Eosinophilic granulomatosis with polyangiitis in inovative rheumatology
Authors:
Zb. Hrnčíř
Authors‘ workplace:
II. interní gastroenterologická klinika LF UK a FN, Hradec Králové
Published in:
Čes. Revmatol., 32, 2024, No. 2, p. 67-74.
Category:
Overview
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic, necrotizing, ANCA (Antineutrophil Cytoplasmic Antibodies) associated vasculitis that affects small- and medium-sized blood vessels. ANCA positive status (myeloperoxydase-ANCA > 90%) is present in ~ 40% of EGPA, and eosinophilia is essential. The main pathogenetic pathways include ANCA-mediated NETosis (Neutrophil Extracellular Traps) and IL-5-driven eosinophilic pathology. Eosinophilic asthma is obligatory in the history of EGPA patients. Antileukotriene receptor therapy of asthma is under suspicion to participate in the clinical manifestation of EGPA. Multiorgan manifestations of EGPA include variable cutaneous eruptions including palpable purpura, pulmonary infiltrates/nodulosis, peripheral neuropathy, glomerulonephritis, and cardiac involvement. A short case report of severe EGPA with fatal myocardial infarction is demonstrated. For the classification of EGPA recent ACR/EAAR (formerly EULAR) 2022 criteria are presented; this classification system has 99% specificity, but only 85% sensitivity, and repeated evaluation in time is needed in case of persistent susceptibility to EGPA. In severe EGPA a start of IV glucocorticoid (GC) „pulse therapy“ with rituximab or cyclophosphamide is needed. Nonsevere and maintenance EGPA therapy includes, especially p.o. GC, cytostatics, and anti-IL-5 biologic therapy using mepolizumab. The goal of the review is to incorporate actual knowledge of EGPA into innovative practice in clinical rheumatology.
Keywords:
ANCA – Eosinophilia – NETosis – mepolizumab – EGPA
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