5,912 results on '"Churg-Strauss Syndrome"'
Search Results
2. Study of Mepolizumab-based Regimen Compared to Conventional Therapeutic Strategy in Patients With Eosinophilic Granulomatosis With Polyangiitis (E-merge) (E-merge)
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French Vasculitis Study Group and URC-CIC Paris Descartes Necker Cochin
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- 2024
3. Longitudinal Study for Eosinophilic Granulomatosis With Polyangiitis
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GlaxoSmithKline, AstraZeneca, and Peter Merkel, Professor
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- 2024
4. Efficacy and Safety of Benralizumab in EGPA Compared to Mepolizumab. (MANDARA)
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- 2024
5. Trial of Efficacy and Safety of NS-229 Versus Placebo in Patients With Eosinophilic Granulomatosis With Polyangiitis
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Nippon Shinyaku Co., Ltd.
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- 2024
6. Explore the Efficacy and Mechanism of Action of Tezepelumab in Eosinophilic Granulomatosis With Polyangiitis (RACEMATE)
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AstraZeneca
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- 2024
7. Long-term Access Program (LAP) of Mepolizumab for Subjects Who Participated in Study MEA115921
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- 2024
8. eGPA and Local Inflammation Within the Ear, Nose and Throat Area
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GlaxoSmithKline
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- 2024
9. The Epigenomic Signature of Eosinophilic Granulomatosis With Polyangiitis (EPI-EGPA)
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Careggi Hospital, University of Florence, and Augusto Vaglio, Professor
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- 2024
10. Clinical Transcriptomics in Systemic Vasculitis (CUTIS) (CUTIS)
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National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Center for Advancing Translational Sciences (NCATS), Office of Rare Diseases Research (ORDR), and Peter Merkel, Chief, Division of Rheumatology Professor of Medicine and Epidemiology
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- 2024
11. VCRC Tissue Repository
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National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Center for Advancing Translational Sciences (NCATS), Office of Rare Diseases (ORD), and Peter Merkel, Chief, Division of Rheumatology Professor of Medicine and Epidemiology
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- 2024
12. Mesenchymal stem cell therapy in eosinophilic granulomatosis with polyangiitis-related lower limb gangrene: a case report.
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Wang, Hui, Zhang, Qian, Wu, Sensen, Pan, Dikang, Ning, Yachan, Wang, Cong, Guo, Jianming, and Gu, Yongquan
- Abstract
Background: Eosinophilic granulomatosis with polyangiitis (EGPA), a rare but life-threatening systemic vasculitis, is distinguished by marked eosinophilia and presents with diverse symptoms, including asthma, cutaneous purpura, ecchymosis, skin necrosis, cardiac lesions, peripheral neuropathy, and necrotizing vasculitis. The etiology of EGPA involves a complex interaction among humoral, adaptive, innate, and allergic immune responses. Standard treatment employs prolonged high-dose glucocorticoid therapy, which is critical for survival; however, some patients' symptoms cannot be relieved. Case report: This case report details the medical management of an 11-year-old patient with EGPA, who was at risk of bilateral lower limb amputation due to differential arterial occlusion and severe, necrotizing vasculitis-induced gangrene in both feet. Treatment modalities administered included systemic infusion of Umbilical Cord Mesenchymal Stem Cells (UC-MSCs), targeted gastrocnemius muscle injections, and application of a Placenta-Derived Mesenchymal Stem Cells (PD-MSCs) hydrogel. Results: After receiving a four-month regimen of allogeneic mesenchymal stem cell therapy via intravenous and local administration, the patient showed normalized eosinophil counts, reestablished blood flow in the dorsal arteries, and marked improvement in foot ulcerations. Conclusion: Mesenchymal stem cell therapy is a promising option for severe EGPA cases refractory to glucocorticoids. [ABSTRACT FROM AUTHOR]
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- 2024
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13. Trends in prevalence, treatment use, and disease burden in patients with eosinophilic granulomatosis with polyangiitis in Japan: Real-world database analysis.
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Sada, Ken-ei, Suzuki, Takeo, Joksaite, Sandra, Ju, Shinyoung, Logie, John, Mu, George, Hwee, Jeremiah, Kunishige, Hideaki, Ishii, Takeo, Adlak, Amit, Vadlamudi, Harini, and Alfonso-Cristancho, Rafael
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CHURG-Strauss syndrome , *DISEASE relapse , *DATABASES , *CORPORATION reports , *CONFIDENCE intervals - Abstract
Objectives: We report the prevalence of eosinophilic granulomatosis with polyangiitis (EGPA) and describe oral corticosteroid (OCS) use and disease burden before and after the mepolizumab approval in 2018 for EGPA in Japan. Methods: Two retrospective studies (GSK IDs: 218083 and 218084) used two databases: (1) the JMDC insurer database (Japanese health insurer claims) was used to report annual EGPA prevalence and OCS use in mepolizumab-treated patients and (2) Medical Data Vision database was used to report annual treatment use, OCS dose, relapses, and healthcare resource utilization in patients with EGPA. Results: EGPA prevalence (95% confidence interval) increased from 4.2 (0.1, 23.4) in 2005 to 58.6 (53.2, 64.5) per 1,000,000 in 2020. Median OCS dose (mg/day) decreased from a range of 4.8–7.7 during 2010–2017 to 4.5–4.8 during 2018–2020 (lowest dose in 2020). The proportion of patients with prednisolone-equivalent daily OCS dose >10 mg decreased from 2017 (11.9%) to 2020 (10.3%), while the median dose halved. The proportion of patients with EGPA relapses (64.3% to 41.6%) and hospitalization (27.8% to 23.6%) decreased from 2010 to 2020. Conclusions: EGPA prevalence increased between 2005 and 2020. With the introduction of mepolizumab for EGPA in 2018, real-world OCS use, relapses, and healthcare resource utilization decreased. [ABSTRACT FROM AUTHOR]
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- 2024
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14. Real-world safety and effectiveness of mepolizumab for patients with eosinophilic granulomatosis with polyangiitis in Japan: A 48-week interim analysis of the MARS study.
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Ishii, Tomonori, Kunishige, Hideaki, Kobayashi, Tamami, Hayashi, Etsuko, Komatsubara, Masaki, Ishii, Takeo, Alfonso-Cristancho, Rafael, Tamaoki, Jun, and Howarth, Peter
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CHURG-Strauss syndrome , *PATIENTS' attitudes , *PREVENTIVE medicine , *INTERLEUKIN-5 , *HOSPITAL emergency services - Abstract
Objectives: The objective of the study is to assess real-world, long-term safety/effectiveness of mepolizumab for eosinophilic granulomatosis with polyangiitis (EGPA) in Japan. Methods: The Mepolizumab long-term study to Assess Real-world Safety and effectiveness of EGPA in Japan (MARS) (GSK ID: 213684/NCT04551989) is an ongoing 96-week study of patients with EGPA who received four-weekly mepolizumab 300 mg subcutaneously for ≥96 weeks before study entry (baseline) and continued treatment. This interim analysis included safety from baseline to Week 48 (observation period) and clinical outcomes before mepolizumab and during the observation period. Results: Of 118 patients enrolled, 29% (34/118) experienced adverse events (AEs), of which 13% (15/118) experienced serious AEs; none were considered mepolizumab related. The median oral corticosteroid (OCS) dose decreased from 6.9 (pre-mepolizumab) to 3.0 (baseline) and 2.0 mg/day (Weeks 45–48); the proportion of patients receiving no OCS increased from 8% to 32% and 38%, respectively. Patients experiencing clinical symptoms decreased from 94% (pre-mepolizumab) to 73% (baseline) and 67% (Week 48). During the observation period, 5% of patients experienced EGPA relapse; the rates of EGPA-related hospitalisations, EGPA-related emergency room/unscheduled visits, and asthma exacerbations were 0.05, 0.09, and 0.08 event/person-year, respectively. Conclusions: The results of mepolizumab treatment for ≥144 weeks (before baseline plus observation) were consistent with the known safety profile and allowed OCS dose reduction while improving disease control versus pre-treatment among patients with EGPA. [ABSTRACT FROM AUTHOR]
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- 2024
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15. Ocular manifestations in ANCA-associated vasculitis: a comprehensive analysis from Chinese medical centers.
