Brunetto Silvia, Buta Federica, Gangemi Sebastiano, Ricciardi Luisa
Department of Clinical and Experimental Medicine, School and Unit of Allergy and Clinical Immunology, "G. Martino" Hospital, University of Messina, 98124 Messina, Italy.
Int J Mol Sci. 2025 Aug 2;26(15):7472. doi: 10.3390/ijms26157472.
Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic vasculitis with eosinophilic inflammation and variable clinical presentations. Although skin manifestations are frequent, current classification criteria do not include them, which may underestimate their diagnostic value. This prospective observational study aimed to assess systemic and skin involvement as well as eosinophilia, anti-neutrophil cytoplasmic antibody (ANCA), and Anti-nuclear antibodies (ANA) serum levels in 20 EGPA patients followed for one year at the University Hospital of Messina, Italy, before starting Mepolizumab, 300 mg. Eosinophilia, ANCA status, systemic and skin involvement were also evaluated at 6 and 12 months; a literature review on these data supplements our findings. Skin involvement was present in 55% of patients, including purpura, urticarial vasculitis, angioedema, maculopapular rash, and nodules, mostly in ANCA-negative patients, though purpura was more frequent in ANCA-positive cases but without any statistically significant correlation. ANAs were present in 50% of patients, together with ANCA in two subjects and without in eight. Mepolizumab significantly reduced eosinophil levels, BVASs, and corticosteroid dependence, with notable improvement in skin symptoms. In conclusion, skin manifestations are common in EGPA and may represent useful indicators of disease activity. Their integration with ANCA status, eosinophil counts, and positivity to other autoantibodies could enhance diagnostic and monitoring strategies identifying different clusters of EGPA patients even if the small sample size limits the generalizability of the findings.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的系统性血管炎，伴有嗜酸性粒细胞炎症和多种临床表现。虽然皮肤表现很常见，但目前的分类标准并未将其纳入，这可能会低估它们的诊断价值。这项前瞻性观察性研究旨在评估意大利墨西拿大学医院对20例EGPA患者在开始使用300mg美泊利珠单抗治疗前，随访一年期间的全身和皮肤受累情况，以及嗜酸性粒细胞增多、抗中性粒细胞胞浆抗体（ANCA）和抗核抗体（ANA）的血清水平。在6个月和12个月时也评估了嗜酸性粒细胞增多、ANCA状态、全身和皮肤受累情况；对这些数据的文献综述补充了我们的研究结果。55%的患者出现皮肤受累，包括紫癜、荨麻疹性血管炎、血管性水肿、斑丘疹和结节，大多见于ANCA阴性患者，不过紫癜在ANCA阳性病例中更常见，但无任何统计学显著相关性。50%的患者存在ANA，其中2例同时伴有ANCA，8例不伴有ANCA。美泊利珠单抗显著降低了嗜酸性粒细胞水平、BVAS评分和对皮质类固醇的依赖性，皮肤症状有明显改善。总之，皮肤表现在EGPA中很常见，可能是疾病活动的有用指标。即使样本量较小限制了研究结果的普遍性，但将它们与ANCA状态、嗜酸性粒细胞计数以及对其他自身抗体的阳性反应相结合，可能会增强诊断和监测策略，从而识别不同类型的EGPA患者。
