[Eosinophilic granulomatosis with polyangiitis: a review article]

Area of Science:

• Rheumatology
• Immunology
• Pulmonology

Background:

• Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small vessel vasculitis characterized by granulomatous inflammation, eosinophilia, and allergic symptoms like asthma and rhinosinusitis.
• Clinical manifestations vary, with eosinophilic infiltration potentially affecting the heart, lungs, gastrointestinal tract, and nervous system, impacting prognosis.
• Distinguishing EGPA from hypereosinophilic syndrome (HES) presents a clinical challenge.

Purpose of the Study:

• To summarize the key features, diagnostic considerations, and therapeutic approaches for Eosinophilic granulomatosis with polyangiitis (EGPA).
• To highlight the diverse clinical manifestations and prognostic factors associated with EGPA.
• To differentiate EGPA from other eosinophilic disorders and outline current treatment strategies.

Main Methods:

• Review of existing literature on Eosinophilic granulomatosis with polyangiitis (EGPA).
• Analysis of diagnostic criteria, including ANCA testing (pANCA, MPO-ANCA).
• Summary of current therapeutic guidelines for EGPA management.

Main Results:

• EGPA involves small and medium-sized vessels, often presenting with asthma, rhinosinusitis, and eosinophilia.
• Cardiac involvement is linked to a poorer prognosis, while MPO-ANCA positivity correlates with neurologic and renal disease.
• ANCA-negative EGPA is more associated with cardiac and pulmonary manifestations.

Conclusions:

• Effective management of EGPA requires tailored treatment based on disease severity and organ involvement.
• Glucocorticoids and cyclophosphamide are used for severe EGPA, with rituximab as an alternative.
• Mepolizumab (IL-5 blockade) is an approved treatment for EGPA without severe organ involvement.