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Liu, Shulin, Xu, Mei, Zhao, Xinyu, Yang, Jingyuan, Zhang, Wenfei, and Chen, Youxin
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MACHINE learning , *CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *LEUCOCYTES , *OCULAR manifestations of general diseases - Abstract
Introduction: This study aimed to explore ocular manifestations in ANCA-associated vasculitis (AAV), focusing on granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) and to examine the associations with laboratory parameters and other systemic manifestations. Methods: This retrospective study reviewed data from 533 AAV patients across two major Chinese medical centers from January 2016 to November 2023. Data including diagnosis, cranial manifestations of disease, ocular complications, and laboratory parameters were analyzed. Univariate and multivariable logistic regression analyses assessed associations across disease manifestations. Machine learning models were also utilized to predict the risk of retinal/eye involvement in AAV patients. Results: Among 533 patients (210 GPA, 217 MPA, 99 EGPA, and 7 unclassified AAV), ocular complications were observed in 20.64% of them, with a distribution of 36.67% in GPA, 7.37% in MPA, and 18.18% in EGPA. The most common ocular manifestations included scleritis and retro-orbital mass/dacryocystitis, which were notably prevalent in GPA patients. Retinal involvement was observed in 9.09% of EGPA cases. The machine learning models yielded that eosinophil percentage (EOS%), high-sensitivity C-reactive protein (hsCRP), and CD4 + T cell/CD8 + T cell ratio (T4/T8) can predict retinal involvement. Furthermore, the white blood cell, EOS%, APTT, IgA, hsCRP, PR3-ANCA, and T4/T8 can predict eye involvement. Conclusion: Ocular manifestations are a prevalent complication across all forms of AAV. Predictive models developed through machine learning offer promising tools for early intervention and tailored patient care. This necessitates a multidisciplinary approach, integrating rheumatology and ophthalmology expertise for optimal patient outcomes. Key Points • This study provides new insights into the ocular manifestations of ANCA-associated vasculitis across different subtypes, revealing a notable prevalence of ocular complications. • A machine learning model is built to predict eye/retinal involvement, enhancing early diagnostic capabilities. • The findings could guide more systematic ocular screenings in ANCA-associated vasculitis patients and influence treatment protocols. [ABSTRACT FROM AUTHOR]
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- 2024
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16. Successful combination therapy of mepolizumab and dupilumab in a patient with EGPA: a future therapeutic option?
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Martins-Martinho, Joana, Costa, Roberto Pereira da, Abreu, Tiago, and Ponte, Cristina
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NASAL polyps ,HYPEREOSINOPHILIC syndrome ,DUPILUMAB ,PULMONARY aspergillosis ,CHURG-Strauss syndrome ,ASTHMATICS ,FORCED expiratory volume - Published
- 2024
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17. Atypical clinical presentation of eosinophilic angiocentric fibrosis with cutaneous and upper respiratory tract involvement.
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Liñán‐Barroso, Juan Manuel, Valérdiz‐Menéndez, Nicolás, García‐Morillo, José Salvador, and Bernabeu‐Wittel, José
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SYMPTOMS , *CHURG-Strauss syndrome , *RESPIRATORY infections , *GRANULOMATOSIS with polyangiitis , *SKIN diseases , *NASAL mucosa , *EOSINOPHILIA , *EOSINOPHILIC granuloma - Abstract
This article discusses a case of eosinophilic angiocentric fibrosis (EAF) with atypical clinical presentation involving the skin and upper respiratory tract. EAF is a rare fibroinflammatory condition that primarily affects the orbit and upper respiratory tract. The case report describes a 17-year-old female who initially presented with a firm plaque on her thigh, which later became inflamed and coincided with upper respiratory tract infections. The patient was eventually diagnosed with EAF based on skin and nasal biopsies, and she responded well to treatment with methotrexate and prednisone. The article emphasizes the importance of recognizing both cutaneous and upper respiratory involvement in EAF for accurate diagnosis and management. [Extracted from the article]
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- 2024
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18. Aural Manifestations of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis—Diagnosis, Symptoms, Treatment.
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Kaczmarczyk, Michał S., Jurkiewicz, Dariusz, Niemczyk, Stanisław, and Rymarz, Aleksandra
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CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *ANTINEUTROPHIL cytoplasmic antibodies , *FACIAL paralysis , *OTITIS media - Abstract
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of vasculitis sharing a common pathophysiology, which affects small and medium blood vessels. There are three categories of AAV: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). As a systemic disease, AAV can affect basically every organ. The goal of this publication is to sum up and underline the problem of the aural manifestation of AAV; it details the definition of Otitis Media with Antineutrophil Cytoplasmic Antibody Associated Vasculitis (OMAAV) and allows for a better understanding of the specific tasks of medical professionals taking part in the diagnostic and therapeutic process. Among others, this publication is directed to otolaryngologists who may encounter patients with AAV and often are the first specialists who see patients with early symptoms of AAV. This publication presents brief characteristics of AAV, descriptions of aural manifestations and symptoms, differential diagnosis, and both pharmacological and surgical treatment options, based on current recommendations and information found in the literature and clinical databases. [ABSTRACT FROM AUTHOR]
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- 2024
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19. ST-Elevation Myocardial Infarction Due to Coronary Vasospasm Associated with Eosinophilic Granulomatosis with Polyangiitis: A Case Report.
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Allen, Christopher, Poyorena, Christopher, and Querin, Lauren B.
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CHURG-Strauss syndrome ,ST elevation myocardial infarction ,CHEST pain ,CORONARY vasospasm ,CORONARY artery disease ,IMMUNOSUPPRESSIVE agents ,DISEASE complications - Abstract
Introduction: ST-elevation myocardial infarction (STEMI) can be caused by underlying coronary artery vasospasm (CAV) with or without associated atherosclerotic disease. Coronary artery vasospasm is a rare but potentially devastating manifestation of eosinophilic granulomatosis with polyangiitis (EGPA). Case Report: We describe a 54-year-old male with a known history of EGPA and coronary artery disease presenting to the emergency department with chest pain and an inferior STEMI on electrocardiogram. He was ultimately taken for coronary angiography and found to have a discrete vasospastic lesion in the right coronary artery that was treated with intra-coronary nitroglycerin and calcium channel blockers. He was continued on immunosuppressant agents (prednisone and mepolizumab) for management of EGPA and followed up with outpatient cardiology and rheumatology for vasospastic angina. Conclusion: This case highlights a rare cause of STEMI, discusses the nuances in treatment of STEMI due to CAV, and provides background on pathophysiology and treatment of EGPA. [ABSTRACT FROM AUTHOR]
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- 2024
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20. Biologic therapy in rare eosinophil-associated disorders: remaining questions and translational research opportunities.
