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

2025; Oxford University Press; Linguagem: Inglês

10.1093/rheumatology/keaf021

1462-0332

Piero Ruscitti, Antonio Vitale, Ilenia Di Cola, Valeria Caggiano, Pierpaolo Palumbo, Ernesto Di Cesare, Andrea Hinojosa‐Azaola, Jiram Torres‐Ruiz, Guillermo Arturo Guaracha-Basáñez, Eduardo Martín‐Nares, Giuseppe Lopalco, Maria Concetta Morrone, Florenzo Iannone, Henrique Ayres Mayrink Giardini, Rafael Alves Cordeiro, Isabele Parente de Brito Antonelli, Onorina Berardicurti, Luca Navarini, Francesco Ciccia, M. C. Visconti, Daniela Iacono, Haner Di̇reskeneli̇, Şükran Erten, Haihong Yao, M. Thabet, Samar Tharwat, Gaafar Ragab, Verónica Gómez‐Caverzaschi, Petros P. Sfikakis, Lampros Fotis, Francesco La Torre, Armin Maier, Αναστάσιος Καραμανάκος, Ibrahim Almaghlouth, Micol Frassi, Abdurrahman Tufan, Marcello Govoni, Jurgen Sota, Gabriele Simonini, Giacomo Emmi, Francesca Li Gobbi, Paola Parronchi, Stefania Costi, Piercarlo Sarzi‐Puttini, Daniela Opriş-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 Deniz Batu, Gian Domenico Sebastiani, Emanuela Del Giudice, Giovanni Conti, Laura Breda, Antonio Gidaro, Maria Francesca Gicchino, Carla Gaggiano, Antonio 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, Anna Giardina, Ewa Więsik‐Szewczyk, Alberto Balistreri, Claudia Fabiani, Bruno Frediani, Lorenzo Dagna, Roberto Giacomelli, Luca Cantarini,

Inflammasome and immune disorders

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.