Saenz Rios Florentino, Devaraj Sandhya, Movva Giri, Movva Hari, Nguyen Quan D
Radiology, University of Texas Medical Branch, Galveston, USA.
Family Medicine, The University of Texas Health Science Center, Tyler, USA.
Cureus. 2020 Dec 13;12(12):e12055. doi: 10.7759/cureus.12055.
Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has not been described in granulomatosis with polyangiitis (GPA) in a pediatric patient. We present a biopsy case that confirmed granulomatosis with polyangiitis with significant eosinophilia > 30% on the initial presentation. Etiologies that could account for eosinophilia were excluded during workup. The patient's presentation of pulmonary alveolar hemorrhage, conjunctivitis arthritis, high-titer cytoplasmic antineutrophil, PR3-ANCA antibodies, and cytoplasmic antibodies (cANCA) was consistent with a clinical picture of atypical GPA in a pediatric patient. This case presents a rare opportunity not only due to GPA's low incidence in the pediatric population but due to the unusual nature of significant eosinophilia in GPA.
显著嗜酸性粒细胞增多是变应性肉芽肿性血管炎（Churg-Strauss综合征）的一个突出特征，但在儿童肉芽肿性多血管炎（GPA）患者中尚未见报道。我们报告1例活检病例，该病例确诊为肉芽肿性多血管炎，初诊时嗜酸性粒细胞显著增多>30%。在检查过程中排除了可能导致嗜酸性粒细胞增多的病因。该患者出现肺泡出血、结膜炎关节炎、高滴度胞浆抗中性粒细胞胞浆抗体（PR3-ANCA）和胞浆抗体（cANCA），符合儿童非典型GPA的临床表现。该病例不仅因GPA在儿童人群中发病率低，还因其嗜酸性粒细胞显著增多的不寻常性质，提供了一个难得的机会。
