Department of Pediatrics, Center for Pediatric Research Leipzig (CPL), University of Leipzig Medical Center, Leipzig, Germany.
Hauner Children's Hospital and KUBUS Research Center, University of Munich, Munich, Germany.
Pediatr Pulmonol. 2020 Apr;55(4):909-917. doi: 10.1002/ppul.24680. Epub 2020 Feb 10.
Pediatric lymphocytic interstitial pneumonia (LIP) and follicular bronchiolitis (FB) are poorly characterized lymphoproliferative disorders. We present and quantify demographics, radiological and histopathologic patterns, treatments and their responses, and outcomes in non-HIV-infected children with LIP and FB.
This structured registry-based study included a retrospective chart review, blinded analysis of imaging studies and lung biopsies, genetic testing, and evaluation of treatments and outcomes.
Of the 13 patients (eight females) studied, eight had FB, four had combined LIP/FB, and one had isolated LIP; diagnoses were highly concordant between the pathologists. Most patients became symptomatic during the first 2 years of life, with a mean lag time to diagnosis of 4 years. The most common symptoms were coughing and respiratory infections (11 out of 13 each), dyspnea (10 out of 13), and wheezing (eight out of 13). Autoantibodies were found in eight out of 13 patients. In three patients, disease-causing mutations in the COPA gene were identified. CT revealed hilar lymphadenopathy (five out of 12), ground-glass opacity (eight out of 12), consolidation (five out of 12), and cysts (four out of 13). Systemic steroids as intravenous pulses (11 out of 13) or oral intake (10 out of 13) were the main treatments and showed high response rates of 100% and 90%, respectively. Within the mean observation period of 68 months, all children had chronic courses, eight out of 13 had severe diseases, two died, and one worsened.
Children with LIP/FB have chronic diseases that occurred in early childhood and were commonly associated with immune dysregulation as well as high morbidity and mortality. Early diagnosis and treatment may be crucial to improve the outcome.
小儿淋巴细胞性间质性肺炎（LIP）和滤泡性细支气管炎（FB）是特征不明显的淋巴增生性疾病。我们介绍并量化了非 HIV 感染儿童中 LIP 和 FB 的人口统计学、影像学和组织病理学模式、治疗及其反应和结局。
这项基于结构化注册的研究包括回顾性病历分析、影像学研究和肺活检的盲法分析、基因检测，以及对治疗和结局的评估。
在研究的 13 名患者（8 名女性）中，8 名患有 FB，4 名患有 LIP/FB 合并症，1 名患有孤立性 LIP；病理学家的诊断高度一致。大多数患者在生命的头 2 年内出现症状，平均诊断延迟时间为 4 年。最常见的症状是咳嗽和呼吸道感染（各 11 例）、呼吸困难（各 10 例）和喘息（各 8 例）。13 例患者中有 8 例发现自身抗体。在 3 例患者中，确定了 COPA 基因的致病突变。CT 显示肺门淋巴结肿大（12 例中有 5 例）、磨玻璃影（12 例中有 8 例）、实变（12 例中有 5 例）和囊肿（13 例中有 4 例）。静脉注射脉冲（13 例中有 11 例）或口服（13 例中有 10 例）全身类固醇是主要治疗方法，分别显示出 100%和 90%的高反应率。在平均 68 个月的观察期内，所有儿童都有慢性病程，13 例中有 8 例病情严重，2 例死亡，1 例恶化。
患有 LIP/FB 的儿童患有在幼儿期发生的慢性疾病，通常与免疫失调以及高发病率和死亡率相关。早期诊断和治疗可能对改善结局至关重要。
