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Eosinophilic granulomatosis with polyangiitis

What's new

Updated 2024 KDIGO guidelines for the diagnosis and management of 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

Severity assessment: as per CanVasc 2021 guidelines, stratify patients with newly diagnosed or relapsing ANCA-associated vasculitides according to the extent and severity of the disease to tailor treatment accordingly.
B

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

Biopsy: as per EULAR 2024 guidelines, perform a biopsy to establish a new diagnosis of ANCA-associated vasculitis and for further evaluation of patients suspected of having relapsing vasculitis.
B

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

Kidney transplantation: as per KDIGO 2024 guidelines, delay transplantation until patients are in complete clinical remission for ≥ 6 months. Do not delay transplantation because of the persistence of ANCA.
E

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

General counseling: as per EULAR 2024 guidelines, provide patients with ANCA-associated vasculitis education focusing on the impact of ANCA-associated vasculitis and its prognosis, key warning symptoms, and treatment (including treatment-related complications).
B

Preventative measures

Prophylaxis for P. jirovecii pneumonia: as per EULAR 2024 guidelines, administer TMP/SMX for prophylaxis against P. jirovecii pneumonia and other infections in patients with ANCA-associated vasculitis receiving rituximab, cyclophosphamide, and/or high doses of corticosteroids.
B

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