Xie Jun, Zhao Ying-Yue, Liu Jing, Nong Guang-Min
Department of Pediatrics, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China.
World J Clin Cases. 2020 Jun 26;8(12):2662-2666. doi: 10.12998/wjcc.v8.i12.2662.
Diffuse alveolar hemorrhage (DAH) is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury (mainly capillaries, including arteries and veins), causing pulmonary microcirculation blood to accumulate in the alveolar space. DAH is classified by the histological absence or presence of pulmonary capillaritis (PC) and is rarely reported in the literature.
This is a report of three girls aged 6-11 years with DAH and PC. Two patients had decreased hemoglobin and one had increased erythrocyte sedimentation rate. High-resolution computed tomography showed bilateral diffuse pulmonary infiltrate, and diagnosis of PC was confirmed by lung biopsy. Immunofluorescence test in one case showed granular IgG and a small amount of granular IgA deposit on the alveolar walls, and was negative in the other two cases, describing isolated pauci-immune PC. Treatment was with glucocorticoid alone or combination with immunosuppressants, and the symptoms resolved in all patients.
PC is classified as isolated and immune-mediated PC associated with systemic disease. It can be controlled in most children with glucocorticoid alone or combined with immunosuppressants.
弥漫性肺泡出血（DAH）是一种多病因导致的肺毛细血管出血或肺血管小血管损伤（主要是毛细血管，包括动脉和静脉），致使肺微循环血液积聚于肺泡腔隙。DAH 根据组织学上是否存在肺毛细血管炎（PC）进行分类，在文献中鲜有报道。
本文报告了 3 例年龄在 6 - 11 岁的患有 DAH 和 PC 的女孩。2 例患者血红蛋白降低，1 例红细胞沉降率升高。高分辨率计算机断层扫描显示双侧弥漫性肺部浸润，肺活检确诊为 PC。1 例患者的免疫荧光试验显示肺泡壁上有颗粒状 IgG 和少量颗粒状 IgA 沉积，另外 2 例为阴性，描述为孤立性寡免疫性 PC。治疗采用单独使用糖皮质激素或联合免疫抑制剂，所有患者症状均缓解。
PC 分为孤立性和与系统性疾病相关的免疫介导性 PC。大多数儿童单独使用糖皮质激素或联合免疫抑制剂即可控制病情。
