Piero Ruscitti, Antonio Vitale, Ilenia Di Cola, Valeria Caggiano, Pierpaolo Palumbo, Ernesto Di Cesare, Andrea Hinojosa-Azaola, Jiram Torres-Ruiz, Guillermo Arturo, Guaracha-Basañez, Eduardo Martín-Nares, Giuseppe Lopalco, Maria Morrone, Florenzo Iannone, Henrique A. Mayrink Giardini, Rafael Alves Cordeiro, Isabele, Parente de Brito Antonelli, Onorina Berardicurti, Luca Navarini, Francesco Ciccia, Maria Chiara Visconti, Daniela Iacono, Haner Direskeneli, Sukran Erten, Haihong Yao, Maissa Thabet, Samar Tharwat, Gaafar Ragab, Verónica Gómez, Caverzaschi, Petros P. Sfikakis, Lampros Fotis, Francesco La Torre, Armin Maier, Anastasios Karamanakos, Ibrahim A. Almaghlouth, Micol Frassi, Abdurrahman Tufan, Marcello Govoni, Jurgen Sota, Gabriele Simonini, Giacomo Emmi, Francesca Li Gobbi, Paola Parronchi, Stefania Costi, Piercarlo Sarzi-Puttini, Daniela Opris-Belinski, Paolo Sfriso, Maria Tarsia, Maria Cristina Maggio, Sara Monti, Özgül Soysal Gündüz, Donato Rigante, Elena Bartoloni, Elena Verrecchia, Annamaria Iagnocco, Ombretta Viapiana, Elena Bargagli, Ezgi D. Batu, Gian Domenico Sebastiani, Emanuela Del Giudice, Giovanni Conti, Luciana Breda, Antonio Gidaro, Maria Francesca Gicchino, Carla Gaggiano, Antonio Luca Brucato, Paola Triggianese, Joanna Makowska, Francesco Carubbi, Nicola Farina, Giuliana Guggino, Amato De Paulis, Maria Antonietta Mazzei, Nunzia Di Meglio, Alberto Lo Gullo, Alessandro Conforti, Benson Ogunjimi, Laura Calabrese, Pietro Rubegni, Annarita Giardina, Ewa Wiesik-Szewczyk, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Lorenzo Dagna, Roberto Giacomelli, Luca Cantarin
Multiple Institutions
Italy, Mexico, Brazil, Turkey, China Tunisia, Egypt, Spain, Greece, Saudi Arabia, Australia, Romania, Poland and Belgium
Rheumatology
Rheumatology 2025;
DOI: 10.1093/rheumatology/keaf021
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.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No