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Khoury, Paneez, Roufosse, Florence, Kuang, Fei Li, Ackerman, Steven J, Akuthota, Praveen, Bochner, Bruce S, Johansson, Mats W, Mathur, Sameer K, Ogbogu, Princess U, Spencer, Lisa A, Wechsler, Michael E, Zimmermann, Nives, Klion, Amy D, and Group, International Eosinophil Society Clinical Research Interest
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CHURG-Strauss syndrome ,TREATMENT effectiveness ,THERAPEUTICS ,BIOTHERAPY ,PHYSICIANS ,HYPEREOSINOPHILIC syndrome - Abstract
Rare eosinophil-associated disorders (EADs), including hypereosinophilic syndrome, eosinophilic granulomatosis with polyangiitis, and eosinophilic gastrointestinal disorders, are a heterogeneous group of conditions characterized by blood and/or tissue hypereosinophilia and eosinophil-related clinical manifestations. Although the recent availability of biologic therapies that directly and indirectly target eosinophils has the potential to dramatically improve treatment options for all EADs, clinical trials addressing their safety and efficacy in rare EADs have been relatively few. Consequently, patient access to therapy is limited for many biologics, and the establishment of evidence-based treatment guidelines has been extremely difficult. In this regard, multicenter retrospective collaborative studies focusing on disease manifestations and treatment responses in rare EADs have provided invaluable data for physicians managing patients with these conditions and helped identify important questions for future translational research. During the Clinical Pre-Meeting Workshop held in association with the July 2023 biennial meeting of the International Eosinophil Society in Hamilton, Ontario, Canada, the successes and limitations of pivotal multicenter retrospective studies in EADs were summarized and unmet needs regarding the establishment of guidelines for use of biologics in rare EADs were discussed. Key topics of interest included (1) clinical outcome measures, (2) minimally invasive biomarkers of disease activity, (3) predictors of response to biologic agents, and (4) long-term safety of eosinophil depletion. Herein, we report a summary of these discussions, presenting a state-of-the-art overview of data currently available for each of these topics, the limitations of the data, and avenues for future data generation through implementation of multidisciplinary and multicenter studies. [ABSTRACT FROM AUTHOR]
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- 2024
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21. Tezepelumab for refractory eosinophilic granulomatosis with polyangiitis-related asthma.
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Vincent-Galtié, Nina, Marquant, Quentin, Catherinot, Emilie, Ackermann, Felix, Magnan, Antoine, Tcherakian, Colas, and Groh, Matthieu
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THYMIC stromal lymphopoietin , *ASTHMA , *ASTHMATICS , *CHURG-Strauss syndrome , *PULMONARY eosinophilia , *WHEEZE - Abstract
Conventional immunosuppressants are ineffective for the management of EGPA-related asthma. Tezepelumab is a human monoclonal antibody that inhibits thymic stromal lymphopoietin (TLSP) that has proven efficacy in several phase 3 studies for the treatment of asthma. We treated with off-label tezepelumab the first two patients with severe refractory EPGA-related asthma. These preliminary findings suggest that targeting upstream signaling of the T2 inflammatory pathway can improve symptoms, reduce BVAS and increase Asthma Control Test scores, even in patients with refractory asthma who have failed several previous lines of treatment. Nevertheless, by analogy with dupilumab-induced IL-4/13 blockade, the persistence of sputum eosinophilia (reported in both patients) raises questions as to whether TSLP inhibition could lead to a rebound of eosinophilia and potentially to eosinophil-related symptoms in patients with EGPA. [ABSTRACT FROM AUTHOR]
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- 2024
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22. A case of eosinophilic granulomatosis with polyangiitis associated with diffuse alveolar haemorrhage: A case report and case-based review.
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Rira Kawaguchi, Hirohisa Usagawa, Yoshia Miyawaki, and Hiroshi Oiwa
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CHURG-Strauss syndrome , *BRAIN natriuretic factor , *PROGNOSIS , *PHYSICIANS , *LEUCOCYTES , *LEUKOCYTOCLASTIC vasculitis , *PULMONARY eosinophilia - Published
- 2024
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23. Stellate ganglion block in the treatment of SAPHO syndrome: A case report.
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Chenhao Jiang, Liangyu Cai, Jiannan Zhang, and Hongmei Zhou
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STELLATE ganglion block , *LEUKOCYTE count , *BLOOD cell count , *CHURG-Strauss syndrome , *TRANSCRIPTION factors , *NECK pain - Abstract
This article explores the use of stellate ganglion block (SGB) as a potential treatment for SAPHO syndrome, a rare and challenging inflammatory disease that affects the bones and skin. The study presents a case where SGB successfully alleviated symptoms and prevented disease progression in a patient with SAPHO syndrome. SGB, which blocks sympathetic nerves and improves immune function, has been used to treat other chronic pain and inflammatory conditions. The article suggests that SGB may be a promising treatment option for SAPHO syndrome and calls for more research in this area. [Extracted from the article]
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- 2024
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24. Multiple lymphadenopathies in eosinophilic granulomatosis with polyangiitis: Differentiating from IgG4-related lymphadenopathy.
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Jun-ichi Kurashina, Yasuhiro Shimojima, Dai Kishida, Takanori Ichikawa, Takeshi Uehara, and Yoshiki Sekijima
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CHURG-Strauss syndrome , *PLASMA cell diseases , *NOSOLOGY , *PLASMA cells , *GRANULOMATOSIS with polyangiitis , *HYPEREOSINOPHILIC syndrome - Abstract
This article presents a case study of a 75-year-old man with eosinophilic granulomatosis with polyangiitis (EGPA) who had multiple lymphadenopathies. The lymph node tissue biopsy revealed features of both EGPA and IgG4-related lymphadenopathy (IgG4-LAD). While IgG4-LAD is a distinct clinical category, some autoimmune diseases like EGPA can have similar lymphadenopathies. The presence of IgG4-positive plasma cells suggests a potential connection between EGPA and IgG4-related disease (IgG4-RD). The article also mentions the coexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-RD in some cases, as well as the role of hypocomplementemia in disease activity. [Extracted from the article]
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- 2024
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25. A case of subarachnoid haemorrhage associated with MPO-ANCA-positive eosinophilic granulomatosis with polyangiitis, successfully treated with glucocorticoid, cyclophosphamide, and mepolizumab.
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Yuki Satake, Shunsuke Sakai, Tetsuro Takao, and Takako Saeki
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CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *BLOOD diseases , *SYMPTOMS , *CEREBRAL arteriovenous malformations , *HYPEREOSINOPHILIC syndrome - Abstract
This article discusses a case of subarachnoid hemorrhage (SAH) associated with eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease. The patient experienced SAH along with other symptoms such as purpura, peripheral neuropathy, lung lesions, and rapidly progressive glomerulonephritis. Treatment included immunosuppressive therapy and the use of mepolizumab, a monoclonal antibody. The article highlights the importance of recognizing SAH as a potentially fatal complication of EGPA and the need for prompt treatment. Further research is needed to evaluate the effectiveness of mepolizumab for CNS involvement in EGPA. [Extracted from the article]
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- 2024
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26. Clinical Characteristics, Predictors for Mortality and Comparison of the Birmingham Vasculitis Activity Score and the Five-Factor Score on Survival in ANCA-Associated Vasculitis in Hong Kong.
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Yeung, Joshua Ka Ho and Young, Joyce Kit Yu
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GASTROINTESTINAL hemorrhage , *VASCULITIS , *GRANULOMATOSIS with polyangiitis , *CHURG-Strauss syndrome , *MORTALITY , *PNEUMOCYSTIS jiroveci - Published
- 2024
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27. Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden.
