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

Piero Ruscitti¹, Antonio Vitale^{2

3}, Ilenia Di Cola¹, Valeria Caggiano^{2

3}, Pierpaolo Palumbo⁴, Ernesto Di Cesare¹, Andrea Hinojosa-Azaola⁵, Jiram Torres-Ruiz⁵, Guillermo Arturo Guaracha-Basañez⁵, Eduardo Martín-Nares⁵, Giuseppe Lopalco⁶, Maria Morrone⁶, Florenzo Iannone⁶, Henrique A Mayrink Giardini⁷, Rafael Alves Cordeiro⁷, Isabele Parente de Brito Antonelli⁷, Onorina Berardicurti^{8

9}, Luca Navarini^{8

9}, Francesco Ciccia¹⁰, Maria Chiara Visconti¹⁰, Daniela Iacono¹⁰, Haner Direskeneli¹¹, Sukran Erten¹², Haihong Yao^{13

14}, Maissa Thabet¹⁵, Samar Tharwat^{16

17}, Gaafar Ragab^{18

19}, Verónica Gómez-Caverzaschi²⁰, Petros P Sfikakis²¹, Lampros Fotis²², Francesco La Torre²³, Armin Maier²⁴, Anastasios Karamanakos²⁵, Ibrahim A Almaghlouth^{26

27}, Micol Frassi²⁸, Abdurrahman Tufan²⁹, Marcello Govoni³⁰, Jurgen Sota^{2

3}, Gabriele Simonini³¹, Giacomo Emmi^{32

33

34}, Francesca Li Gobbi³⁵, Paola Parronchi³⁶, Stefania Costi³⁷, Piercarlo Sarzi-Puttini³⁸, Daniela Opris-Belinski³⁹, Paolo Sfriso⁴⁰, Maria Tarsia^{2

3}, Maria Cristina Maggio⁴¹, Sara Monti^{42

43}, Özgül Soysal Gündüz⁴⁴, Donato Rigante^{45

46}, Elena Bartoloni⁴⁷, Elena Verrecchia^{48

49}, Annamaria Iagnocco⁵⁰, Ombretta Viapiana⁵¹, Elena Bargagli^{3

52}, Ezgi D Batu⁵³, Gian Domenico Sebastiani⁵⁴, Emanuela Del Giudice⁵⁵, Giovanni Conti⁵⁶, Luciana Breda⁵⁷, Antonio Gidaro⁵⁸, Maria Francesca Gicchino⁵⁹, Carla Gaggiano^{2

