[Eosinophilic granulomatosis with polyangiitis : Update on classification and management]

Area of Science:

• Rheumatology
• Immunology
• Pulmonology

Background:

• Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, chronic inflammatory systemic disease linked to bronchial asthma and eosinophilia.
• Vasculitis affecting small to medium-sized vessels is a hallmark, though not always present initially.
• Antineutrophil cytoplasmic antibodies (ANCA) are found in about one-third of patients, distinguishing genetically distinct EGPA subtypes.

Purpose of the Study:

• To summarize the current understanding of Eosinophilic granulomatosis with polyangiitis (EGPA).
• To highlight the distinct characteristics of ANCA-positive and ANCA-negative EGPA.
• To review current and emerging treatment strategies for EGPA.

Main Methods:

• Literature review of Eosinophilic granulomatosis with polyangiitis (EGPA) pathogenesis, clinical features, and treatment.
• Analysis of genetic distinctions between ANCA-positive and ANCA-negative EGPA.
• Evaluation of treatment outcomes with conventional therapies and biologics.

Main Results:

• Cardiac involvement in EGPA signifies a poor prognosis.
• Permanent organ damage can result from EGPA or its treatment, particularly glucocorticoids.
• Mepolizumab, an IL-5 antagonist, effectively reduces relapses and glucocorticoid requirements in specific EGPA populations.
• Rituximab is a potential option for severe ANCA-positive EGPA recurrence, despite limited formal evidence.

Conclusions:

• EGPA encompasses distinct ANCA-positive and ANCA-negative forms with varying clinical frequencies.
• Standard EGPA treatment involves glucocorticoids and immunosuppressants, with biologics gaining prominence.
• Targeted therapies like mepolizumab and rituximab show promise in managing EGPA, particularly in relapsing or severe cases.