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Rathmann, Jens, Segelmark, Mårten, and Mohammad, Aladdin J
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VASCULITIS , *RESEARCH funding , *ANTINEUTROPHIL cytoplasmic antibodies , *MICROSCOPIC polyangiitis , *DESCRIPTIVE statistics , *GRANULOMATOSIS with polyangiitis , *STATISTICS , *CHURG-Strauss syndrome , *RHEUMATOLOGY , *CONFIDENCE intervals , *COMPARATIVE studies , *ALGORITHMS , *NOSOLOGY , *SENSITIVITY & specificity (Statistics) , *INTER-observer reliability , *BLOOD - Abstract
Objective To evaluate the ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification and compare them with the European Medicines Agency (EMA) algorithm and with classification based only on ANCA serology. Methods In the analysis, 374 cases (47% female) were classified according to the EMA algorithm, ANCA serology and ACR/EULAR criteria. The agreement rate was calculated using the kappa (κ) statistic. Results Under EMA, 192 patients were classified as granulomatosis with polyangiitis (GPA), 159 as microscopic polyangiitis (MPA) and 23 as eosinophilic granulomatosis with polyangiitis (EGPA). The ACR/EULAR criteria classified 199 patients as GPA, 136 as MPA and 22 as EGPA. Four patients (1.1%) met criteria of two disease categories, and 13 (3.5%) were unclassifiable. The observed agreement between EMA and ACR/EULAR was 85% for GPA, 75% for MPA and 96% for EGPA. The unweighted κ statistic was 0.66 (95% CI: 0.60, 0.74). Of the 188 PR3-ANCA positive patients, 186 (98.9%) were classified as GPA using ACR/EULAR criteria, and 135 of 161 (83.9%) MPO-ANCA positive patients were classified as MPA. With a classification solely based on ANCA specificity, agreement with ACR/EULAR was 99% for GPA and 88% for MPA. Conclusions EMA and ACR/EULAR classification give similar results. A small proportion of patients cannot be classified or fall into two categories. Some patients exhibiting granuloma, a key feature of GPA, are nevertheless classified as MPA, conflicting with the current view of histopathology of AAV. There is high agreement of ANCA-based classification with that of ACR/EULAR, reflected in the considerable weight granted to ANCA in the new criteria. These crucial elements within the new criteria necessitate a consensus discussion among field experts. [ABSTRACT FROM AUTHOR]
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- 2024
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28. Variation in approaches to acute ANCA‐associated vasculitis in Australia and New Zealand: rituximab, plasma exchange and glucocorticoids.
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Chua, Justin C. M., Dentrinos, Laura V., Kitching, Arthur R., and Ryan, Jessica
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VASCULITIS , *RESEARCH funding , *ANTINEUTROPHIL cytoplasmic antibodies , *RITUXIMAB , *CHI-squared test , *DESCRIPTIVE statistics , *DRUG efficacy , *CHURG-Strauss syndrome , *PLASMA exchange (Therapeutics) , *GLUCOCORTICOIDS , *CYCLOPHOSPHAMIDE , *IMMUNOMODULATORS - Abstract
Background: Anti‐neutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV) is a rare autoimmune disease which is managed by a range of specialities. There are limited data on treatment practices in Australia and New Zealand. Aims: To understand current patterns of acute AAV treatment in Australia and New Zealand. Methods: An online survey was conducted between July and October 2022 investigating physicians' views on the management of AAV, focusing on induction therapy. The survey contained questions pertaining to access to treatment and responses to clinical management scenarios. Eosinophilic granulomatosis with polyangiitis was not included. A chi‐squared test of independence was performed for statistical analysis. Results: From a total of 55 responses, plasma exchange was difficult to access for 44% of respondents, more so in rural centres, and they also had difficulty accessing infusion centres. New Zealand clinicians had more difficulty accessing rituximab, with only 44% reporting easy access compared with Australian clinicians (93%). With clinical management scenarios, there was variation in the dosing regimen of glucocorticoids and initiation of plasma exchange, with 42% of respondents prescribing a glucocorticoid regimen different from the standard of care, the 'reduced‐dose' arm of the Plasma Exchange and Glucocorticoids for the Treatment of ANCA‐Associated Vasculitis trial. The choice of cyclophosphamide or rituximab for induction therapy was based on patient characteristics and medical history. Conclusions: There is substantial variation in approaches to the acute management of AAV in Australia and New Zealand, including differences in resource availability. This variation in care demonstrates the need to implement current practice guidelines and institute contemporary monitoring of AAV management, to achieve best patient outcomes. [ABSTRACT FROM AUTHOR]
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- 2024
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29. Editorial: The present and future in rhinology.
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Sarafoleanu, Codrut
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NOSE , *NASAL polyps , *MEDICAL personnel , *CILIARY motility disorders , *CHURG-Strauss syndrome , *HUMAN anatomy - Abstract
This article explores the advancements in rhinology, focusing on the use of artificial intelligence, technology, and innovative techniques. It discusses how these advancements have improved diagnosis, treatment planning, and patient care options. The article also highlights the integration of virtual reality and augmented reality in surgical preparation, as well as the use of artificial intelligence and machine learning algorithms in decision-making and personalized patient care. Additionally, it mentions the potential of mobile health technology applications in improving patient compliance and disease management. The article concludes by discussing future innovations and emphasizes the importance of maintaining the human connection in medicine. [Extracted from the article]
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- 2024
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30. Eosinophilic granulomatosis with polyangiitis developed during treatment with benralizmab for severe asthma: A case report and literature review.
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Sakabe, Mitsukuni, Tobino, Kazunori, Obata, Yumi, Sogabe, Shota, Uchida, Kazuki, and Murakami, Yosuke
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CHURG-Strauss syndrome , *LITERATURE reviews , *ASTHMA , *PULMONARY eosinophilia , *SKIN biopsy , *AUTOIMMUNE diseases - Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disorder characterized by necrotizing vasculitis, asthma, and eosinophilia. We report a case of EGPA that developed during benralizumab treatment for severe asthma and provide a literature review. A 79‐year‐old Japanese male with severe asthma presented with generalized purpura 4 months after initiating benralizumab treatment. He had reduced his oral prednisolone dose from 7.5 to 2 mg/day. Laboratory tests revealed eosinophilia, and skin biopsy showed vasculitis with eosinophilic infiltration. He was diagnosed with EGPA and treated with corticosteroids, azathioprine, and mepolizumab, which led to rapid improvement and sustained remission. Five cases of EGPA developing during benralizumab treatment have been reported, with onset ranging from 14 to 36 weeks after initiation. Clinicians should monitor for EGPA development in patients receiving benralizumab, particularly during oral corticosteroid reduction. [ABSTRACT FROM AUTHOR]
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- 2024
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31. Monocentric study of IL-5 monoclonal antibody induction therapy for eosinophilic granulomatosis with polyangiitis.
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Wang, Chrong-Reen, Tsai, Hung-Wen, and Shieh, Chi-Chang
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CHURG-Strauss syndrome ,MONOCLONAL antibodies ,SUBCUTANEOUS injections ,DISEASE relapse ,DISEASE remission - Abstract
Although sporadic case reports have demonstrated successful management of eosinophilic granulomatosis with polyangiitis (EGPA) by anti-IL-5 therapy, larger-scale monocentric studies for the efficacy of mepolizumab (MEP), an IL-5 monoclonal antibody, are still lacking in Taiwan. Hospitalized EGPA patients aged at least 18 years were enrolled from November 1998 to October 2023, and analyzed for demographic, clinical, laboratory, medication and outcome data, focusing on the efficacy and safety of biologics use, particularly induction therapy with MEP. Twenty-seven EGPA patients aged 10–70 years (43 ± 15) at disease diagnosis were recruited with 21 under combined corticosteroids/cyclophosphamide induction therapy. Seventeen patients received biologics with 13 under MEP therapy. Ten patients aged 19–71 years (48 ± 15) completed 12-month induction therapy with a 100 mg quadri-weekly subcutaneous injection regimen indicated for active or relapse disease. There were reduced BVAS with complete remission in 6 and partial remission in 4 patients, lower CRP levels, decreased eosinophil counts with an inhibition of 92∼96 %, and tapered prednisolone dosages from 5 to 25 (13.0 ± 6.3) to 0–10 (3.3 ± 3.1) mg/day. Only one patient had an adverse event of injection site reactions. Nine patients received the same regimen for annual maintenance therapy. All had a persistent clinical remission. In these patients, 13–56 injections (41 ± 15) were prescribed with a follow-up period of 12∼52 months (38 ± 14). In this retrospective study, induction therapy with a 12-month 100 mg MEP quadri-weekly subcutaneous injection regimen demonstrates the efficacy and safety for active and relapsing EGPA patients. [ABSTRACT FROM AUTHOR]
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- 2024
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32. Treatment strategies for ANCA-associated vasculitides: from standard protocols to future horizons.
