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Eosinophilic granulomatosis with polyangiitis
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of eosinophilic granulomatosis with polyangiitis are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2024), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2024), the European Expert Group on Eosinophilic Granulomatosis with Polyangiitis (EGPA-EEG 2023), the Canadian Vasculitis Research Network (CanVasc 2021), the Vasculitis ...
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Screening and diagnosis
Diagnosis: as per EGPA-EEG 2023 guidelines, suspect EGPA in patients with asthma, chronic rhinosinusitis, and eosinophilia developing end-organ involvement, particularly peripheral neuropathy, lung infiltrates, cardiomyopathy, or other complications (such as skin, gastrointestinal, or kidney involvement).
B
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Differential diagnosis
Classification and risk stratification
Diagnostic investigations
ANCA: as per EULAR 2024 guidelines, obtain proteinase 3-ANCA and myeloperoxidase-ANCA testing, with a high-quality antigen-specific assay as the primary method of testing, in patients with signs and/or symptoms raising suspicion of a diagnosis of ANCA-associated vasculitis.
A
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Serum immunoglobulins
Echocardiography
Diagnostic procedures
Medical management
General principles
As per EULAR 2024 guidelines:
Offer multidisciplinary management in centers with, or with ready access to, expertise in vasculitis in patients with ANCA-associated vasculitis.
B
Offer the best care based on shared decision-making between the patient and the physician, taking efficacy, safety, and costs into account, in patients with ANCA-associated vasculitis.
B
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Induction of remission (general principles)
Induction of remission (non-severe)
Induction of remission (severe)
Maintenance of remission
Management of refractory disease
Induction of remission (avacopan)
Therapeutic procedures
Plasma exchange
As per KDIGO 2024 guidelines:
Consider performing plasma exchange in patients with serum creatinine > 3.4 mg/dL (> 300 mcmol/L), rapidly increasing serum creatinine, or requiring dialysis, and in patients with diffuse alveolar hemorrhage having hypoxemia.
E
Perform plasma exchange in patients with an overlap syndrome of ANCA-associated vasculitis and anti-GBM.
E
Surgical interventions
Specific circumstances
Pediatric patients, evaluation: as per SHARE initiative 2019 guidelines, use the Chapel Hill definition (2012) when referring to EGPA in the pediatric population, recognizing that there are no pediatric diagnostic or classification criteria for EGPA.
B
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Pediatric patients (treatment)
Pregnant patients
Patients with sinonasal involvement
Patients with respiratory tract involvement (topical/inhaled therapy)
Patients with respiratory tract involvement (plasma exchange)
Patients on leukotriene inhibitors
Patient education
Preventative measures
Follow-up and surveillance
Clinical follow-up
As per EULAR 2024 guidelines:
Obtain periodic assessment for treatment-related adverse effects and comorbidities in patients with ANCA-associated vasculitis. Offer prophylaxis and lifestyle advice to reduce treatment-related complications and other comorbidities.
B
Obtain structured clinical assessment, rather than ANCA and/or CD19-positive B cell testing alone, to inform decisions on changes in treatment in patients with ANCA-associated vasculitis.
B
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Laboratory follow-up
Management of refractory disease
Management of relapse