Oiwa Hiroshi, Yahata Akihiro, Nakamoto Naoki
Department of Rheumatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.
Clin Rheumatol. 2025 Aug 18. doi: 10.1007/s10067-025-07634-2.
INTRODUCTION/OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that frequently affects small arteries in the skin and the nerve but can also involve the medium-sized arteries, resembling polyarteritis nodosa (PAN). This study aimed to evaluate the clinical value of biomarkers in EGPA in relation to the size of the affected arteries.
In an inception cohort of EGPA, we examined the relationships between eosinophil counts, myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, levels of rheumatoid factor (RF) and Immunoglobulin E (IgE) and pre-defined outcomes based on artery size. These outcomes included histologically confirmed cutaneous vasculitis, glomerulonephritis, PAN-like arteritis in the cranial and abdominal arteries, and cardiomyopathy.
IgE levels were significantly elevated in patients with cutaneous vasculitis and cardiomyopathy compared to those without the respective conditions. The median IgE values (range) were 3620 IU/mL (1160-6520) vs. 1000 IU/mL (229-5490) for cutaneous vasculitis (p = 0.0077), and 4065 IU/mL (1320-6520) vs. 1205 IU/mL (229-4030) for cardiomyopathy (p = 0.032). A numerically higher frequency of MPO-ANCA was observed in cases with PAN-like arteritis compared to those without (4/5 vs 8/25), similar to the findings for MPO-ANCA in glomerulonephritis. The crude odds ratio of MPO-ANCA positivity for both glomerulonephritis and PAN-like arteritis was 8.50 (95% CI: 1.05-181.45). The optimal IgE thresholds for detecting cutaneous vasculitis and cardiomyopathy were 1860 IU/mL and 2465 IU/mL, respectively, with areas under the curve of 0.82 and 0.84, indicating favorable diagnostic performance.
IgE levels were associated with cutaneous vasculitis and cardiomyopathy in an inception cohort of EGPA in Japan. Further studies with larger cohorts are needed to confirm these findings, as our results are based on a retrospective study with a small sample size. Key Points • IgE levels were associated with cutaneous vasculitis in EGPA. • IgE levels were associated with cardiomyopathy in EGPA. • PAN-like arteritis tended to be associated with MPO-ANCA positivity.
引言/目的：嗜酸性肉芽肿性多血管炎（EGPA）是一种坏死性血管炎，常累及皮肤和神经中的小动脉，但也可累及中动脉，类似于结节性多动脉炎（PAN）。本研究旨在评估EGPA中生物标志物与受累动脉大小相关的临床价值。
在一个EGPA起始队列中，我们研究了嗜酸性粒细胞计数、髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）阳性、类风湿因子（RF）和免疫球蛋白E（IgE）水平与基于动脉大小的预先定义结局之间的关系。这些结局包括组织学确诊的皮肤血管炎、肾小球肾炎、颅动脉和腹动脉中的PAN样动脉炎以及心肌病。
与无相应疾病的患者相比，皮肤血管炎和心肌病患者的IgE水平显著升高。皮肤血管炎患者的IgE中位数（范围）为3620 IU/mL（1160 - 6520），而无皮肤血管炎患者为1000 IU/mL（229 - 5490）（p = 0.0077）；心肌病患者为4065 IU/mL（1320 - 6520），无心肌病患者为1205 IU/mL（229 - 4030）（p = 0.032）。与无PAN样动脉炎的病例相比，PAN样动脉炎病例中MPO-ANCA的出现频率在数值上更高（4/5 vs 8/25），这与肾小球肾炎中MPO-ANCA的发现相似。肾小球肾炎和PAN样动脉炎中MPO-ANCA阳性的粗比值比为8.50（95%CI：1.05 - 181.45）。检测皮肤血管炎和心肌病的最佳IgE阈值分别为1860 IU/mL和2465 IU/mL，曲线下面积分别为0.82和0.84，表明诊断性能良好。
在日本的一个EGPA起始队列中，IgE水平与皮肤血管炎和心肌病相关。由于我们的结果基于一项小样本的回顾性研究，需要更大队列的进一步研究来证实这些发现。要点 • EGPA中IgE水平与皮肤血管炎相关。 • EGPA中IgE水平与心肌病相关。 • PAN样动脉炎倾向于与MPO-ANCA阳性相关。