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Reggiani, Francesco, Stella, Matteo, Calatroni, Marta, and Sinico, Renato Alberto
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CHURG-Strauss syndrome ,MICROSCOPIC polyangiitis ,VASCULITIS ,COMPLEMENT inhibition ,PROGNOSIS ,GRANULOMATOSIS with polyangiitis - Abstract
ANCA-associated vasculitides (AAV), classified into granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis represent a group of disorders characterized by necrotizing vasculitis of small vessels, endothelial injury and tissue damage. The outcomes and prognosis of AAV have undergone significant changes with the introduction of glucocorticoids (GCs) and other immunosuppressants (cyclophosphamide, azathioprine, methotrexate, and mycophenolate mofetil). The enhanced understanding of pathogenesis has subsequently led to the incorporation into clinical practice of drugs targeting specific therapeutic targets. After an extensive literature search of Pubmed, Medline, Embase of the most recent evidence, we provide an overview of available treatments, highlighting how newer drugs have integrated into standard protocols. Our review also explores potential new therapeutic targets, including B cell depletion and inhibition, T cell inhibition, complement inhibition, and IL-5 and IgE inhibition. There is hope that the new treatment targets currently under study in AAV may enable a faster and more lasting clinical response, ensuring the reduction of possible side effects from therapies. Moreover, numerous aspects necessitate further exploration in the future, such as tailoring of GCs, integration of GCs-sparing agents, efficacy of combination therapy, optimal maintenance therapy, to reduce organ-damage and improve quality of life. [ABSTRACT FROM AUTHOR]
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- 2024
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33. Asthma bronchiale und allergische Rhinitis – die Hautprobe offenbart eine schwerwiegende Systemerkrankung.
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Wölbing, Priscila, Dugas-Breit, Susanne, Hartschuh, Wolfgang, and Toberer, Ferdinand
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Copyright of Die Dermatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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- 2024
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34. The Association Between Age at Diagnosis and Disease Characteristics and Damage in Patients With ANCA-Associated Vasculitis.
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Corbridge, Thomas, Khalidi, Nader, Koening, Curry, Langford, Carol, McAlear, Carol, Monach, Paul, Moreland, Larry, Pagnoux, Christian, Rhee, Rennie, Seo, Philip, Silver, Jared, Specks, Ulrich, Warrington, Kenneth, Wechsler, Michael, Merkel, Peter, Bloom, Jessica, Pickett-Nairn, Kaci, Silveira, Lori, Fuhlbrigge, Robert, Cuthbertson, David, and Akuthota, Praveen
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Child ,Middle Aged ,Young Adult ,Humans ,Female ,Aged ,Male ,Antibodies ,Antineutrophil Cytoplasmic ,Prospective Studies ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Granulomatosis with Polyangiitis ,Microscopic Polyangiitis ,Hemorrhage ,Churg-Strauss Syndrome - Abstract
OBJECTIVE: This study examined the relationship between age at diagnosis and disease characteristics and damage in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: Analysis of a prospective longitudinal cohort of patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA) in the Vasculitis Clinical Research Consortium (2013-2021). Disease cohorts were divided by age at diagnosis (years): children (65). Data included demographics, ANCA type, clinical characteristics, Vasculitis Damage Index (VDI) scores, ANCA Vasculitis Index of Damage (AVID) scores, and novel disease-specific and non-disease-specific damage scores built from VDI and AVID items. RESULTS: Analysis included data from 1020 patients with GPA/MPA and 357 with EGPA. Female predominance in GPA/MPA decreased with age at diagnosis. AAV in childhood was more often GPA and proteinase 3-ANCA positive. Children with GPA/MPA experienced more subglottic stenosis and alveolar hemorrhage; children and young adults with EGPA experienced more alveolar hemorrhage, need for intubation, and gastrointestinal involvement. Older adults (GPA/MPA) had more neurologic manifestations. After adjusting for disease duration, medications, tobacco, and ANCA, all damage scores increased with age at diagnosis for GPA/MPA (P
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- 2023
35. Genetic and Non-Genetic Contributions to Eosinophilic Granulomatosis with Polyangiitis: Current Knowledge and Future Perspectives
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Mirko Treccani, Laura Veschetti, Cristina Patuzzo, Giovanni Malerba, Augusto Vaglio, and Davide Martorana
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EGPA ,genetics ,ANCA ,eosinophils ,Churg–Strauss syndrome ,Biology (General) ,QH301-705.5 - Abstract
In this work, we present a comprehensive overview of the genetic and non-genetic complexity of eosinophilic granulomatosis with polyangiitis (EGPA). EGPA is a rare complex systemic disease that occurs in people presenting with severe asthma and high eosinophilia. After briefly introducing EGPA and its relationship with the anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitis (AAVs), we delve into the complexity of this disease. At first, the two main biological actors, ANCA and eosinophils, are presented. Biological and clinical phenotypes related to ANCA positivity or negativity are explained, as well as the role of eosinophils and their pathological subtypes, pointing out their intricate relations with EGPA. Then, the genetics of EGPA are described, providing an overview of the research effort to unravel them. Candidate gene studies have investigated biologically relevant candidate genes; the more recent genome-wide association studies and meta-analyses, able to analyze the whole genome, have confirmed previous associations and discovered novel risk loci; in the end, family-based studies have dissected the contribution of rare variants and the heritability of EGPA. Then, we briefly present the environmental contribution to EGPA, reporting seasonal events and pollutants as triggering factors. In the end, the latest omic research is discussed and the most recent epigenomic, transcriptomic and microbiome studies are presented, highlighting the current challenges, open questions and suggesting approaches to unraveling this complex disease.
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- 2024
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36. A Study to Evaluate the Efficacy and Safety of SHR-1703 in Subjects With Eosinophilic Granulomatosis With Polyangiitis (EGPA)
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- 2023
37. Pediatric Vasculitis Initiative (PedVas)
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BC Childrens Hospital Research Institute, University of Oxford, and David Cabral, Principle Investigator
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- 2023
38. Efficacy and Safety of Depemokimab Compared With Mepolizumab in Adults With Relapsing or Refractory Eosinophilic Granulomatosis With Polyangiitis (EGPA) (OCEAN)
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- 2023
39. The Evaluation of Changing the Eponym Churg–Strauss Syndrome Due to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.
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Sargin, Gokhan
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CHURG-Strauss syndrome , *VASCULITIS , *DATABASES , *MUCOCUTANEOUS lymph node syndrome - Abstract
Background: Eponyms do not describe any pathogenesis of a disease. So, there is no other way than to memorize the disease or anatomical area. Over the years, new nomenclatures have been suggested for some diseases due to a better understanding of the pathogenesis. In this article, the changes in the use of Churg–Strauss syndrome were investigated. Methods: In the study, a computerized search was performed using the PubMed database. Books and documents, clinical trials, editorials, meta-analyses, reviews, and case reports were included in the study. Data were obtained from the title of the database, and the variations or distribution by year for the nomenclature of the most related studies were evaluated. Results: Overall, 68.3% of the articles included CSS, 25.7% included eosinophilic granulomatous polyangiitis (EGPA), and 6.0% included both nomenclatures. When evaluated in terms of the distribution according to years, it was determined that there was a statistically significant increase in use in terms of EGPA. When evaluated among specific section journals, the highest rate was in Rheumatology (29.4%). The highest rate of using CSS was in the Rheumatology (25.1%) journals, followed by Pulmonary/Respiratory (17%), Cardiovascular (12%), and Allergy/Immunology/Biology (9.8%). The use of EGPA combined with CSS decreased in all the specific journals from 2012 to the present. Conclusions: The findings of the study revealed that the number of articles with the eponym of EGPA showed an increased frequency in contrast to a decreasing frequency for those with CSS during recent years. Today, with the elaboration of the disease pathogenesis and the increase in knowledge, the trend has shifted in this direction. [ABSTRACT FROM AUTHOR]
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- 2024
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40. Eosinophilic granulomatosis with polyangiitis and its association with montelukast: a case-based review.
