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- Vrsta/tip rada: Pregledni (revijski) članak
- PREGLEDAJTE RAD
- PREUZMITE RAD
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Abstract
Introduction. Eosinophilic granulomatosis with polyangiitis is a rare, systemic, immune-mediated vasculitis of small- to medium sized blood vessels, characterised by eosinophil-rich inflammatory response, necrotising vasculitis, and granulomatous tissue reactions.
Pathogenesis. The disease arises from a complex inter play of T helper 2- and T helper 1 cells mediated immunity, eosinophil activation, B-cell-autoantibody responses, and epithelial derived alarmins. Anti-neutrophil cytoplasmic antibodies positive (predominantly myeloperoxidase) patients exhibit a vasculitic phenotype, whereas anti-neutrophil cytoplasmic antibodies-negative patients show eosinophil-driven organ involvement. Key laboratory features include marked peripheral eosinophilia, elevated inflammatory markers, increased immunoglobulin E levels, and raised immunoglobulin G4 level.
Clinical Presentation. The disease evolves through overlapping prodromal, eosinophilic, and vasculitic phases, producing variable organ involvement and a relapsing-remitting course. Heterogeneous presentation may obscure diagnosis and affect prognosis.
Diagnosis and Differential Diagnosis. Diagnosis integrates clinical features, laboratory findings, and histopathologicy analysis. Differential diagnoses include other anti-neutrophil cytoplasmic antibody-associated vasculitides, hypereosinophilic syndromes, eosinophilic lung disorders, drug-induced reactions, parasitic infections, and immunoglobulin G4-related disease.
Prognosis. Despite limited evidence, overall survival is generally favourable.
Therapy. Management is guided by severity and prognostic factors. Glucocorticoids remain foundational, while biologics targeting interleukin-5, interleukin-4/ interleukin-13, anti-immunoglobulin E – as well as emerging approaches including B-cell depletion, alarmin inhibition, C5a receptor antagonism, and cellular therapies provide steroid-sparing options and improved disease control.
Conclusion. Despite favorable survival, morbidity persists due to disease relapses, organ specific complications, and long-term immunosuppression. Advances in immunogenetics, molecular profiling, and targeted therapies support a precision–medicine approach, multidisciplinary care and individualised management to optimise patient outcomes in eosinophilic granulomatosis with polyangiitis.