From the Pediatric Allergy and Immunology Unit, Ain Shams University, Cairo, Egypt.
World Allergy Organ J. 2008 Feb;1(2):34-40. doi: 10.1097/WOX.0b013e3181626fde.
The rate of reporting of childhood Churg-Strauss syndrome (CSS) has increased lately because of either increased awareness to the disease or a real increase in incidence. It is defined as one of the antineutrophil cytoplasmic antibody-associated vasculitides, but the antineutrophil cytoplasmic antibody positivity is less reported in pediatric cases. The cause of CSS remains unknown. Several lines of evidence suggest genetic predisposition, which may entail inherited tendency to dysregulation of the cellular immune system. With the addition of leukotriene receptor antagonists to the treatment regimen of asthma, an association to CSS was presumed. However, the nature of this relationship remains to be elucidated. In addition, some environmental factors seem to provoke transient effects that resemble the disease. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. Three clinical stages have been described in the clinical evolution of CSS: prodromal phase involving allergic rhinitis and asthma (usually without family history of atopy), a second phase that involves peripheral eosinophilia and eosinophilic tissue infiltration, and the hallmark of the final phase is systemic vasculitis. Pulmonary disease is a central feature of pediatric CSS, but other manifestations include skin lesions, testicular pain, hypertension, seizures, and nephropathy. More subtle presentations in children include cervical lymphadenopathy, acute abdominal pain, deep venous thrombosis, oral ulceration, multiple colonic ulcers, chorea, bilateral optic neuropathy, and retinal artery occlusions. Churg-Strauss syndrome patients usually respond well to corticosteroid therapy. Several trials reported additional benefit from cyclophosphamide, azathioprine, and methotrexate, whereas the therapeutic effects of etanercept, plasma exchange, and intravenous immunoglobulin therapy are controversial. The relapse rate is approximately 25% to 30%, but corticosteroids have significantly increased survival, which now approaches greater than 75% at 5 years. However, there is limited information about survival or long-term outcome in childhood.
儿童变应性肉芽肿性血管炎（CSS）的报告率最近有所增加，原因可能是对该病的认识提高，也可能是发病率的真实增加。它被定义为抗中性粒细胞胞质抗体相关性血管炎之一，但儿科病例中抗中性粒细胞胞质抗体阳性的报道较少。CSS 的病因仍不清楚。有几条证据表明存在遗传易感性，这可能需要细胞免疫系统失调的遗传倾向。随着白三烯受体拮抗剂加入哮喘的治疗方案，假定与 CSS 有关。然而，这种关系的性质仍有待阐明。此外，一些环境因素似乎会引发类似于疾病的短暂影响。患者的症状由不同程度的嗜酸性粒细胞炎症和坏死性血管炎定义，可能影响任何器官。在 CSS 的临床演变中已经描述了三个临床阶段：前驱期涉及变应性鼻炎和哮喘（通常没有特应性家族史），第二期涉及外周血嗜酸性粒细胞增多和嗜酸性组织浸润，最后一期的标志是全身性血管炎。肺部疾病是儿科 CSS 的主要特征，但其他表现包括皮肤损伤、睾丸疼痛、高血压、癫痫发作和肾病。儿童中更微妙的表现包括颈淋巴结病、急性腹痛、深静脉血栓形成、口腔溃疡、多发性结肠溃疡、舞蹈病、双侧视神经病变和视网膜动脉闭塞。CSS 患者通常对皮质类固醇治疗反应良好。几项试验报告了环磷酰胺、硫唑嘌呤和甲氨蝶呤的额外益处，而依那西普、血浆置换和静脉注射免疫球蛋白治疗的疗效存在争议。复发率约为 25%至 30%，但皮质类固醇显著提高了生存率，目前在 5 年内达到 75%以上。然而，关于儿童的生存或长期预后的信息有限。
