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Eosinophilic granulomatosis with polyangiitis

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 2023), the European Expert Group on Eosinophilic Granulomatosis with Polyangiitis (EGPA-EEG 2023), the Canadian Vasculitis Research Network (CanVasc 2021), the Vasculitis Foundation (VF/ACR 2021), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021), and the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019).
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Guidelines

1.Screening and diagnosis

Diagnosis: 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

2.Classification and risk stratification

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

3.Diagnostic investigations

Antineutrophil cytoplasmic antibodies: as per EGPA-EEG 2023 guidelines, obtain ANCA testing in all patients with suspected EGPA.
B

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  • Serum immunoglobulins

  • Echocardiography

4.Diagnostic procedures

Biopsy: as per EGPA-EEG 2023 guidelines, perform a biopsy when feasible in the evaluation of patients with suspected EGPA, although it is not essential to make the diagnosis of EGPA.
B

5.Medical management

General principles
As per EULAR 2023 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

6.Therapeutic procedures

Plasma exchange
Consider performing plasma exchange in patients with serum creatinine > 5.7 mg/dL (500 mmol/l) requiring dialysis or with rapidly increasing serum creatinine, and in patients with diffuse alveolar hemorrhage having hypoxemia.
C
Perform plasma exchange in patients with an overlap syndrome of ANCA-associated vasculitis and anti-GBM.
B

7.Surgical interventions

Kidney transplantation: delay kidney transplantation until patients are in complete clinical remission for ≥ 6 months. Do not delay transplantation because of the persistence of ANCA.
B

8.Specific circumstances

Pediatric patients, evaluation: 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 renal involvement

  • Patients with sinonasal involvement

  • Patients with respiratory tract involvement (topical/inhaled therapy)

  • Patients with respiratory tract involvement (plasma exchange)

  • Patients on leukotriene inhibitors

9.Patient education

General counseling: 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

10.Preventative measures

Prophylaxis for Pneumocystis jirovecii pneumonia: as per EULAR 2023 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

11.Follow-up and surveillance

Serial clinical assessment
As per EGPA-EEG 2023 guidelines:
Use validated clinical tools in the assessment of disease activity on follow-up.
B
Obtain routine monitoring of EGPA-related manifestations, with particular reference to lung function, cardiovascular events, and neurological complications. Obtain long-term monitoring of comorbidities, such as cancer, infections, and osteoporosis.
B

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  • Serial laboratory assessment

  • Management of relapse