Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry

Piero Ruscitti¹, Ilenia Di Cola², Antonio Vitale³, Valeria Caggiano³, Pierpaolo Palumbo⁴, Ernesto Di Cesare⁴, Jiram Torres-Ruiz⁵, Guillermo Arturo Guaracha-Basañez⁵, Eduardo Martín-Nares⁵, Francesco Ciccia⁶, Daniela Iacono⁶, Flavia Riccio⁶, Maria Cristina Maggio⁷, Samar Tharwat⁸, Soad Hashad⁹, Donato Rigante¹⁰, Augusta Ortolan¹¹, Henrique A Mayrink Giardini¹², Isabele Parente de Brito Antonelli¹², Rafael Alves Cordeiro¹², Roberto Giacomelli¹³, Luca Navarini¹³, Onorina Berardicurti¹³, Alessandro Conforti¹⁴, Daniela Opris-Belinski¹⁵, Jurgen Sota³, Carla Gaggiano³, Giuseppe Lopalco¹⁶, Fiorenzo Iannone¹⁶, Francesco La Torre¹⁷, Violetta Mastrorilli¹⁷, Marcello Govoni¹⁸, Francesca Ruffilli¹⁸, Giacomo Emmi¹⁹, Edoardo Biancalana²⁰, Petros P Sfikakis²¹, Maria Tektonidou²¹, José Hernández-Rodríguez²², Verónica Gómez-Caverzaschi²², Özgül Soysal Gündüz²³, Giovanni Conti²⁴, Serena Patroniti²⁴, Antonio Gidaro²⁵, Arianna Bartoli²⁵, Alma Nunzia Olivieri²⁶, M Francesca Gicchino²⁶, Antonio Luca Brucato²⁷, Lorenzo Dagna²⁸, Alessandro Tomelleri²⁸, Corrado Campochiaro²⁸, Amato De Paulis²⁹, Ilaria Mormile³⁰, Francesca Della Casa³⁰, Haner Direskeneli³¹, Fatma Alibaz-Oner³¹, Anastasios Karamanakos³², Aikaterini Dimouli³², Gaafar Ragab³³, Ayman Abdelmonem Ahmed Mahmoud³⁴, Abdurrahman Tufan³⁵, Hamit Kucuk³⁵, Riza Kardas³⁵, Ezgi D Batu³⁶, Seza Ozen³⁶, Ewa Wiesik-Szewczyk³⁷, Andrea Hinojosa-Azaola⁵, Alberto Balistreri³⁸, Claudia Fabiani³⁹, Bruno Frediani³, Luca Cantarini; International AIDA Network and the Autoinflammatory Diseases Working Group of the Italian Society of Rheumatology