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Alexander, Grace, Moore, Steven A., and Lenert, Petar S.
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CHURG-Strauss syndrome , *DRUG side effects , *MONTELUKAST , *LITERATURE reviews , *DISEASE relapse , *MYOSITIS - Abstract
The association between the use of certain medications (including sulfonamides, hydralazine, and procainamide) and the occurrence of drug-induced lupus or hepatitis is well established. More recently, cases of immune-related adverse events ranging from inflammatory polyarthritis to necrotizing myositis in patients taking checkpoint inhibitors have been reported. However, data linking drugs to systemic vasculitis are scarce and at times debatable. Propylthiouracil, hydralazine, and minocycline have been associated with rare cases of ANCA-associated syndromes, including life-threatening pulmonary-renal syndromes and systemic polyarteritis nodosa-like diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) has been reported in patients taking leukotriene inhibitors. Since the link between the use of leukotriene inhibitors and occurrence of EGPA remains highly controversial, we performed a literature review for cases of EGPA in patients taking montelukast without prior history of oral corticosteroid use. We found 24 cases, along with our own two cases described, making 26 cases in total. The mean age was 43 and a majority (18/26) were female. In majority of cases EGPA-like disease never relapsed after they were taken off leukotriene inhibitors suggesting a clear causal relationship between the use of these drugs and occurrence of eosinophil-rich systemic EGPA. [ABSTRACT FROM AUTHOR]
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- 2024
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41. Large vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis.
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Kaymakci, Mahmut S, Elfishawi, Mohanad M, Langenfeld, Hannah E, Hanson, Andrew C, Crowson, Cynthia S, Bois, Melanie C, Ghaffar, Umar, Koster, Matthew J, Specks, Ulrich, and Warrington, Kenneth J
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VASCULITIS , *BLOOD vessels , *ANTINEUTROPHIL cytoplasmic antibodies , *MICROSCOPIC polyangiitis , *METHOTREXATE , *DESCRIPTIVE statistics , *RITUXIMAB , *AORTA , *GRANULOMATOSIS with polyangiitis , *CHURG-Strauss syndrome , *CLINICS , *TEMPORAL arteries , *ALGORITHMS , *HISTOLOGY , *GLUCOCORTICOIDS , *SYMPTOMS - Abstract
Objectives ANCA-associated vasculitis (AAV) is currently categorized under the small vessel vasculitides. There is limited knowledge about large vessel involvement in AAV (L-AAV), mainly described in case reports and small series. L-AAV can involve temporal arteries (TA-AAV), aorta (A-AAV), and periaortic soft tissue (PA-AAV). We sought to characterize the features of patients with L-AAV. Methods Patients older than 18 years at diagnosis of TA-AAV, A-AAV and PA-AAV seen at the Mayo Clinic, Rochester between 1 January 2000 and 31 December 2021 were identified through a proprietary medical text search algorithm. Patients were included if diagnosed with L-AAV, fulfilled 2022 ACR/EULAR classification criteria for GPA, MPA or EGPA, had positive ANCA test results, and had more than one outpatient or inpatient visit. Results The study cohort consists of 36 patients with L-AAV. Of those, 23 had p-ANCA and/or MPO-ANCA, and 13 had c-ANCA and/or PR3-ANCA. Mean (s. d.) age at AAV diagnosis was 63.4 (12.79) years; 20 (56%) were male. Seventeen patients had TA-AAV, 10 had A-AAV and 9 had PA-AAV. Most patients (n = 25, 69%) were diagnosed with large vessel vasculitis and AAV within a 1-year timespan. Twenty-five (69%) patients had histopathological confirmation of AAV diagnosis in a location other than temporal artery, aorta or periaortic soft tissue. Glucocorticoids (36/36), rituximab (19/36) and methotrexate (18/36) were the most frequent treatments. Conclusion This is the largest single-centre cohort of patients with L-AAV to date. AAV can involve large arteries, albeit infrequent. AAV-targeted therapy should be considered in patients with L-AAV. [ABSTRACT FROM AUTHOR]
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- 2024
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42. ANCA-associated vasculitis—treatment standard.
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Chalkia, Aglaia and Jayne, David
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CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *VASCULITIS , *ANTINEUTROPHIL cytoplasmic antibodies , *COMPLEMENT inhibition - Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are characterized by small-vessel necrotizing inflammation, and prior to the advent of immunosuppressive therapy frequently had a fatal outcome. Treatment has transformed AAV into a relapsing/remitting disease with increased drug-related toxicities and organ damage. The use of glucocorticoids, cyclophosphamide and immunosuppressives (including azathioprine, mycophenolate and methotrexate) was optimized through a sequence of clinical trials establishing a standard of care against which subsequent targeted therapies could be developed. Improved understanding of pathophysiology has supported the development of B-cell depletion and complement inhibition in granulomatosis with polyangiitis and microscopic polyangiitis, and interleukin 5 inhibition for eosinophilic granulomatosis with polyangiitis, leading to the approval of newer agents for these conditions. There has been an increased attention on minimizing the adverse effects of treatment and on understanding the epidemiology of comorbidities in AAV. This review will focus on recent evidence from clinical trials, especially with respect to glucocorticoids, avacopan, plasma exchange, rituximab and mepolizumab, and their interpretation in the 2022 management recommendations by the European League of Associations of Rheumatology. [ABSTRACT FROM AUTHOR]
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- 2024
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43. Performance of the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for antineutrophil cytoplasmic antibody-associated vasculitis in previously diagnosed adult patients from Türkiye.
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Yilmaz, Sedat, Kucuk, Hamit, Ozgunen, Merve Sungur, Kardas, Riza Can, Tecer, Duygu, Vasi, Ibrahim, Cinar, Muhammet, and Ozturk, Mehmet Akif
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VASCULITIS , *NONPROFIT organizations , *PREDICTIVE tests , *CROSS-sectional method , *ANTINEUTROPHIL cytoplasmic antibodies , *PROFESSIONAL associations , *MICROSCOPIC polyangiitis , *RETROSPECTIVE studies , *GRANULOMATOSIS with polyangiitis , *CHURG-Strauss syndrome , *STATISTICS , *SENSITIVITY & specificity (Statistics) , *RHEUMATOLOGISTS , *ALGORITHMS - Abstract
Objectives: This study aimed to evaluate the applicability of the new 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria in Turkish adult patients previously diagnosed with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Patients and methods: One hundred sixty-four patients (96 males, 68 females; mean age: 49.6±14.4 years; range, 18 to 87 years) diagnosed with AAV by experienced rheumatologists between July 2016 and May 2022 were included in this retrospective cross-sectional study and reclassified based on the 1990 ACR criteria, the European Medicines Agency (EMEA) algorithm, and the 2022 ACR/EULAR criteria. For external validation, 83 patients (48 males, 35 females; mean age: 47.3±17.5 years; range, 19 to 81 years) diagnosed with immunoglobulin (Ig)A vasculitis were included. Results: One hundred twenty-six (76.8%) patients had granulomatosis with polyangiitis (GPA), 13 (7.9%) patients had eosinophilic granulomatosis with polyangiitis (EGPA), and 25 (15.2%) patients had microscopic polyangiitis (MPA). According to the criteria, the number of unclassified patients was nine (5.5%) for both the 2022 ACR/EULAR AAV classification criteria and the EMEA algorithm. The new criteria had an almost perfect agreement with the clinician's diagnosis (Cohen's kappa coefficient [k]=0.858 for GPA, k=0.820 for EGPA, and k=0.847 for MPA). The kappa statistics for agreement of 2022 ACR/EULAR classification criteria with the EMEA algorithm were found 0.794 for GPA, 0.820 for EGPA, and 0.700 for MPA. None of the 83 patients diagnosed with IgA vasculitis could be classified as GPA, EGPA, or MPA using the new ACR/EULAR AAV classification criteria. Conclusion: The 2022 ACR/EULAR classification criteria for AAV showed substantial or perfect agreement with the clinical diagnosis and the EMEA algorithm. [ABSTRACT FROM AUTHOR]
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- 2024
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44. Application of biological agents in the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis.
