Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

被引:67
作者
Nguyen, Yann [1 ]
Guillevin, Loic [1 ]
机构
[1] Univ Paris 05, Hop Cochin, Referral Ctr Rare System & Autoimmune Dis, Dept Internal Med, Paris, France
关键词
eosinophilic granulomatosis with polyangiitis; eosinophilia; systemic necrotizing vasculitis; ANCA-ASSOCIATED VASCULITIS; TERM-FOLLOW-UP; POOR-PROGNOSIS FACTORS; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; SYSTEMIC-NECROTIZING-VASCULITIDES; DAILY ORAL CYCLOPHOSPHAMIDE; POLYARTERITIS-NODOSA; ALLERGIC GRANULOMATOSIS; VESSEL VASCULITIS; INTRAVENOUS IMMUNOGLOBULIN;
D O I
10.1055/s-0038-1669454
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a systemic necrotizing vasculitis of small-and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses. Overall survival has improved markedly since the use of glucocorticoids and immunosuppressants, but relapse rates remain high. All patients require glucocorticoids, and for those with severe/refractory disease and FFS-defined poor prognoses, immunosuppressants should be used (cyclophosphamide for induction and azathioprine for maintenance therapy). Recent advances in EGPA management, including several novel immunomodulatory drugs and targeted biotherapies, were or are being evaluated to improve EGPA patients' prognoses.
引用
收藏
页码:471 / 481
页数:11
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