Affiliations

¹ P. Ruscitti, MD, PhD, I. Di Cola, MD, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy; cantariniluca@hotmail.com piero.ruscitti@univaq.it.
² P. Ruscitti, MD, PhD, I. Di Cola, MD, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
³ A. Vitale, MD, V. Caggiano, MD, J. Sota, MD, C. Gaggiano, MD, B. Frediani, MD, L. Cantarini, MD, PhD, Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, and Azienda Ospedaliero-Universitaria Senese (European Reference Network for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases [ERN-RITA] Center), Siena, Italy.
⁴ P. Palumbo, MD, E. Di Cesare, MD, Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, L'Aquila, Italy.
⁵ J. Torres-Ruiz, MD, G.A. Guaracha-Basañez, MD, E. Martín-Nares, MD, A. Hinojosa-Azaola, MD, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁶ F. Ciccia, MD, D. Iacono, MD, F. Riccio, MD, Department of Precision Medicine, University of Campania "Luigi Vanvitelli," Naples, Italy.
⁷ M.C. Maggio, MD, University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro," University of Palermo, Palermo, Italy.
⁸ S. Tharwat, MD, Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, and Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
⁹ S. Hashad, MD, Rheumatology Department, Tripoli Children Hospital, Tripoli, Libya.
¹⁰ D. Rigante, MD, Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, and Università Cattolica Sacro Cuore, Rome, Italy.
¹¹ A. Ortolan, MD, Rheumatology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
¹² H.A. Mayrink Giardini, MD, I.P. de Brito Antonelli, MD, R.A. Cordeiro, MD, Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
¹³ R. Giacomelli, MD, L. Navarini, MD, O. Berardicurti, MD, Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, and Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rome, Italy.
¹⁴ A. Conforti, MD, U.O. Medicina Generale, Ospedale San Paolo di Civitavecchia, ASL Roma 4, Civitavecchia, Rome, Italy.
¹⁵ D. Opris-Belinski, MD, Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
¹⁶ G. Lopalco, MD, F. Iannone, MD, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
¹⁷ F. La Torre, MD, V. Mastrorilli, MD, Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
¹⁸ M. Govoni, MD, F. Ruffilli, MD, Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
¹⁹ G. Emmi, MD, Department of Medical, Surgical and Health Sciences, University of Trieste, Italy, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, and Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia.
²⁰ E. Biancalana, MD, Department of Experimental and Clinical Medicine, Florence, Italy.
²¹ P.P. Sfikakis, MD, M. Tektonidou, MD, Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
²² J. Hernández-Rodríguez, MD, V. Gómez-Caverzaschi, MD, Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona (ERN-RITA Center), University of Barcelona, Barcelona, Spain.
²³ Ö.S. Gündüz, MD, Department of Internal Medicine, Division of Rheumatology, Celal Bayar University School of Medicine, Manisa, Turkey.
²⁴ G. Conti, MD, S. Patroniti, MD, Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), "G. Martino" Messina, Italy.
²⁵ A. Gidaro, MD, A. Bartoli, MD, Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
²⁶ A.N. Olivieri, MD, M.F. Gicchino, MD, Department of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli," Naples, Italy.
²⁷ A.L. Brucato, MD, Pediatric Unit, Fatebenefratelli Hospital, Milan, Italy.
²⁸ L. Dagna, MD, A. Tomelleri, MD, C. Campochiaro, MD, School of Medicine, Vita-Salute San Raffaele University, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.
²⁹ A. De Paulis, MD, Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, and Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
³⁰ I. Mormile, MD, F. Della Casa, MD, Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
³¹ H. Direskeneli, MD, F. Alibaz-Oner, MD, Department of Internal Medicine, Division of Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey.
³² A. Karamanakos, MD, A. Dimouli, MD, Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
³³ G. Ragab, MD, Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, and Faculty of Medicine, Newgiza University, 6th of October City, Egypt.
³⁴ A.A.A. Mahmoud, MD, Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt.
³⁵ A. Tufan, MD, H. Kucuk, MD, R. Kardas, MD, Gazi University Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey.
³⁶ E.D. Batu, MD, S. Ozen, MD, Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
³⁷ E. Wiesik-Szewczyk, MD, 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.
³⁸ A. Balistreri, MD, Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, and Azienda Ospedaliero-Universitaria Senese (ERN-RITA Center), Siena, Italy.
³⁹ C. Fabiani, MD, Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, and Azienda Ospedaliero-Universitaria Senese (ERN-RITA Center), Siena, Italy.

PMID: 39547690
DOI: 10.3899/jrheum.2024-0683

Free article

Observational Study

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry

Piero Ruscitti et al. J Rheumatol. 2025.

Free article

. 2025 Mar 1;52(3):226-233.

doi: 10.3899/jrheum.2024-0683.