3}, Antonio Luca Brucato⁶⁰, Paola Triggianese^{61

62}, Joanna Makowska⁶³, Francesco Carubbi⁶⁴, Nicola Farina^{65

66}, Giuliana Guggino⁶⁷, Amato De Paulis^{68

69}, Maria Antonietta Mazzei^{3

70}, Nunzia Di Meglio^{3

70}, Alberto Lo Gullo⁷¹, Alessandro Conforti⁷², Benson Ogunjimi^{73

74

75

76}, Laura Calabrese^{3

77

78}, Pietro Rubegni^{3

77

78}, Annarita Giardina⁷⁹, Ewa Wiesik-Szewczyk⁸⁰, Alberto Balistreri^{3

81}, Claudia Fabiani^{3

82}, Bruno Frediani^{2

3}, Lorenzo Dagna^{65

66}, Roberto Giacomelli^{8

9}, Luca Cantarini^{2

3}

Affiliations

¹ Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
² Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
³ Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
⁴ Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, L'Aquila, Italy.
⁵ Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁶ Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
⁷ Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
⁸ Clinical and research section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome, Italy.
⁹ Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rheumatology, Immunology and Clinical Medicine Unit, Rome, Italy.
¹⁰ Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
¹¹ Department of Internal Medicine, Division of Rheumatology, Faculty of Medic, Marmara University, Istanbul, Turkey.
¹² Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yıldırım Beyazıt University, Ankara, Turkey.
¹³ Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
¹⁴ Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China.
¹⁵ Internal Medicine Department, Faculty of Medicine of Sousse, Farhat Hached University Hospital, University of Sousse, Sousse, Tunisia.
¹⁶ Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
¹⁷ Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
¹⁸ Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
¹⁹ Faculty of Medicine, Newgiza University, 6th of October City, Egypt.
²⁰ Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain.
²¹ Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
²² Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
²³ Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
²⁴ Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
²⁵ Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
²⁶ Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
²⁷ College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
²⁸ Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy.
²⁹ Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.
³⁰ Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
³¹ NEUROFARBA Department, Rheumatology Unit, Meyer Children's Hospital IRCCS, University of Florence, Florence, Italy.
³² Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy.
³³ Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy.
³⁴ Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia.
³⁵ Rheumatology Unit, Hospital S. Giovanni di Dio, Azienda USL-Toscana Centro, Florence, Italy.
³⁶ Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
³⁷ Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.
³⁸ Rheumatology Unit, IRCCS Ospedale Galeazzi-S. Ambrogio, Università degli Studi di Milano, Milan, Italy.
³⁹ Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁴⁰ Rheumatology Unit, Department of Medicine, University of Padua, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Padua, Italy.
⁴¹ University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro", University of Palermo, Palermo, Italy.
⁴² Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
⁴³ Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.
⁴⁴ Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
⁴⁵ Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
⁴⁶ Periodic Fever Research Center, Università Cattolica Sacro Cuore, Rome, Italy.
⁴⁷ Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.
⁴⁸ Rare Diseases and Periodic Fevers Research Centre, Università Cattolica del Sacro Cuore, Rome, Italy.
⁴⁹ Department of Aging, Neurological, Orthopedic and Head and Neck Sciences, Fondazione Policlinico Universitario Agostino Gemelli Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy.
⁵⁰ Academic Rheumatology Centre, Dipartimento Scienze Cliniche e Biologiche, Università degli Studi di Torino, Torino, Italy.
⁵¹ Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Italy.
⁵² UOC Respiratory Diseases and Lung Transplant, Department Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
⁵³ Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey.
⁵⁴ UOC di Reumatologia, Azienda Ospedaliera San Camillo Forlanini, Roma, Italy.
⁵⁵ Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Latina, Italy.
⁵⁶ Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), "G. Martino" Messina, Messina, Italy.
⁵⁷ Department of Paediatrics, University of Chieti-Pescara, Chieti, Italy.
⁵⁸ Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
⁵⁹ Department of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
⁶⁰ Pediatric Unit, Fatebenefratelli Hospital, Milan, Italy.
⁶¹ Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
⁶² Molecular Medicine and Applied Biotechnology, University of Rome Tor Vergata, Rome, Italy.
⁶³ Department of Rheumatology, Medical University of Lodz, Lodz, Poland.
⁶⁴ Department of Life, Health & Environmental Sciences and Internal Medicine and Nephrology Unit, Department of Medicine, University of L'Aquila and ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
⁶⁵ Faculty of Medicine, Università Vita-Salute San Raffaele, Milan, Italy.
⁶⁶ Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.
⁶⁷ Rheumatology Section, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, Palermo, Italy.
⁶⁸ Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
⁶⁹ Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
⁷⁰ Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy.
⁷¹ UOSD Reumatologia, ARNAS Garibaldi, Catania, Italy.
⁷² U.O. Medicina Generale, Ospedale San Paolo di Civitavecchia, Rome, Italy.
⁷³ Antwerp Unit for Data Analysis and Computation in Immunology and Sequencing, University of Antwerp, Antwerp, Belgium.
⁷⁴ Antwerp Center for Translational Immunology and Virology, Vaccine and Infectious Disease Institute, University of Antwerp, Antwerp, Belgium.
⁷⁵ Department of Paediatrics, Antwerp University Hospital, Antwerp, Belgium.
⁷⁶ Center for Health Economics Research and Modeling Infectious Diseases, Vaccine and Infectious Disease Institute, University of Antwerp, Antwerp, Belgium.
⁷⁷ Dermatology Unit, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy.
⁷⁸ Institute of Dermatology, Catholic University of the Sacred Heart, Rome, Italy.
⁷⁹ 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, Italy.
⁸⁰ 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, Poland.
⁸¹ Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
⁸² Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.

PMID: 39832276
DOI: 10.1093/rheumatology/keaf021

Observational Study

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 et al. Rheumatology (Oxford). 2025.

. 2025 Dec 1;64(SI):SI169-SI178.

doi: 10.1093/rheumatology/keaf021.

