The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still’s Disease Registry
Authors: 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 
Published: January 2025

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.