Alessio Coi1, Simone Barsotti2, Michele Santoro3, Fabio Almerigogna4, Elena Bargagli5, Marzia Caproni6,7, Giacomo Emmi8, Bruno Frediani9, Serena Guiducci10, Marco Matucci Cerinic10, Marta Mosca2, Paola Parronchi8, Renato Prediletto3,11, Enrico Selvi12, Gabriele Simonini13, Antonio Gaetano Tavoni14, Fabrizio Bianchi3,11, Anna Pierini3,11. 1. Institute of Clinical Physiology, National Research Council, Via Moruzzi 1, Pisa, Italy. alessio.coi@ifc.cnr.it. 2. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. 3. Institute of Clinical Physiology, National Research Council, Via Moruzzi 1, Pisa, Italy. 4. Immunoallergology Unit, , Careggi University Hospital, Florence, Italy. 5. Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, Siena, Italy. 6. Rare Dermatological Diseases Unit, USL Toscana Centro, Firenze, Italy. 7. ERN-SKIN Diseases Centre, Department of Health Sciences, University of Florence, Firenze, Italy. 8. Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy. 9. Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, "Le Scotte" Hospital, Siena, Italy. 10. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. 11. Fondazione Toscana "Gabriele Monasterio", Pisa, Italy. 12. Rheumatology Unit, Azienda Ospedaliero Universitaria Senese, Siena, Italy. 13. Rheumatology Unit, A. Meyer Children's University Hospital, University of Florence, Florence, Italy. 14. Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Abstract
BACKGROUND: Systemic Sclerosis (SSc) is a chronic autoimmune disease with a complex pathogenesis that includes vascular injury, abnormal immune activation, and tissue fibrosis. We provided a complete epidemiological characterization of SSc in the Tuscany region (Italy), considering prevalence and incidence, survival, comorbidities and drug prescriptions, by using a multi-database population-based approach. Cases of SSc diagnosed between 1st January 2003 and 31st December 2017 among residents in Tuscany were collected from the population-based Rare Diseases Registry of Tuscany. All cases were linked to regional health and demographic databases to obtain information about vital statistics, principal causes of hospitalization, complications and comorbidities, and drug prescriptions. RESULTS: The prevalence of SSc in Tuscany population resulted to be 22.2 per 100,000, with the highest prevalence observed for the cases aged ≥ 65 years (33.2 per 100,000, CI 95% 29.6-37.3). In females, SSc was predominant (86.7% on the total) with an overall sex ratio F/M of 6.5. Nevertheless, males presented a more severe disease, with a lower survival and significant differences in respiratory complications and metabolic comorbidities. Complications and comorbidities such as pulmonary involvement (HR = 1.66, CI 95% 1.17-2.35), congestive heart failure (HR = 2.76, CI 95% 1.80-4.25), subarachnoid and intracerebral haemorrhage (HR = 2.33, CI 95% 1.21-4.48) and malignant neoplasms (HR = 1.63, CI 95% 1.06-2.52), were significantly associated to a lower survival, also after adjustment for age, sex and other SSc-related complications. Disease-modifying antirheumatic drugs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors were the drugs with the more increasing prevalence of use in the 2008-2017 period. CONCLUSIONS: The multi-database approach is important in the investigation of rare diseases where it is often difficult to provide accurate epidemiological indicators. A population-based registry can be exploited in synergy with health databases, to provide evidence related to disease outcomes and therapies and to assess the burden of disease, relying on a large cohort of cases. Building an integrated archive of data from multiple databases linking a cohort of patients to their comorbidities, clinical outcomes and survival, is important both in terms of treatment and prevention.
BACKGROUND:Systemic Sclerosis (SSc) is a chronic autoimmune disease with a complex pathogenesis that includes vascular injury, abnormal immune activation, and tissue fibrosis. We provided a complete epidemiological characterization of SSc in the Tuscany region (Italy), considering prevalence and incidence, survival, comorbidities and drug prescriptions, by using a multi-database population-based approach. Cases of SSc diagnosed between 1st January 2003 and 31st December 2017 among residents in Tuscany were collected from the population-based Rare Diseases Registry of Tuscany. All cases were linked to regional health and demographic databases to obtain information about vital statistics, principal causes of hospitalization, complications and comorbidities, and drug prescriptions. RESULTS: The prevalence of SSc in Tuscany population resulted to be 22.2 per 100,000, with the highest prevalence observed for the cases aged ≥ 65 years (33.2 per 100,000, CI 95% 29.6-37.3). In females, SSc was predominant (86.7% on the total) with an overall sex ratio F/M of 6.5. Nevertheless, males presented a more severe disease, with a lower survival and significant differences in respiratory complications and metabolic comorbidities. Complications and comorbidities such as pulmonary involvement (HR = 1.66, CI 95% 1.17-2.35), congestive heart failure (HR = 2.76, CI 95% 1.80-4.25), subarachnoid and intracerebral haemorrhage (HR = 2.33, CI 95% 1.21-4.48) and malignant neoplasms (HR = 1.63, CI 95% 1.06-2.52), were significantly associated to a lower survival, also after adjustment for age, sex and other SSc-related complications. Disease-modifying antirheumatic drugs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors were the drugs with the more increasing prevalence of use in the 2008-2017 period. CONCLUSIONS: The multi-database approach is important in the investigation of rare diseases where it is often difficult to provide accurate epidemiological indicators. A population-based registry can be exploited in synergy with health databases, to provide evidence related to disease outcomes and therapies and to assess the burden of disease, relying on a large cohort of cases. Building an integrated archive of data from multiple databases linking a cohort of patients to their comorbidities, clinical outcomes and survival, is important both in terms of treatment and prevention.
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