Frontiers in Medicine (May 2023)
Axial spondyloarthritis in patients with recurrent fever attacks: data from the AIDA network registry for undifferentiated autoInflammatory diseases (USAIDs)
- Antonio Vitale,
- Antonio Vitale,
- Valeria Caggiano,
- Valeria Caggiano,
- Isabel Silva,
- Daniel G. Oliveira,
- Daniel G. Oliveira,
- Piero Ruscitti,
- Francesco Ciccia,
- Ibrahim Vasi,
- Abdurrahman Tufan,
- Giuseppe Lopalco,
- Ibrahim A. AlMaghlouth,
- Jurgen Sota,
- Jurgen Sota,
- Ewa Wiesik-Szewczyk,
- Carla Gaggiano,
- Carla Gaggiano,
- Henrique Ayres Mayrink Giardini,
- Veronica Spedicato,
- Gaafar Ragab,
- Gaafar Ragab,
- Florenzo Iannone,
- Alberto Balistreri,
- Micol Frassi,
- José Hernández-Rodríguez,
- Claudia Fabiani,
- Claudia Fabiani,
- Paolo Falsetti,
- Paolo Falsetti,
- Nunzia Di Meglio,
- Nunzia Di Meglio,
- Bruno Frediani,
- Bruno Frediani,
- Maria Antonietta Mazzei,
- Maria Antonietta Mazzei,
- Donato Rigante,
- Donato Rigante,
- Raquel Faria,
- Luca Cantarini,
- Luca Cantarini
Affiliations
- Antonio Vitale
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Antonio Vitale
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Valeria Caggiano
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Valeria Caggiano
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Isabel Silva
- Medicine Department, Centro Hospitalar Universitário de Santo António, Porto, Portugal
- Daniel G. Oliveira
- UMIB—Unit for Multidisciplinary Research in Biomedicine, ICBAS School of Medicine and Biomedical Sciences, University of Porto, Porto, Portugal
- Daniel G. Oliveira
- Instituto de Investigação e Inovação em Saúde, Universidade do Porto, Porto, Portugal
- Piero Ruscitti
- Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, L’Aquila, Italy
- Francesco Ciccia
- Department of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy
- Ibrahim Vasi
- Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Türkiye
- Abdurrahman Tufan
- Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Türkiye
- Giuseppe Lopalco
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
- Ibrahim A. AlMaghlouth
- 0Rheumatology Unit, Department of Medicine, King Saud University, Riyadh, Saudi Arabia
- Jurgen Sota
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Jurgen Sota
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Ewa Wiesik-Szewczyk
- 1Department 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
- Carla Gaggiano
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Carla Gaggiano
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Henrique Ayres Mayrink Giardini
- 2Rheumatology Division, Faculdade de Medicina, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), Universidade de São Paulo, São Paulo, Brazil
- Veronica Spedicato
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
- Gaafar Ragab
- 3Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt
- Gaafar Ragab
- 4Faculty of Medicine, Newgiza University, Giza, Egypt
- Florenzo Iannone
- Rheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy
- Alberto Balistreri
- 5Bioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy
- Micol Frassi
- 6Rheumatology 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
- José Hernández-Rodríguez
- 7Vasculitis Research Unit and Autoinflammatory Diseases Clinical Unit, Department of Autoimmune Diseases, Hospital Clinic of Barcelona, IDIBAPS, University of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Barcelona, Spain
- Claudia Fabiani
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Claudia Fabiani
- 8Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy
- Paolo Falsetti
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Paolo Falsetti
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Nunzia Di Meglio
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Nunzia Di Meglio
- 9Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy
- Bruno Frediani
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Bruno Frediani
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Maria Antonietta Mazzei
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- Maria Antonietta Mazzei
- 9Unit of Diagnostic Imaging, Department of Medical, Surgical and Neuro Sciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Italy
- Donato Rigante
- 0Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
- Donato Rigante
- 1Rare Diseases and Periodic Fevers Research Centre, Università Cattolica Sacro Cuore, Rome, Italy
- Raquel Faria
- 2Unidade de Imunologia ClínicaCentro Hospitalar Universitário de Santo António Porto [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Porto, Portugal
- Luca Cantarini
- Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, University of Siena, Siena, Italy
- Luca Cantarini
- Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Siena, Italy
- DOI
- https://doi.org/10.3389/fmed.2023.1195995
- Journal volume & issue
-
Vol. 10
Abstract
BeckgroundDespite the recent advances in the field of autoinflammatory diseases, most patients with recurrent fever episodes do not have any defined diagnosis. The present study aims at describing a cohort of patients suffering from apparently unexplained recurrent fever, in whom non-radiographic axial spondylarthritis (SpA) represented the unique diagnosis identified after a complete clinical and radiologic assessment.Materials and methodsPatients’ data were obtained from the international registry on Undifferentiated Systemic AutoInflammatory Diseases (USAIDs) developed by the AutoInflammatory Disease Alliance (AIDA) network.ResultsA total of 54 patients with recurrent fever episodes were also affected by non-radiographic axial SpA according to the international classification criteria. SpA was diagnosed after the start of fever episodes in all cases; the mean age at the diagnosis of axial SpA was 39.9 ± 14.8 years with a diagnostic delay of 9.3 years. The highest body temperature reached during flares was 42°C, with a mean temperature of 38.8 ± 1.1°C. The most frequent manifestations associated to fever were: arthralgia in 33 (61.1%) cases, myalgia in 24 (44.4%) cases, arthritis in 22 (40.7%) cases, headache in 15 (27.8%) cases, diarrhea in 14 (25.9%) cases, abdominal pain in 13 (24.1%) cases, and skin rash in 12 (22.1%) cases. Twenty-four (44.4%) patients have taken daily or on-demand non-steroidal anti-inflammatory drugs (NSAIDs) and 31 (57.4%) patients have been treated with daily or on demand oral glucocorticoids. Colchicine was used in 28 (51.8%) patients, while other conventional disease modifying anti-rheumatic drugs (cDMARDs) were employed in 28 (51.8%) patients. Forty (74.1%) patients underwent anti-tumor necrosis factor (TNF) agents and 11 (20.4%) were treated with interleukin (IL)-1 inhibitors. The response to TNF inhibitors on recurrent fever episodes appeared more effective than that observed with anti-IL-1 agents; colchicine and other cDMARDs were more useful when combined with biotechnological agents.ConclusionSigns and symptoms referring to axial SpA should be inquired in patients with apparently unexplained recurrent fever episodes. The specific treatment for axial SpA may lead to a remarkable improvement in the severity and/or frequency of fever episodes in patients with unexplained fevers and concomitant axial SpA.
Keywords
