斯蒂尔病患者肺部受累情况的临床评估，来自多中心国际AIDA网络斯蒂尔病登记处的结果。

The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry.

作者信息

Ruscitti Piero, Vitale Antonio, Di Cola Ilenia, Caggiano Valeria, Palumbo Pierpaolo, Di Cesare Ernesto, Hinojosa-Azaola Andrea, Torres-Ruiz Jiram, Guaracha-Basañez Guillermo Arturo, Martín-Nares Eduardo, Lopalco Giuseppe, Morrone Maria, Iannone Florenzo, Giardini Henrique A Mayrink, Cordeiro Rafael Alves, de Brito Antonelli Isabele Parente, Berardicurti Onorina, Navarini Luca, Ciccia Francesco, Visconti Maria Chiara, Iacono Daniela, Direskeneli Haner, Erten Sukran, Yao Haihong, Thabet Maissa, Tharwat Samar, Ragab Gaafar, Gómez-Caverzaschi Verónica, Sfikakis Petros P, Fotis Lampros, La Torre Francesco, Maier Armin, Karamanakos Anastasios, Almaghlouth Ibrahim A, Frassi Micol, Tufan Abdurrahman, Govoni Marcello, Sota Jurgen, Simonini Gabriele, Emmi Giacomo, Li Gobbi Francesca, Parronchi Paola, Costi Stefania, Sarzi-Puttini Piercarlo, Opris-Belinski Daniela, Sfriso Paolo, Tarsia Maria, Maggio Maria Cristina, Monti Sara, Gündüz Özgül Soysal, Rigante Donato, Bartoloni Elena, Verrecchia Elena, Iagnocco Annamaria, Viapiana Ombretta, Bargagli Elena, Batu Ezgi D, Sebastiani Gian Domenico, Del Giudice Emanuela, Conti Giovanni, Breda Luciana, Gidaro Antonio, Gicchino Maria Francesca, Gaggiano Carla, Brucato Antonio Luca, Triggianese Paola, Makowska Joanna, Carubbi Francesco, Farina Nicola, Guggino Giuliana, De Paulis Amato, Mazzei Maria Antonietta, Di Meglio Nunzia, Lo Gullo Alberto, Conforti Alessandro, Ogunjimi Benson, Calabrese Laura, Rubegni Pietro, Giardina Annarita, Wiesik-Szewczyk Ewa, Balistreri Alberto, Fabiani Claudia, Frediani Bruno, Dagna Lorenzo, Giacomelli Roberto, Cantarini Luca

机构信息

Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.

Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.

出版信息

Rheumatology (Oxford). 2025 Jan 20. doi: 10.1093/rheumatology/keaf021.

DOI:10.1093/rheumatology/keaf021

PMID:39832276

Abstract

OBJECTIVES

To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.

METHODS

A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry. Still's disease-lung involvement was defined by the presence of pleuritis, parenchymal features, acute respiratory distress syndrome (ARDS), and/or pulmonary arterial hypertension.

RESULTS

In total, 90 patients with Still's disease and lung involvement were assessed (mean age 36.3 ± 17.8 years, 35.6% male sex). Among them, 13.3% of patients were paediatrics. These patients with lung involvement mainly showed pleuritis in 72.2% of cases, parenchymal features in 34.4%, ARDS in 9.5%, and pulmonary arterial hypertension in 2.3%. After that we focused on patients characterised by parenchymal lung involvement, which is an emergent issue of clinical concern. These patients with parenchymal lung disease were significantly characterized by sore throat, pericarditis, and higher values of systemic score than others. Finally, the administration of both IL-1 or IL-6 inhibitors was not associated with the presence of parenchymal lung involvement.

CONCLUSION

The clinical characteristics of patients with Still's disease and lung involvement were described in the AIDA network. We also provided a clinical profile of patients with parenchymal lung involvement considering its prognostic relevance. Although providing a clinical landscape of these patients, further studies are needed to fully clarify this issue.

摘要

目的

评估斯蒂尔病患者的肺部受累情况，斯蒂尔病是一种累及儿童和成人的炎症性疾病。探索这些患者实质性肺部受累的可能相关因素。

方法

开展一项多中心观察性研究，评估自身炎症性疾病联盟（AIDA）网络斯蒂尔病登记处纳入的、以肺部受累为特征的连续性斯蒂尔病患者。斯蒂尔病肺部受累定义为存在胸膜炎、实质性病变特征、急性呼吸窘迫综合征（ARDS）和/或肺动脉高压。

结果

共评估了90例有肺部受累的斯蒂尔病患者（平均年龄36.3±17.8岁，男性占35.6%）。其中，13.3%为儿科患者。这些肺部受累患者主要表现为胸膜炎（72.2%的病例）、实质性病变特征（34.4%）、ARDS（9.5%）和肺动脉高压（2.3%）。之后我们关注以实质性肺部受累为特征的患者，这是一个新出现的临床关注问题。这些实质性肺部疾病患者的显著特征是喉咙痛、心包炎，且全身评分高于其他患者。最后，使用白细胞介素-1或白细胞介素-6抑制剂与实质性肺部受累的存在无关。