ultraviolet radiation exposure. Case control studies demonstrate a consistent relationship to silica exposure a substance well known to have profound immunological effects. The genetic risk factors for these diseases will be considered and a model of how genes and environment may interact to produce these diseases discussed. Low vitamin D levels, silica exposure, other environmental triggers may interact with genes that influence the immune and inflammatory response to initiate and sustain these diseases. As such, the broad concepts developed about the etiopathogenesis of the anti-neutrophil antibody associated vasculitides shows many parallels to other autoimmune diseases. Much more information is required about both genes and the environment, but the model helps define the questions that need to be answered