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Weijun Liu, Guanyuan Tian, Chao Chen, Mingying Zhang, Zhanmao Chen, Tietao Chen, Zhibin Lin, Wuzhong Wu, Yiqaing Wu, Kefei Wu, and Qinghua Liu
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CHURG-Strauss syndrome ,ANTINEUTROPHIL cytoplasmic antibodies ,VASCULITIS ,GLUCOCORTICOIDS ,RITUXIMAB ,MICROSCOPIC polyangiitis ,MEDICAL research - Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been traditionally treated using glucocorticoids and immunosuppressants. However, these treatment modes are associated with high recurrence AAV rates and adverse reactions. Therefore, treatment strategies for AAV need to be urgently optimized. The efficacy and safety of biological agents in the treatment of vasculitis have been clinically validated. This review comprehensively summarizes the evidence-based support for the clinical use of existing biological agents in AAV. The findings reveal that multiple biological agents not only effectively reduce the adverse reactions associated with glucocorticoids and immunosuppressants but also demonstrate significant therapeutic efficacy. Notably, rituximab, an anti-CD20 antibody, has emerged as a first-line treatment option for AAV. Mepolizumab has shown promising results in relapsed and refractory eosinophilic granulomatosis with polyangiitis. Other biological agents targeting cytokines, complement, and other pathways have also demonstrated clinical benefits in recent studies. The widespread application of biological agents provides new insights into the treatment of AAV and is expected to drive further clinical research. These advancements not only improve patient outcomes but also offer more possibilities and hope in the field of AAV treatment. [ABSTRACT FROM AUTHOR]
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- 2024
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45. Benralizumab Reduces Respiratory Exacerbations and Oral Glucocorticosteroid Dose in Patients with Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis.
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Mümmler, Carlo, Mertsch, Pontus, Barnikel, Michaela, Haubner, Frank, Schönermarck, Ulf, Grabmaier, Ulrich, Schulze-Koops, Hendrik, Behr, Jürgen, Kneidinger, Nikolaus, and Milger, Katrin
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CHURG-Strauss syndrome ,ASTHMATICS ,REMISSION induction ,DISEASE exacerbation ,ASTHMA - Abstract
Background: Benralizumab reduces exacerbations and long-term oral glucocorticosteroid (OCS) exposure in patients with severe eosinophilic asthma. In patients with eosinophilic granulomatosis with polyangiitis (EGPA), uncontrolled symptoms and exacerbations of asthma and chronic rhinosinusitis (CRS) are important reasons for continued OCS therapies. We aimed to describe outcomes of patients with severe asthma and EGPA treated with benralizumab in real-life. Methods: We retrospectively analyzed adult patients from the Severe Asthma Unit at LMU Munich diagnosed with severe asthma and EGPA treated with benralizumab, differentiating two groups: Group A, patients with a stable daily OCS dose and diagnosis of EGPA > 6 months ago; and Group B, patients treated with high-dose daily OCS due to recent diagnosis of EGPA < 6 months ago. We compared outcome parameters at baseline and 12 months after initiation of benralizumab, including respiratory exacerbations, daily OCS dose, and lung function. Results: Group A included 17 patients, all receiving OCS therapy and additional immunosuppressants; 15 patients (88%) continued benralizumab for more than 12 months, demonstrating a significant reduction in daily OCS dose and exacerbations while FEV1 increased. Group B included 9 patients, all with high-dose daily OCS and some receiving cyclophosphamide pulse therapy for life-threatening disease. Benralizumab addition during induction was well tolerated. A total of 7/9 (78%) continued benralizumab for more than 12 months and preserved EGPA remission at the 12-month timepoint. Conclusion: In this real-life cohort of patients with severe asthma and EGPA, benralizumab initiation during remission maintenance reduced respiratory exacerbations and daily OCS dose. Benralizumab initiation during remission induction was associated with a high rate of clinical EGPA remission. [ABSTRACT FROM AUTHOR]
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- 2024
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46. Eosinophilic granulomatous polyangiitis with central nervous system involvement in children: a case report and literature review.
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Nana Nie, Lin Liu, Cui Bai, Dahai Wang, Shan Gao, Jia Liu, Ranran Zhang, Yi Lin, Qiuye Zhang, and Hong Chang
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CENTRAL nervous system ,LITERATURE reviews ,CHURG-Strauss syndrome ,LEUCOCYTES ,ANTINEUTROPHIL cytoplasmic antibodies ,PULMONARY eosinophilia ,EPILEPSY - Abstract
Objective: To explore the clinical characteristics and treatment outcomes of children with central nervous system (CNS) involvement in eosinophilic granulomatosis with polyangiitis (EGPA). Methods: A child who presented with EGPA complicated by CNS involvement was admitted to our hospital in June 2023. The clinical features were analyzed retrospectively, and relevant literatures were reviewed to provide a comprehensive overview of this condition. Results: A ten-year-old girl, who had a history of recurrent cough and asthma accompanied by peripheral blood eosinophilia for eight months, was admitted to our hospital. On admission, spotted papules were visible on her hands and feet, bilateral pulmonary rales were audible. The laboratory examination revealed that the proportion of eosinophils (EOS) exceeded 10% of white blood cells, the anti-neutrophil cytoplasmic antibody (MPO-ANCA) was positive, the immunoglobulin G level was 15.80g/L, and the immunoglobulin E level was greater than 2500.00IU/mL. The imaging examination showed multiple patchy and nodular high-density shadows in both lungs as well as sinusitis. Pulmonary function tests indicated moderate ventilation and diffusion dysfunction. Bone marrow cytology demonstrated a significant increase in the proportion of eosinophils. Skin pathology confirmed leukocytoclastic vasculitis. During the hospitalization, the child had a convulsion. The magnetic resonance imaging (MRI) scan of the brain showed multiple abnormal signal shadows in the bilateral cerebral cortex and the electroencephalogram (EEG) showed epileptic waves. Following the administration of methylprednisolone pulse therapy in combination with cyclophosphamide treatment, her cough and asthma resolved, the skin rash disappeared without any further convulsions. We found that only a young EGPA patient with CNS involvement had been previously reported. The previously reported case began with long-term fever, weight loss, and purpuric rash. Both patients responded well to treatment with glucocorticoids and cyclophosphamide, experiencing significant improvement in their clinical symptoms and normalization of their peripheral blood eosinophils. Conclusion: The diagnosis of EGPA in children can be challenging. When a child is affected by EGPA, it is essential to remain vigilant for signs of CNS involvement. The treatment with glucocorticoids and cyclophosphamide is effective in managing EGPA in children. [ABSTRACT FROM AUTHOR]
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- 2024
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47. Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome.
