[Multidisciplinary expert consensus on diagnosis and treatment of eosinophilic granulomatosis with polyangiitis (2025 Edition)].

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), a rare autoimmune disease affecting multiple systems, is prone to misdiagnosis and missed diagnosis due to its diverse and complex clinical manifestations, which affect the prognosis. Therefore, early diagnosis of the disease, development of standardized treatment protocols, management of complications, and multidisciplinary team collaboration are crucial. The writing group of the Multidisciplinary Expert Consensus on the Diagnosis and Treatment of Eosinophilic Granulomatosis with Polyangiitis, has comprehensively updated and revised the 2018 edition of the "Multidisciplinary Expert Consensus on the Diagnosis and Treatment of Eosinophilic Granulomatosis with Polyangiitis" based on the latest research findings. The revision adds etiology and pathogenesis, updates diagnosis and assessment, treatment, and prognosis, and compiles 13 recommendations. This revision aims to improve the diagnostic and therapeutic capabilities of clinicians for EGPA, highlight the importance of multidisciplinary collaboration in EGPA management, provide the most up-to-date guidance for clinical practice, and consequently improve treatment outcomes and patients' quality of life.The recommendations are listed below.Recommendation 1: Patients with asthma, peripheral blood eosinophilia, and damage to other systems should be suspected of having EGPA (1, B).Recommendation 2: Patients suspected of having EGPA should undergo a full examination and assessment. ANCA testing should be performed in all such patients. If feasible, biopsy of the affected tissue is also recommended (1, B).Recommendation 3: The diagnosis of EGPA should be based on highly suggestive indicative clinical features, laboratory tests, imaging, and objective evidence of vasculitis. Differential diagnosis and multidisciplinary assessment are essential. The 1990 ACR or 2022 ACR/EULAR classification criteria for EGPA are recommended for diagnosis (1, B).Recommendation 4: All patients with EGPA should be assessed for disease severity and status (1, A).Recommendation 5: The treatment goal for EGPA is to achieve disease remission promptly, maintain long-term organ function, prevent disease progression, enhance quality of life for patients, and increase survival rates (1, D).Recommendation 6: Treatment of EGPA should be based on disease severity (severe or non-severe) and status (active, remission, new-onset, responsive, refractory, relapsed) (1, B).Recommendation 7: For induction of remission in patients with active severe EGPA, corticosteroid pulse therapy or corticosteroids in combination with cyclophosphamide or rituximab are recommend (1, B).Recommendation 8: For induction of remission in patients with active non-severe EGPA, the preferred treatment is corticosteroids with mepolizumab. Other options include corticosteroids with methotrexate, azathioprine, or mycophenolate mofetil (1, A).Recommendation 9: To maintain remission in patients with severe EGPA, it is suggested to use corticosteroids in combination with rituximab, methotrexate, azathioprine, or mepolizumab. Corticosteroids should be gradually reduced to the minimum effective dose according to the patient's condition (1, B).Recommendation 10: For the maintenance of remission in patients with non-severe EGPA, it is recommended to continue with the original targeted therapy or immunosuppressants, while gradually reducing corticosteroids to the minimum effective dose (1, B).Recommendation 11: For induction of remission in patients with severe relapsing EGPA, it is recommended to use corticosteroids combined with rituximab or cyclophosphamide (2, C).Recommendation 12: For non-severe relapsing EGPA, it is important to distinguish between systemic and respiratory symptom relapses. In patients receiving methotrexate, azathioprine, mycophenolate mofetil, or low-dose corticosteroids, non-severe systemic relapses should be treated by increasing corticosteroids and/or using mepolizumab (2, B).Recommendation 13: The prodromal stage of EGPA may only present with respiratory symptoms. Close follow-up is needed, and referral to a tertiary hospital with expertise is recommended when necessary. Intervention as for non-severe EGPA may be considered to use in these patients (1, B).