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

Piero Ruscitti 1, 2
Antonio Vitale 3, 4, 5, 6
Ilenia Di Cola 2
Valeria Caggiano 3, 4, 5, 6
Ernesto Di Cesare 1, 2
Andrea Hinojosa-Azaola 8
Jiram Torres-Ruiz 8, 9
Guillermo Arturo Guaracha-Basañez 9
Guillermo A. Guaracha-Basáñez 8
Eduardo Martín-Nares 8
Giuseppe Lopalco 10, 11
Maria Morrone 10, 11
Henrique A Mayrink Giardini 12
Henrique Ayres Mayrink Giardini 13
Rafael Alves Cordeiro 13
Isabele Parente de Brito Antonelli 12, 13
Onorina Berardicurti 14, 15
Luca Navarini 14, 15, 16, 17
Francesco Ciccia 18
Maria Chiara Visconti 18, 19
Daniela Iacono 18, 19
Haner Direskeneli 20, 21
Sukran Erten 22, 23
Haihong Yao 24, 25, 26
Maissa Thabet 27, 28
Samar Tharwat 29, 30, 31, 32
Gaafar Ragab 33, 34
Verónica Gómez-Caverzaschi 35, 36
Petros P. Sfikakis 37
P. Sfikakis 38
Lampros Fotis 39
Francesco La Torre 40, 41
Armin Maier 42, 43
Anastasios Karamanakos 44, 45
Ibrahim A Almaghlouth 46, 47
Micol Frassi 48, 49
Abdurrahman Tufan 50
Marcello Govoni 51, 52
Jurgen Sota 3, 4, 5, 6
Gabriele Simonini 53, 54
Giacomo Emmi 55, 56, 57, 58, 59
Francesca Li Gobbi 60, 61
Paola Parronchi 62
Stefania Costi 63, 64
Piercarlo Sarzi-Puttini 65, 66
Daniela Opris-Belinski 67, 68
Paolo Sfriso 69, 70
Maria Tarsia 3, 4, 5, 6
Maria Cristina Maggio 71, 72
Sara Monti 73, 74, 75, 76
Özgül Soysal Gündüz 77, 78
Donato Rigante 79, 80, 81
Elena Verrecchia 83, 84
Annamaria Iagnocco 85, 86
Ombretta Viapiana 87, 88
Elena Bargagli 4, 6, 89, 90
Ezgi D Batu 91
Ezgi Deniz Batu 92
GIAN DOMENICO SEBASTIANI 93, 94
Giovanni Conti 96, 97
Luciana Breda 98
L. Breda 99
Antonio Gidaro 100, 101
Maria Francesca Gicchino 102, 103
Carla Gaggiano 3, 4, 5, 6
Antonio Luca Brucato 104, 105
Paola Triggianese 106, 107, 108
Joanna Makowska 109, 110
Francesco Carubbi 111, 112
Nicola Farina 113, 114
Giuliana Guggino 115
Amato de Paulis 116, 117, 118
Maria Antonietta Mazzei 4, 6, 119, 120
Nunzia Di Meglio 4, 6, 119, 120
Alberto Lo Gullo 121
A.L.o Gullo 121
Alessandro Conforti 122, 123
Benson Ogunjimi 124, 125, 126, 127, 128, 129, 130, 131
Laura Calabrese 4, 6, 132, 133, 134, 135
Pietro Rubegni 4, 6, 132, 133, 134, 135
Annarita Giardina 136, 137
Ewa Wiesik-Szewczyk 138
Ewa Więsik-Szewczyk 139
Alberto Balistreri 4, 6, 140, 141
Claudia Fabiani 4, 6, 142, 143
Bruno Frediani 3, 4, 5, 6
Lorenzo Dagna 113, 114, 144, 145
Roberto Giacomelli 14, 15, 16, 17
4
 
Autoinflammatory and Autoimmune Diseases (RITA) Center] Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, , Siena,
6
 
Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] , Siena,
7
 
Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1 , L’Aquila,
14
 
Clinical and research section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico , Rome,
15
 
Department of Medicine, University of Rome “Campus Biomedico” School of Medicine, Rheumatology, Immunology and Clinical Medicine Unit , Rome,
26
 
Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135) , Beijing,
30
 
Horus University Department of Internal Medicine, Faculty of Medicine, , New Damietta,
32
 
Department of Internal Medicine, Faculty of Medicine, Horus University , New Damietta,
34
 
Faculty of Medicine, Newgiza University , 6th of October City,
42
 
Central Hospital of Bolzano Rheumatology Unit, Department of Medicine, , Bolzano,
43
 
Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano , Bolzano,
44
 
“Evangelismos” General Hospital Department of Rheumatology, , Athens,
45
 
Department of Rheumatology, “Evangelismos” General Hospital , Athens,
58
 
Clinical Medicine and Rheumatology Unit, Cattinara University Hospital , Trieste,
60
 
Azienda USL-Toscana Centro Rheumatology Unit, Hospital S. Giovanni di Dio, , Florence,
61
 
Rheumatology Unit, Hospital S. Giovanni di Dio, Azienda USL-Toscana Centro , Florence,
68
 
Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy , Bucharest,
81
 
Periodic Fever Research Center, Università Cattolica Sacro Cuore , Rome,
115
 
Rheumatology Section, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone , Palermo,
121
 
UOSD Reumatologia, ARNAS Garibaldi , Catania,
122
 
Ospedale San Paolo di Civitavecchia, ASL Roma 4, Civitavecchia U.O. Medicina Generale, , Rome,
123
 
U.O. Medicina Generale, Ospedale San Paolo di Civitavecchia , Rome,
136
 
National Relevance and High Specialization Hospital Trust ARNAS Civico, Di Cristina, Benfratelli Department of Clinical Medicine, Internal Medicine Unit with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic Foot Healthcare, , Palermo,
137
 
Department of Clinical Medicine, Internal Medicine Unit with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic Foot Healthcare, National Relevance and High Specialization Hospital Trust ARNAS Civico, Di Cristina, Benfratelli , Palermo,
138
 
Military Institute of Medicine, National Research Institute Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, , Warsaw,
139
 
Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, National Research Institute , Warsaw,
Publication typeJournal Article
Publication date2025-01-20
scimago Q1
wos Q1
SJR1.721
CiteScore9.9
Impact factor4.4
ISSN14620324, 14620332, 14602172
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.

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Ruscitti P. et al. The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still's Disease Registry // Rheumatology. 2025.
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Ruscitti P. et al. The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still's Disease Registry // Rheumatology. 2025.
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@article{2025_Ruscitti,
author = {Piero Ruscitti and Antonio Vitale and Ilenia Di Cola and Valeria Caggiano and Pierpaolo Palumbo and Ernesto Di Cesare and Andrea Hinojosa-Azaola and Jiram Torres-Ruiz and Guillermo Arturo Guaracha-Basañez and Guillermo A. Guaracha-Basáñez and Eduardo Martín-Nares and Giuseppe Lopalco and Maria Morrone and Florenzo Iannone and Henrique A Mayrink Giardini and Henrique Ayres Mayrink Giardini and Rafael Alves Cordeiro and Isabele Parente de Brito Antonelli and Onorina Berardicurti and Luca Navarini and Francesco Ciccia and Maria Chiara Visconti and Daniela Iacono and Haner Direskeneli and Sukran Erten and Haihong Yao and Maissa Thabet and Samar Tharwat and Gaafar Ragab and Verónica Gómez-Caverzaschi and Petros P. Sfikakis and P. Sfikakis and Lampros Fotis and Francesco La Torre and Armin Maier and Anastasios Karamanakos and Ibrahim A Almaghlouth and Micol Frassi and Abdurrahman Tufan and Marcello Govoni and Jurgen Sota and Gabriele Simonini and Giacomo Emmi and Francesca Li Gobbi and Paola Parronchi and Stefania Costi and Piercarlo Sarzi-Puttini and Daniela Opris-Belinski and Paolo Sfriso and Maria Tarsia and others},
title = {The clinical assessment of lung involvement in patients with Still’s disease, results from the multicentre international AIDA Network Still's Disease Registry},
journal = {Rheumatology},
year = {2025},
publisher = {Oxford University Press},
month = {jan},
url = {https://academic.oup.com/rheumatology/advance-article/doi/10.1093/rheumatology/keaf021/7964734},
doi = {10.1093/rheumatology/keaf021}
}