Department of Pediatric Pulmonology, Necmettin Erbakan University Faculty of Medicine, Konya, Turkey.
Department of Pediatric Allergy and Immunology, Necmettin Erbakan University Faculty of Medicine, Konya, Turkey.
Pediatr Pulmonol. 2023 Oct;58(10):2769-2776. doi: 10.1002/ppul.26577. Epub 2023 Jul 20.
Bronchiolitis obliterans is characterized by partial or total occlusion of the bronchioles due to inflammation and fibrosis, and the most common form is postinfectious bronchiolitis obliterans (PIBO). This study aimed to retrospectively present our intravenous immunoglobulin (IVIG) treatment experience in PIBO patients with a clinically severe course despite receiving commonly used treatment protocols.
The study included patients aged 0-18 with subtle immunological abnormalities who were followed up in our center for PIBO between 2010 and 2021. Clinical evaluation, body mass index (BMI), computerized tomography (CT) image scoring, and immunological parameters were recorded before and after IVIG treatment.
Of the 11 patients included in the study, 90% were male, the mean age at diagnosis was 27.1 months (range: 5-68 months) and the mean current age was 81.4 months (range: 15-188 months). The number of hospital visits due to infection and the frequency of hospitalizations decreased markedly in the patients who underwent IVIG therapy. Oxygen therapy was discontinued in all patients, and improvements in radiological severity scores were observed. BMI z-scores improved over the baseline values after IVIG therapy.
Corticosteroids are considered the best first-line treatment to control inflammation in PIBO. In our study group, PIBO patients showed favorable clinical and radiological responses to regular IVIG treatment, possibly due to minor immune deficiency secondary to steroids or as a result of undetected adaptive and innate immune defects involved in the etiology of severe PIBO.
闭塞性细支气管炎是由于炎症和纤维化导致细支气管部分或完全闭塞的疾病，最常见的形式是感染后闭塞性细支气管炎（PIBO）。本研究旨在回顾性介绍我们在静脉注射免疫球蛋白（IVIG）治疗 PIBO 患者方面的经验，这些患者尽管接受了常用的治疗方案，但仍有临床严重的病程。
本研究纳入了 2010 年至 2021 年期间在我们中心因 PIBO 而接受随访的、存在细微免疫异常的 0-18 岁患者。在接受 IVIG 治疗前后，记录了临床评估、体重指数（BMI）、计算机断层扫描（CT）图像评分和免疫参数。
在纳入研究的 11 名患者中，90%为男性，诊断时的平均年龄为 27.1 个月（范围：5-68 个月），当前的平均年龄为 81.4 个月（范围：15-188 个月）。接受 IVIG 治疗的患者因感染而就诊的次数和住院频率明显减少。所有患者均停止吸氧治疗，且影像学严重程度评分有所改善。接受 IVIG 治疗后，BMI z 评分较基线值有所改善。
皮质类固醇被认为是控制 PIBO 炎症的最佳一线治疗药物。在我们的研究组中，PIBO 患者对常规 IVIG 治疗表现出良好的临床和影像学反应，这可能是由于类固醇引起的轻微免疫缺陷，或是由于涉及严重 PIBO 病因的适应性和固有免疫缺陷未被发现。
