110. classification Criteria for the Anca-Associated Vasculitides

Robson, Joanna; Grayson, Peter C.; Ponte, Cristina; Suppiah, Ravi; Craven, Anthea; Khalid, Sara; Judge, Andrew; Hutchings, Andrew; Watts, Richard A.; Merkel, Peter A.; Luqmani, Raashid

doi:10.1093/rheumatology/kez058.050

Cited by 35 publications

(42 citation statements)

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“…The diagnosis of EGPA has been a controversial topic over the years in terms of whether pathology with biopsy is required for definitive diagnosis or whether a clinical diagnosis based on the presence of syndromic components is sufficient [82]. Several groups have developed diagnostic criteria for EGPA, including the Chapel Hill, American College of Rheumatology (ACR) and Lanham criteria [81,83,84]; however, the phase 3 mepolizumab clinical trial diverged from these standards, defining EGPA by the presence of asthma and eosinophilia (>1000 cells•µL −1 and/or >10% of leukocytes) in addition to at least two of the following: positive biopsy findings (eosinophilic vasculitis, perivascular eosinophilic infiltration, eosinophil-rich granulomatous inflammation), neuropathy (mononeuritis multiplex or polyneuropathy), non-fixed pulmonary infiltrates, sinonasal abnormality (rhinosinusitis with or without nasal polyps), cardiomyopathy, glomerulonephritis, alveolar haemorrhage, palpable purpura, or a positive test for ANCA (myeloperoxidase or proteinase 3) [85]. A positive ANCA test is not mandatory and is present in only a subset (approximately 35-40%) of patients with EGPA [82].…”

Section: Eosinophils and Other Airway Diseasesmentioning

confidence: 99%

Eosinophils and eosinophilic immune dysfunction in health and disease

2022

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The functions ascribed to eosinophils have classically been limited to host defence against certain parasitic infections and potentially deleterious effects in the setting of specific diseases that are associated with elevated eosinophil counts in blood and/or tissue. The ability to induce eosinophil depletion either experimentally in animal models or through targeted therapies in humans has extended our understanding of the roles played by eosinophils in health and homeostasis as well as in disease pathogenesis. When associated with human disease aetiology, the eosinophil takes on a pathogenic rather than a protective role. This maladaptive response, called “eosinophilic immune dysfunction” herein, appears central to exacerbation pathogenesis and disease control in severe asthma and may be involved in the aetiology of other eosinophil-related conditions ranging from organ-system-limited diseases such as phenotypic subsets of chronic obstructive pulmonary disease and chronic rhinosinusitis with nasal polyposis to more broadly systemic diseases such as eosinophilic granulomatosis with polyangiitis and hypereosinophilic syndrome. In this review, we describe the evidence supporting eosinophilic functions related to health and homeostasis and explore the contribution of eosinophilic immune dysfunction to human disease.

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Section: Eosinophils and Other Airway Diseasesmentioning

confidence: 99%

Eosinophils and eosinophilic immune dysfunction in health and disease

2022

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“…За поставяне на диагноза EGPA пациентите трябва да отговарят на определени критерии, фигуриращи в поне една от препоръките на: Американски Колегиум по ревматология (American College of Rheumatology, ACR) за грануломатозен полиангиит (1990), консенсусната конференция от Chapel Hill за номенклатура на васкулитите (Chapel Hill Consensus Conference, CHCC) (1994, 2012) и алгоритъма на Европейската агенция по лекарствата (European Medical Agency, EMA) (2007) [7][8][9][10]. През 2017 г. пред ACR и Европейската Лига срещу Ревматизма (European League Against Rheumatism, EULAR) са предложени нови класификационни критерии за първичните системни васкулити, предоставящи 85% сензитивност и 99% специфичност за диагностициране на EGPA [7,11,12].…”

Section: въведениеunclassified

“…EGPA не се наблюдава често при деца и юноши. В тази възрастова група заболяването има по-агресивен ход, с изразена белодробна и сърдечносъдова манифестация [12]. Не се отбелязват фамилност или етнически различия в появата и развитието на EGPA.…”

Section: обобщениепидемиологичниданниотOrphanetидруги публикацииunclassified

Епидемиология На Еозинофилната Грануломатоза С Полиангиит

Стефанов¹,

Митова²

2021

Редки болести и лекарства сираци

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Еозинофилната грануломатоза с полиангиит (Eosinophilic granulomatosis with polyangiitis, EGPA), наричана до скоро синдром на Churg-Strauss, е системен некротизиращ васкулит с мултиорганно засягане, който се проявява най-често с астма, еозинофилия и некротизиращи екстравазални грануломи, а при част от болните се намират анти-неутрофилни цитоплазмени автоантитела (ANCA). EGPA е един от най-рядко срещаните първични системни васкулити. След първото описание на заболяването през 1951 г., класификацията му е претърпяла множество промени, целящи прецизиране на диагностичния процес и навременно лечение. За поставяне на диагнозата пациентите трябва да отговарят на определени критерии. Липсата на унифициран подход при прилагането на тези критерии, както и припокриването на клиничната симптоматика с други първични системни васкулити затрудняват изучаването на епидемиологията на EGPA. Настоящата публикация има за цел да анализира епидемиологични данни за EGPA и да предостави оценка за разпространението на това заболяване в България.

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“…With these criteria, both clinical and laboratory parameters, including ANCA testing and imaging study findings, are considered, and a different weight or score is applied to each criterion. Nodules, masses, or cavitations on chest images; and inflammation, consolidation, or effusion of the nasal or paranasal sinuses are among the imaging findings (8).…”

Section: Introductionmentioning

confidence: 99%

From Head to Toe: Granulomatosis with Polyangiitis

et al. 2021

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Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody-associated vasculitis. It is an uncommon multisystem disease involving predominantly small vessels and is characterized by granulomatous inflammation, pauci-immune necrotizing glomerulonephritis, and vasculitis. GPA can involve virtually any organ. Clinical manifestations are heterogeneous and can be classified as granulomatous (eg, ear, nose, and throat disease; lung nodules or masses; retro-orbital tumors; pachymeningitis) or vasculitic (eg, glomerulonephritis, alveolar hemorrhage, mononeuritis multiplex, scleritis). The diagnosis of GPA relies on a combination of clinical findings, imaging study results, laboratory test results, serologic markers, and histopathologic results. Radiology has a crucial role in the diagnosis and follow-up of patients with GPA. CT and MRI are the primary imaging modalities used to evaluate GPA manifestations, allowing the differentiation of GPA from other diseases that could simulate GPA. The authors review the main clinical, histopathologic, and imaging features of GPA to address the differential diagnosis in the affected organs and provide a panoramic picture of the protean manifestations of this infrequent disease. The heterogeneous manifestations of GPA pose a significant challenge in the diagnosis of this rare condition. By recognizing the common and unusual imaging findings, radiologists play an important role in the diagnosis and follow-up of patients with GPA and aid clinicians in the differentiation of disease activity versus disease-induced damage, which ultimately affects therapeutic decisions.

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110. classification Criteria for the Anca-Associated Vasculitides

Cited by 35 publications

References 0 publications

Eosinophils and eosinophilic immune dysfunction in health and disease

Eosinophils and eosinophilic immune dysfunction in health and disease

Епидемиология На Еозинофилната Грануломатоза С Полиангиит

From Head to Toe: Granulomatosis with Polyangiitis

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