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Fouka, Evangelia, Drakopanagiotakis, Fotios, and Steiropoulos, Paschalis
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ANTI-glomerular basement membrane disease , *CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *PULMONARY manifestations of general diseases , *VASCULITIS - Abstract
Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals. [ABSTRACT FROM AUTHOR]
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- 2024
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48. Ophthalmic vascular manifestations in eosinophil-associated diseases: a comprehensive analysis of 57 patients from the CEREO and EESG networks and a literature review.
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Chapuis, Elisa, Bousquet, Elodie, Viallard, Jean-François, Terrier, Benjamin, AmouraK, Zahir, Batani, Veronica, Brézin, Antoine, Cacoub, Patrice, Caminati, Marco, Chaza, Thibaud, Comarmond, Cloé, Durieu, Isabelle, Ebbo, Mikael, Grall, Maximilien, Ledoult, Emmanuel, Losappio, Laura, Mattioli, Irene, Mèkinian, Arsène, Padoan, Roberto, and Regola, Francesca
- Subjects
RETINAL vein occlusion ,LITERATURE reviews ,RETINAL artery occlusion ,CHURG-Strauss syndrome ,RETINAL artery ,VENOUS thrombosis - Abstract
Introduction: Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia. Methods: We conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (=0.5 x109/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations. Results: Fifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher's retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x109/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x109/L in all cases (n=4). Discussion: This study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented. [ABSTRACT FROM AUTHOR]
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- 2024
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49. Evaluation of Ministry of Health, Labour and Welfare diagnostic criteria for antineutrophil cytoplasmic antibody–associated vasculitis compared to ACR/EULAR 2022 classification criteria.
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Sada, Ken-ei, Nagasaka, Kenji, Kaname, Shinya, Higuchi, Tomoaki, Furuta, Shunsuke, Nanki, Toshihiro, Tsuboi, Naotake, Amano, Koichi, Dobashi, Hiroaki, Hiromura, Keiju, Bando, Masashi, Wada, Takashi, Arimura, Yoshihiro, Makino, Hirofumi, and Harigai, Masayoshi
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CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *VASCULITIS , *CLASSIFICATION , *ANTINEUTROPHIL cytoplasmic antibodies - Abstract
Objective: This study aimed to evaluate the Ministry of Health, Labour and Welfare (MHLW) diagnostic criteria for antineutrophil cytoplasmic antibody–associated vasculitis compared to the new American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 criteria. Methods: Two nationwide cohort studies were used, and participants were categorised as having eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA) according to the American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 and MHLW criteria. Results: Of the entire patient population, only 10 (2.1%) were unclassifiable according to the MHLW probable criteria, while a significant number of patients (71.3%) met at least two criteria. The MHLW probable criteria for MPA had some challenges in differentiating between MPA and eosinophilic granulomatosis with polyangiitis, and the same was true for MHLW probable criteria for GPA in differentiating MPA from GPA. Nevertheless, improved classification results were obtained when the MHLW probable criteria were applied in the order of eosinophilic granulomatosis with polyangiitis, MPA, and GPA. Conclusions: The application of MHLW criteria could categorise a substantial number of patients with antineutrophil cytoplasmic antibody–associated vasculitis into one of the three antineutrophil cytoplasmic antibody–associated vasculitis diseases. The classification was in accordance with the American College of Rheumatology/European Alliance of Associations for Rheumatology 2022 criteria when considering the order of application. [ABSTRACT FROM AUTHOR]
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- 2024
- Full Text
- View/download PDF
50. Infecciones graves en vasculitis necrosantes sistémicas.
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Pena, Claudia, Costi, Ana Carolina, García, Lucila, and García, Mercedes
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POLYARTERITIS nodosa , *CHURG-Strauss syndrome , *MICROSCOPIC polyangiitis , *VASCULITIS , *VARICELLA-zoster virus , *URINARY tract infections - Abstract
Las infecciones en pacientes con vasculitis sistémica representan una de las principales causas de mortalidad. Son factores de riesgo conocidos la edad, el compromiso orgánico asociado, el uso de corticoides y de inmunosupresores, asi como el requerimiento de diálisis, entre otros. Determinar la prevalencia de infección grave y factores asociados en pacientes diagnosticados de vasculitis asociada a ANCA poliarteritis nudosa (PAN). Estudio retrospectivo realizado en un único centro de reumatología entre los años 2000 y 2018. Se incluyó a pacientes con granulomatosis con poliangitis, granulomatosis eosinofílica con poliangitis, poliangitis microscópica y PAN. Se registraron episodios infecciosos graves que requirieron hospitalización o tratamiento antibiótico/antiviral prolongado, infección recurrente por virus del herpes zóster o infecciones oportunistas. Se incluyó a 105 pacientes, la mediana del tiempo de seguimiento fue de 18 meses; el 58,7% eran mujeres, con una mediana de edad de 52 años; el 41,9% presentaban poliangitis microscópica, el 16,2% granulomatosis eosinofílica con poliangitis, el 40% granulomatosis con poliangitis y el 1,9% PAN. Los compromisos constitucional, pulmonar, renal y otorrinolaringológico fueron los más frecuentes. fue del 34,2% con una mediana de 3 meses desde el diagnóstico de vasculitis. Las infecciones de vías respiratorias bajas (42,8%), la sepsis (31,4%) y el tracto urinario (14,3%) fueron los sitios de infección más comunes. Predominó la etiología bacteriana (67,7%). La mortalidad en el primer episodio fue del 14,3%. Se encontraban en fase de inducción de tratamiento el 72,2%. Las infecciones se asociaron significativamente con edad > 65 años (p = 0,030), compromiso pulmonar (p = 0,016), renal (p = 0,001), BVAS3 >15 y mortalidad (p = 0,0002). La prevalencia de infecciones graves fue del 34,2%. Las infecciones pulmonares, septicemia y las urinarias fueron las más frecuentes y se asociaron a compromiso renal y pulmonar, así como a mortalidad, especialmente en pacientes de edad avanzada. Infections in patients with systemic vasculitis represent one of the main causes of mortality. Corticosteroid use, immunosuppressive therapy, age, associated organic involvement and dialysis dependence are risk factors of infection. To determine the prevalence of severe infection and associated factors in patients diagnosed with ANCA-associated vasculitis and polyarteritis nodosa (PAN). Retrospective study was conduced in a single rheumatology center (2000-2018). We included patients diagnosed with ANCA-associated vasculitis (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis and PAN. Serious infectious events requiring hospitalization or prolonged antibiotic/antiviral treatment, recurrent infection of herpes zoster virus or opportunistic infections were evaluated. Sites of infection, isolated microorganisms and mortality related were analyzed. A total of 105 patients were analyzed, follow-up time median 18 months, 58.7% were women and median age was 52 years. Types of vasculitis: 41.9% microscopic polyangiitis, 16.2% eosinophilic granulomatosis with polyangiitis, 40% granulomatosis with polyangiitis, 1.9% PAN. Constitutional, pulmonary, renal and otorhinolaryngology manifestations were the most frequent. was 34,2%, with a median of 3 months from diagnosis of vasculitis to the infectious event. Low respiratory tract (42.8%), sepsis (31.4%), and urinary tract (14.3%) were the most common sites of infections. Bacterial etiology was the most prevalent (67.7%). Mortality at the first event was 14.3% and a 72.2% of patients were in the induction phase of treatment. Infectious events were significantly associated with age > 65 years (P = 0.030), presence of lung (P = 0.016) and renal involvement (P = 0.001), BVASv3 > 15 and mortality (P = 0.0002). The prevalence of infection was 34.2%. Lower airway infections, septicemia and urinary tract infections were the most prevalent. Infections were associated with renal and pulmonary involvement, age older than 65 years and score BVAS >15. Severe infections were associated with mortality, especially in elderly patients. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
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