Kvedaraite Egle, Lourda Magda, Han HongYa, Tesi Bianca, Mitchell Jenée, Ideström Maja, Mouratidou Natalia, Rassidakis George, von Bahr Greenwood Tatiana, Cohen-Aubart Fleur, Jädersten Martin, Åkefeldt Selma Olsson, Svensson Mattias, Kannourakis George, Bryceson Yenan T, Haroche Julien, Henter Jan-Inge
Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
Center for Infectious Medicine, Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden.
Acta Paediatr. 2021 Apr;110(4):1315-1321. doi: 10.1111/apa.15590. Epub 2020 Oct 8.
To present the first case series of patients with Langerhans cell histiocytosis (LCH) also affected by Crohn's disease (CD), both of which are granulomatous diseases, and in LCH investigate the role of interleukin (IL)-23, which is a well-described disease mediator in CD.
A case series of three patients with LCH and CD were described; a cohort of LCH patients (n = 55) as well as controls (n = 55) were analysed for circulating IL-23 levels; and the relation between the percentage of LCH cells in lesions and circulating IL-23 levels was analysed in seven LCH patients.
Differential diagnostic challenges for these two granulomatous diseases were highlighted in the case series, and it took up to 3 years to diagnose CD. Elevated IL-23 levels were found in LCH patients. The amount of lesional LCH cells correlated with the levels of circulating IL-23.
Both CD and LCH should be considered in patients with inflammatory gastrointestinal involvement. The IL-23 pathway is a common immunological trait between these two granulomatous diseases.
呈现首例同时患有朗格汉斯细胞组织细胞增多症（LCH）和克罗恩病（CD）的病例系列，这两种均为肉芽肿性疾病，并且针对LCH研究白细胞介素（IL）-23的作用，IL-23是CD中一种已被充分描述的疾病介质。
描述了3例患有LCH和CD的病例系列；分析了一组LCH患者（n = 55）以及对照组（n = 55）的循环IL-23水平；并在7例LCH患者中分析了病变中LCH细胞百分比与循环IL-23水平之间的关系。
该病例系列突出了这两种肉芽肿性疾病的鉴别诊断挑战，诊断CD花费了长达3年的时间。在LCH患者中发现IL-23水平升高。病变中的LCH细胞数量与循环IL-23水平相关。
对于有炎症性胃肠道受累的患者，应同时考虑CD和LCH。IL-23通路是这两种肉芽肿性疾病之间的一个共同免疫特征。
