Pulmonary arterial hypertension in adults with Still's disease: another pulmonary manifestation associated with HLA-DRB1*15.

IF 20.3 1区 医学 Q1 RHEUMATOLOGY
Athénaïs Boucly, Stéphane Mitrovic, Maryvonnick Carmagnat, Laurent Savale, Xavier Jaïs, Jean-Luc Taupin, Estibaliz Lazaro, Emilie Berthoux, Nicolas Schleinitz, Maria-Rosa Ghigna, Joanna Kedra, Xavier Mariette, Céline Roussin, Pierre-Antoine Juge, Marc Humbert, Olivier Sitbon, David Montani, Bruno Fautrel
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引用次数: 0

Abstract

Objectives: Inflammatory lung disease in Still's disease (SD) has recently been described. Among its manifestations, pulmonary arterial hypertension (PAH) is a rare and life-threatening event, with only a few case reports published. The objective was to report the largest adult cohort of PAH occurring in the context of SD.

Methods: We identified 16 adult SD patients with PAH (PAH+) by a call for observations from the CRI-IMIDIATE (Club Rhumatismes & Inflammation - Immune-Mediated Inflammatory Disease Alliance for Translational and Clinical Research) network (https://cri-net.com/recherche/reseau-CRI-Imidiate/) and a search of the French pulmonary hypertension network registry. Patient characteristics and disease evolution were retrospectively compared with those of 111 SD controls without PAH (PAH-) followed in a reference centre.

Results: The profile of patients in the PAH+ and PAH- groups differed: 100% versus 69.4% female (P = .006), 75% versus 17.1% Black (P < .0001), more active SD both at diagnosis and throughout the disease course, and more likely to present macrophage activation syndrome (62.5% vs 14.4%, P < .0001) and exhibit eosinophilia during the disease course (68.7% vs 7.2%, P < .0001). For the 84 out of 127 patients with genetic typing, the HLA-DRB1*15 allele was more prevalent in PAH+ than PAH- patients (8/11 [72.7%] vs 22/73 [30.1%], P = .014). PAH+ patients more frequently received canakinumab and immunosuppressants than did PAH- patients and had higher frequency of drug reactions to interleukin 1 (IL-1) and/or IL-6 inhibitors (37.5% vs 7.2%, P = .002). Mortality was higher in PAH+ than PAH- patients (37.5% vs 0.9%, P < .0001), and all deaths were related to SD flare.

Conclusions: These results reinforce the association between the HLA-DRB1*15 allele and severe forms of SD and raise the question of therapeutic optimisation in such patients.

成人斯蒂尔氏病肺动脉高压:与HLA-DRB1*相关的另一种肺部表现
目的:炎症性肺疾病在斯蒂尔氏病(SD)最近被描述。肺动脉高压(pulmonary arterial hypertension, PAH)是一种罕见且危及生命的疾病,目前仅有少数病例报道。目的是报道在SD背景下发生的多环芳烃的最大成人队列。方法:我们通过CRI-IMIDIATE (Club Rhumatismes & Inflammation - immunemediated Inflammatory Disease Alliance for Translational and Clinical Research)网络(https://cri-net.com/recherche/reseau-CRI-Imidiate/)和法国肺动脉高压网络注册表的搜索,确定了16名成年SD PAH (PAH+)患者。回顾性比较参考中心随访的111例无PAH (PAH-) SD对照的患者特征和疾病演变情况。结果:PAH+组和PAH-组患者的情况不同:100%对69.4%的女性(P = 0.006), 75%对17.1%的黑人(P < 0.0001),在诊断时和整个病程中都有更活跃的SD,更有可能出现巨噬细胞激活综合征(62.5%对14.4%,P < 0.0001)和在病程中表现出嗜酸性粒细胞增多(68.7%对7.2%,P < 0.0001)。127例遗传分型患者中的84例中,HLA-DRB1*15等位基因在PAH+患者中比在PAH-患者中更普遍(8/11[72.7%]比22/73 [30.1%],P = 0.014)。PAH+患者比PAH-患者更频繁地接受canakinumab和免疫抑制剂治疗,并且对白细胞介素1 (IL-1)和/或IL-6抑制剂的药物反应频率更高(37.5% vs 7.2%, P = 0.002)。PAH+患者的死亡率高于PAH-患者(37.5% vs 0.9%, P < 0.0001),所有死亡均与SD发作有关。结论:这些结果强化了HLA-DRB1*15等位基因与严重SD之间的关联,并提出了对这类患者进行优化治疗的问题。
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来源期刊
Annals of the Rheumatic Diseases
Annals of the Rheumatic Diseases 医学-风湿病学
CiteScore
35.00
自引率
9.90%
发文量
3728
审稿时长
1.4 months
期刊介绍: Annals of the Rheumatic Diseases (ARD) is an international peer-reviewed journal covering all aspects of rheumatology, which includes the full spectrum of musculoskeletal conditions, arthritic disease, and connective tissue disorders. ARD publishes basic, clinical, and translational scientific research, including the most important recommendations for the management of various conditions.
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