Elena Bartoloni1, Miguel A Gonzalez-Gay2, Carlo Scirè3, Santos Castaneda4, Roberto Gerli5, Francisco Javier Lopez-Longo6, Julia Martinez-Barrio6, Marcello Govoni7, Federica Furini7, Trinitario Pina2, Florenzo Iannone8, Margherita Giannini8, Laura Nuño9, Luca Quartuccio10, Norberto Ortego-Centeno11, Alessia Alunno5, Christopher Specker12, Carlomaurizio Montecucco13, Konstantinos Triantafyllias14, Silvia Balduzzi13, Walter Alberto Sifuentes-Giraldo15, Giuseppe Paolazzi16, Elena Bravi17, Andreas Schwarting18, Raffaele Pellerito19, Alessandra Russo19, Carlo Selmi20, Lesley-Ann Saketkoo21, Enrico Fusaro22, Simone Parisi22, Nicolò Pipitone23, Franco Franceschini24, Ilaria Cavazzana24, Rossella Neri25, Simone Barsotti25, Veronica Codullo13, Lorenzo Cavagna13. 1. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. Electronic address: elena.bartolonibocci@unipg.it. 2. Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. 3. Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy. 4. Rheumatology Department, Hospital Universitario de la Princesa, IIS-IP, Madrid, Spain. 5. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. 6. Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. 7. UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. 8. Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. 9. Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. 10. Clinic of Rheumatology, Department of Medical and Biological Sciences (DSMB), Santa Maria della Misericordia Hospital, Udine, Italy. 11. Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. 12. Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. 13. Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. 14. ACURA Rheumatology Center, Bad Kreuznach, Germany. 15. Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. 16. Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. 17. Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. 18. Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. 19. Division of Rheumatology, Mauriziano Hospital, Turin, Italy. 20. Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy. 21. Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center New Orleans, New Orleans, LA, USA. 22. Rheumatology Department, Città Della Salute e della Scienza, Torino, Italy. 23. Rheumatology Unit, Department of Internal Medicine, Azienda Ospedaliera ASMN, Reggio Emilia, Italy. 24. Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. 25. Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Abstract
OBJECTIVE: Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. METHODS: Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. RESULTS: 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002). CONCLUSION: Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.
OBJECTIVE:Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. METHODS: Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. RESULTS: 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002). CONCLUSION: Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.
Authors: Katalin Szabó; Levente Bodoki; Melinda Nagy-Vincze; Anett Vincze; Erika Zilahi; Peter Szodoray; Katalin Dankó; Zoltán Griger Journal: Biomed Res Int Date: 2018-10-25 Impact factor: 3.411
Authors: Federica Furini; Aldo Carnevale; Gian Luca Casoni; Giulio Guerrini; Lorenzo Cavagna; Marcello Govoni; Carlo Alberto Sciré Journal: Front Med (Lausanne) Date: 2019-10-31
Authors: Raquel López-Mejías; Sara Remuzgo-Martínez; Fernanda Genre; Verónica Pulito-Cueto; Sonia M Fernández Rozas; Javier Llorca; David Iturbe Fernández; Víctor M Mora Cuesta; Norberto Ortego-Centeno; Nair Pérez Gómez; Antonio Mera-Varela; Julia Martínez-Barrio; Francisco Javier López-Longo; Verónica Mijares; Leticia Lera-Gómez; María Piedad Usetti; Rosalía Laporta; Virginia Pérez; Alicia De Pablo Gafas; María Aránzazu Alfranca González; Jaime Calvo-Alén; Fredeswinda Romero-Bueno; Olga Sanchez-Pernaute; Laura Nuno; Gema Bonilla; Alejandro Balsa; Fernanda Hernández-González; Ignacio Grafia; Sergio Prieto-González; Javier Narvaez; Ernesto Trallero-Araguas; Albert Selva-O'Callaghan; Oreste Gualillo; Santos Castañeda; Lorenzo Cavagna; José M Cifrian; Miguel A González-Gay Journal: Sci Rep Date: 2020-01-29 Impact factor: 4.379