دورية أكاديمية
Epidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy)
| العنوان: | Epidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy) |
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| المؤلفون: | Coi A, Barsotti S, Santoro M, Almerigogna F, Bargagli E, Caproni M, Emmi G, Frediani B, Guiducci S, Matucci Cerinic M, Mosca M, Parronchi P, Prediletto R, Selvi E, Simonini G, Tavoni AG, Rare Diseases Working Group, Bianchi F, Pierini A |
| المساهمون: | Coi, A, Barsotti, S, Santoro, M, Almerigogna, F, Bargagli, E, Caproni, M, Emmi, G, Frediani, B, Guiducci, S, Matucci Cerinic, M, Mosca, M, Parronchi, P, Prediletto, R, Selvi, E, Simonini, G, Tavoni, Ag, Rare Diseases Working, Group, Bianchi, F, Pierini, A |
| سنة النشر: | 2021 |
| المجموعة: | ARPI - Archivio della Ricerca dell'Università di Pisa |
| مصطلحات موضوعية: | Systemic sclerosis, Survival, Mortality risk, Comorbidity, Disease registry, Rare disease, Pharmacoepidemiology |
| الوصف: | 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 ... |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | ELETTRONICO |
| اللغة: | English |
| العلاقة: | info:eu-repo/semantics/altIdentifier/pmid/33596949; info:eu-repo/semantics/altIdentifier/wos/WOS:000620917600002; volume:16; issue:1; numberofpages:13; journal:ORPHANET JOURNAL OF RARE DISEASES; https://hdl.handle.net/11568/1115710Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85101679125; https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01733-4Test |
| DOI: | 10.1186/s13023-021-01733-4 |
| الإتاحة: | https://doi.org/10.1186/s13023-021-01733-4Test https://hdl.handle.net/11568/1115710Test |
| حقوق: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.E2E01B6B |
| قاعدة البيانات: | BASE |
| DOI: | 10.1186/s13023-021-01733-4 |
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