Constantine Evangelia, Bartholomeo Adam M, Powers Inna, Lysinger Jerimiah L, Hauk Pia J, Abbott Jordan, de Keyser Heather H, Gilman Nicholas J, Galambos Csaba, Toland Angus, Willard Nicholas, Weinman Jason, Mong David, Lin Clara, Bloom Jessica L
School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Department of Pediatrics, Section of Rheumatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Pediatr Rheumatol Online J. 2025 Jun 2;23(1):60. doi: 10.1186/s12969-025-01115-1.
ANCA-associated vasculitis is a systemic autoimmune disease involving small- and medium-sized blood vessels. Eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg Strauss Syndrome) is the least common form in childhood with few cases reported. We present two unique pediatric cases, both of which were treated with anti-interleukin-5 therapy.
Case one is a 13-year-old male with asthma and allergies who presented with one month of cough and periorbital edema and subsequently developed submandibular swelling. Evaluation identified chronic sinusitis, weight loss, positive c-ANCA and anti-MPO IgG antibodies, peripheral blood eosinophilia, pulmonary eosinophilia, tracheal and pulmonary nodules, and eosinophilic infiltration of the submandibular salivary gland with granulomas and fibrosis fitting a diagnosis of EGPA. He improved with glucocorticoids and mepolizumab with a significant partial response, and eventually switched to benralizumab and mycophenolate mofetil with complete response. Case two presented at 19-months-old in acute respiratory distress with a history of reactive airway disease. EGPA diagnosis was confirmed on lung biopsy (eosinophilic capillaritis and interstitial expansion of eosinophils) in the setting of anti-MPO and p-ANCA positivity. He has done very well on mepolizumab for three years.
To our knowledge, this is the first reported case of submandibular salivary gland infiltrate in a child with EGPA and the youngest successfully treated patient with EGPA reported in the literature. These cases demonstrate the variation in age and disease manifestations seen in children with EGPA as well as positive responses to anti-interleukin-5 therapy. Children with EGPA may present with common or unusual complaints and require astute recognition to avoid delays in diagnosis and long-term damage.
抗中性粒细胞胞浆抗体相关性血管炎是一种累及中小血管的系统性自身免疫性疾病。嗜酸性肉芽肿性多血管炎（EGPA，既往称Churg Strauss综合征）是儿童中最不常见的类型，报道的病例较少。我们报告两例独特的儿科病例，均接受了抗白细胞介素-5治疗。
病例一是一名13岁男性，有哮喘和过敏史，出现咳嗽和眶周水肿1个月，随后出现下颌下肿胀。评估发现慢性鼻窦炎、体重减轻、c-ANCA和抗MPO IgG抗体阳性、外周血嗜酸性粒细胞增多、肺部嗜酸性粒细胞增多、气管和肺部结节，以及下颌下唾液腺嗜酸性粒细胞浸润伴肉芽肿和纤维化，符合EGPA诊断。他使用糖皮质激素和美泊利单抗治疗后病情改善，有显著部分缓解，最终改用贝那利珠单抗和霉酚酸酯，病情完全缓解。病例二19个月大，有反应性气道疾病史，因急性呼吸窘迫就诊。在抗MPO和p-ANCA阳性的情况下，经肺活检（嗜酸性毛细血管炎和嗜酸性粒细胞间质扩张）确诊为EGPA。他使用美泊利单抗治疗三年来情况良好。
据我们所知，这是首例报道的EGPA患儿下颌下唾液腺浸润病例，也是文献中报道的最年轻的成功接受EGPA治疗的患者。这些病例显示了EGPA患儿在年龄和疾病表现上的差异，以及对抗白细胞介素-治疗的阳性反应。EGPA患儿可能表现为常见或不寻常的症状，需要敏锐识别以避免诊断延误和长期损害。