Authors

Affiliations

¹ P. Ruscitti, MD, PhD, I. Di Cola, MD, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy; cantariniluca@hotmail.com piero.ruscitti@univaq.it.
² P. Ruscitti, MD, PhD, I. Di Cola, MD, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
³ A. Vitale, MD, V. Caggiano, MD, J. Sota, MD, C. Gaggiano, MD, B. Frediani, MD, L. Cantarini, MD, PhD, Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, and Azienda Ospedaliero-Universitaria Senese (European Reference Network for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases [ERN-RITA] Center), Siena, Italy.
⁴ P. Palumbo, MD, E. Di Cesare, MD, Department of Diagnostic Imaging, Area of Cardiovascular and Interventional Imaging, Abruzzo Health Unit 1, L'Aquila, Italy.
⁵ J. Torres-Ruiz, MD, G.A. Guaracha-Basañez, MD, E. Martín-Nares, MD, A. Hinojosa-Azaola, MD, Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
⁶ F. Ciccia, MD, D. Iacono, MD, F. Riccio, MD, Department of Precision Medicine, University of Campania "Luigi Vanvitelli," Naples, Italy.
⁷ M.C. Maggio, MD, University Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro," University of Palermo, Palermo, Italy.
⁸ S. Tharwat, MD, Rheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, and Department of Internal Medicine, Faculty of Medicine, Horus University, New Damietta, Egypt.
⁹ S. Hashad, MD, Rheumatology Department, Tripoli Children Hospital, Tripoli, Libya.
¹⁰ D. Rigante, MD, Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, and Università Cattolica Sacro Cuore, Rome, Italy.
¹¹ A. Ortolan, MD, Rheumatology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
¹² H.A. Mayrink Giardini, MD, I.P. de Brito Antonelli, MD, R.A. Cordeiro, MD, Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas, Universidade de São Paulo, São Paulo, Brazil.
¹³ R. Giacomelli, MD, L. Navarini, MD, O. Berardicurti, MD, Clinical and Research Section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, and Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rome, Italy.
¹⁴ A. Conforti, MD, U.O. Medicina Generale, Ospedale San Paolo di Civitavecchia, ASL Roma 4, Civitavecchia, Rome, Italy.
¹⁵ D. Opris-Belinski, MD, Rheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
¹⁶ G. Lopalco, MD, F. Iannone, MD, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J) Policlinic Hospital, University of Bari, Bari, Italy.
¹⁷ F. La Torre, MD, V. Mastrorilli, MD, Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
¹⁸ M. Govoni, MD, F. Ruffilli, MD, Rheumatology Unit, Department of Medical Sciences, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
¹⁹ G. Emmi, MD, Department of Medical, Surgical and Health Sciences, University of Trieste, Italy, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, and Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia.
²⁰ E. Biancalana, MD, Department of Experimental and Clinical Medicine, Florence, Italy.
²¹ P.P. Sfikakis, MD, M. Tektonidou, MD, Joint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
²² J. Hernández-Rodríguez, MD, V. Gómez-Caverzaschi, MD, Department of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona (ERN-RITA Center), University of Barcelona, Barcelona, Spain.
²³ Ö.S. Gündüz, MD, Department of Internal Medicine, Division of Rheumatology, Celal Bayar University School of Medicine, Manisa, Turkey.
²⁴ G. Conti, MD, S. Patroniti, MD, Pediatric Nephrology and Rheumatology Unit, Azienda Ospedaliera Universitaria (AOU), "G. Martino" Messina, Italy.
²⁵ A. Gidaro, MD, A. Bartoli, MD, Department of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
²⁶ A.N. Olivieri, MD, M.F. Gicchino, MD, Department of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli," Naples, Italy.
²⁷ A.L. Brucato, MD, Pediatric Unit, Fatebenefratelli Hospital, Milan, Italy.
²⁸ L. Dagna, MD, A. Tomelleri, MD, C. Campochiaro, MD, School of Medicine, Vita-Salute San Raffaele University, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS Ospedale San Raffaele, Milan, Italy.
²⁹ A. De Paulis, MD, Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, and Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, University of Naples Federico II, Naples, Italy.
³⁰ I. Mormile, MD, F. Della Casa, MD, Department of Translational Medical Sciences, Section of Clinical Immunology, University of Naples Federico II, Naples, Italy.
³¹ H. Direskeneli, MD, F. Alibaz-Oner, MD, Department of Internal Medicine, Division of Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey.
³² A. Karamanakos, MD, A. Dimouli, MD, Department of Rheumatology, "Evangelismos" General Hospital, Athens, Greece.
³³ G. Ragab, MD, Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, and Faculty of Medicine, Newgiza University, 6th of October City, Egypt.
³⁴ A.A.A. Mahmoud, MD, Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt.
³⁵ A. Tufan, MD, H. Kucuk, MD, R. Kardas, MD, Gazi University Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey.
³⁶ E.D. Batu, MD, S. Ozen, MD, Department of Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
³⁷ E. Wiesik-Szewczyk, MD, 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.
³⁸ A. Balistreri, MD, Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, and Azienda Ospedaliero-Universitaria Senese (ERN-RITA Center), Siena, Italy.
³⁹ C. Fabiani, MD, Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, and Azienda Ospedaliero-Universitaria Senese (ERN-RITA Center), Siena, Italy.

PMID: 39547690
DOI: 10.3899/jrheum.2024-0683

Abstract

Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

Results: In total, 73 patients with Still disease and cardiac involvement were assessed (mean age 36.3 [SD 19.9] years; male sex, 42.5%), out of which 21.9% were children. The most common cardiac manifestation was pericarditis, occurring in 90.4% of patients; patients also presented with myocarditis (26%), and less frequently endocarditis (2.7%) and tamponade (1.4%). In comparing clinical features of patients with myocarditis to those without, significantly increased frequencies of skin rash and pleuritis, as well as higher systemic scores, were seen. Further, a higher mortality rate was shown in patients with myocarditis. In regression models, skin rash and the systemic score independently predicted the myocarditis.

Conclusion: The characteristics of patients with Still disease and cardiac involvement were assessed in the AIDA Network. The most common feature was the pericarditis, but a more severe clinical picture was also reported in patients with myocarditis. The latter was associated with increased mortality rate and higher systemic score, identifying patients who should be carefully managed.

Keywords: adult-onset Still disease; juvenile arthritis; myocarditis.

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Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry

Affiliations

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry

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Abstract

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