Piero Ruscitti 1, Antonio Vitale 2 3, Ilenia Di Cola 1, Valeria Caggiano 2 3, Pierpaolo Palumbo 4, Ernesto Di Cesare 1, Andrea Hinojosa-Azaola 5, Jiram Torres-Ruiz 5, Guillermo Arturo Guaracha-Basañez 5, Eduardo Martín-Nares 5, Giuseppe Lopalco 6, Maria Morrone 6, Florenzo Iannone 6, Henrique A Mayrink Giardini 7, Rafael Alves Cordeiro 7, Isabele Parente de Brito Antonelli 7, Onorina Berardicurti 8 9, Luca Navarini 8 9, Francesco Ciccia 10, Maria Chiara Visconti 10, Daniela Iacono 10, Haner Direskeneli 11, Sukran Erten 12, Haihong Yao 13, Maissa Thabet 14, Samar Tharwat 15 16, Gaafar Ragab 17 18, Verónica Gómez-Caverzaschi 19, Petros P Sfikakis 20, Lampros Fotis 21, Francesco La Torre 22, Armin Maier 23, Anastasios Karamanakos 24, Ibrahim A Almaghlouth 25 26, Micol Frassi 27, Abdurrahman Tufan 28, Marcello Govoni 29, Jurgen Sota 2 3, Gabriele Simonini 30, Giacomo Emmi 31 32, Francesca Li Gobbi 33, Paola Parronchi 34, Stefania Costi 35, Piercarlo Sarzi-Puttini 36, Daniela Opris-Belinski 37, Paolo Sfriso 38, Maria Tarsia 2 3, Maria Cristina Maggio 39, Sara Monti 40 41, Özgül Soysal Gündüz 42, Donato Rigante 43, Elena Bartoloni 44, Elena Verrecchia 45 46, Annamaria Iagnocco 47, Ombretta Viapiana 48, Elena Bargagli 49 3, Ezgi D Batu 50, Gian Domenico Sebastiani 51, Emanuela Del Giudice 52, Giovanni Conti 53, Luciana Breda 54, Antonio Gidaro 55, Maria Francesca Gicchino 56, Carla Gaggiano 2 3, Antonio Luca Brucato 57, Paola Triggianese 58, Joanna Makowska 59, Francesco Carubbi 60, Nicola Farina 61 62, Giuliana Guggino 63, Amato De Paulis 64, Maria Antonietta Mazzei 65 3, Nunzia Di Meglio 65 3, Alberto Lo Gullo 66, Alessandro Conforti 67, Benson Ogunjimi 68 69 70 71, Laura Calabrese 72 73 3, Pietro Rubegni 72 73 3, Annarita Giardina 74, Ewa Wiesik-Szewczyk 75, Alberto Balistreri 76 3, Claudia Fabiani 77 3, Bruno Frediani 2 3, Lorenzo Dagna 61 62, Roberto Giacomelli 8 9, Luca Cantarini 2 3
Multiple Institutions
Multiple Countries
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
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