Authors

Affiliations

¹ Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
² Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
³ Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
⁴ Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, L'Aquila, Italy.
⁵ Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁶ Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
⁷ Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
⁸ Clinical and research section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Rome, Italy.
⁹ Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rheumatology, Immunology and Clinical Medicine Unit, Rome, Italy.
¹⁰ Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
¹¹ Department of Internal Medicine, Division of Rheumatology, Faculty of Medic, Marmara University, Istanbul, Turkey.
¹² Department of Rheumatology, Faculty of Medicine Ankara City Hospital, Ankara Yıldırım Beyazıt University, Ankara, Turkey.
¹³ Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
¹⁴ Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China.
¹⁵ Internal Medicine Department, Faculty of Medicine of Sousse, Farhat Hached University Hospital, University of Sousse, Sousse, Tunisia.
¹⁶ Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
¹⁷ Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
¹⁸ Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
¹⁹ Faculty of Medicine, Newgiza University, 6th of October City, Egypt.
²⁰ Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain.
²¹ Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
²² Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
²³ Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
²⁴ Rheumatology Unit, Department of Medicine, Central Hospital of Bolzano, Bolzano, Italy.
²⁵ Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
²⁶ Rheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
²⁷ College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
²⁸ Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy.
²⁹ Department of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.
³⁰ Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
³¹ NEUROFARBA Department, Rheumatology Unit, Meyer Children's Hospital IRCCS, University of Florence, Florence, Italy.
³² Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy.
³³ Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy.
³⁴ Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia.
³⁵ Rheumatology Unit, Hospital S. Giovanni di Dio, Azienda USL-Toscana Centro, Florence, Italy.
³⁶ Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
³⁷ Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, University of Milan, Milan, Italy.
³⁸ Rheumatology Unit, IRCCS Ospedale Galeazzi-S. Ambrogio, Università degli Studi di Milano, Milan, Italy.
³⁹ Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
⁴⁰ Rheumatology Unit, Department of Medicine, University of Padua, [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Padua, Italy.
⁴¹ University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro", University of Palermo, Palermo, Italy.
⁴² Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
⁴³ Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.
⁴⁴ Division of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
⁴⁵ Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
⁴⁶ Periodic Fever Research Center, Università Cattolica Sacro Cuore, Rome, Italy.
⁴⁷ Rheumatology Unit, Department of Medicine and Surgery, University of Perugia, Perugia, Italy.
⁴⁸ Rare Diseases and Periodic Fevers Research Centre, Università Cattolica del Sacro Cuore, Rome, Italy.
⁴⁹ Department of Aging, Neurological, Orthopedic and Head and Neck Sciences, Fondazione Policlinico Universitario Agostino Gemelli Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy.
⁵⁰ Academic Rheumatology Centre, Dipartimento Scienze Cliniche e Biologiche, Università degli Studi di Torino, Torino, Italy.
⁵¹ Rheumatology Unit, Department of Medicine, University and Azienda Ospedaliera Universitaria Integrata of Verona, Italy.
⁵² UOC Respiratory Diseases and Lung Transplant, Department Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
⁵³ Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey.
⁵⁴ UOC di Reumatologia, Azienda Ospedaliera San Camillo Forlanini, Roma, Italy.
⁵⁵ Pediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Latina, Italy.
⁵⁶ Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), "G. Martino" Messina, Messina, Italy.
⁵⁷ Department of Paediatrics, University of Chieti-Pescara, Chieti, Italy.
⁵⁸ Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
⁵⁹ Department of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
⁶⁰ Pediatric Unit, Fatebenefratelli Hospital, Milan, Italy.
⁶¹ Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
⁶² Molecular Medicine and Applied Biotechnology, University of Rome Tor Vergata, Rome, Italy.
⁶³ Department of Rheumatology, Medical University of Lodz, Lodz, Poland.
⁶⁴ Department of Life, Health & Environmental Sciences and Internal Medicine and Nephrology Unit, Department of Medicine, University of L'Aquila and ASL Avezzano-Sulmona-L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
⁶⁵ Faculty of Medicine, Università Vita-Salute San Raffaele, Milan, Italy.
⁶⁶ Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.
⁶⁷ Rheumatology Section, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, Palermo, Italy.
⁶⁸ Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
⁶⁹ Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
⁷⁰ Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy.
⁷¹ UOSD Reumatologia, ARNAS Garibaldi, Catania, Italy.
⁷² U.O. Medicina Generale, Ospedale San Paolo di Civitavecchia, Rome, Italy.
⁷³ Antwerp Unit for Data Analysis and Computation in Immunology and Sequencing, University of Antwerp, Antwerp, Belgium.
⁷⁴ Antwerp Center for Translational Immunology and Virology, Vaccine and Infectious Disease Institute, University of Antwerp, Antwerp, Belgium.
⁷⁵ Department of Paediatrics, Antwerp University Hospital, Antwerp, Belgium.
⁷⁶ Center for Health Economics Research and Modeling Infectious Diseases, Vaccine and Infectious Disease Institute, University of Antwerp, Antwerp, Belgium.
⁷⁷ Dermatology Unit, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy.
⁷⁸ Institute of Dermatology, Catholic University of the Sacred Heart, Rome, Italy.
⁷⁹ 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, Italy.
⁸⁰ 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, Poland.
⁸¹ Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
⁸² Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.

PMID: 39832276
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.

Keywords: Still’s disease; adult-onset Still’s disease; lung involvement; prognosis; systemic juvenile idiopathic arthritis.

© The Author(s) 2025. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.

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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

Affiliations

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

Authors

Affiliations

Abstract

Publication types

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LinkOut - more resources

Full Text Sources