Incidence, prevalence and mortality of systemic sclerosis in Italy: a nationwide population-based study using administra
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Rheumatology International https://doi.org/10.1007/s00296-020-04720-3
INTERNATIONAL
EPIDEMIOLOGY OF RMD
Incidence, prevalence and mortality of systemic sclerosis in Italy: a nationwide population‑based study using administrative health data Jacopo Ciaffi1 · Maria Francesca Morabito2 · Piero Ruscitti3 · Salvatore D’Angelo4 · Luana Mancarella1 · Veronica Brusi1 · Giuseppina Abignano4 · Valentina Pucino5 · Roberto Giacomelli3 · Riccardo Meliconi1,6 · Francesco Ursini1,6 Received: 2 August 2020 / Accepted: 1 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract To study incidence, prevalence and mortality of systemic sclerosis (SSc) in Italy, assessing epidemiological differences between men and women and in distinct age groups. We performed a nationwide population-based study using administrative health data from regional co-payment exemption registries. Patients entitled with SSc-specific co-payment exemption were included. Fourteen of the 20 Italian regions contributed data covering a population of over 45 million individuals. Crude annual incidence rate, annual prevalence, crude annual mortality rate and standardised mortality ratio (SMR) were calculated. In 2016, the overall crude incidence rate of SSc was 18.5 (95% CI 16.9–20.2) per million per year. Incidence rate was 31.0 (95% CI 28.1–34.1) per million in women, and 4.3 (95% CI 3.2–5.6) per million in men. Peak incidence was observed in the age range 55–69 years. Overall annual prevalence was 306.1 (95% CI 301.1–311.2) per million. Prevalence was 530.8 (95% CI 521.5–540.2) per million in women and 67.8 (95% CI 64.4–71.3) per million in men, with a female to male ratio of 7.8:1. Highest prevalence was observed in the range 70–84 years. Crude annual mortality rate was 27.9 (95% CI 24.9–31.1) per 1000 patients. Overall SMR in patients with SSc was 2.8 (95% CI 1.9–3.8). SMR was 3.8 (95% CI 2.9–5.1) in men and 2.6 (95% CI 1.8–3.6) in women. We provided updated estimates on epidemiology of SSc in Italy. Our findings on incidence, prevalence and mortality of SSc are consistent with previously published literature. Keywords Scleroderma · Systemic · Incidence · Prevalence · Mortality
Introduction Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by microvascular dysfunction and excessive collagen deposition, leading to fibrosis of skin and internal organs [1]. The extent of skin involvement defines
* Jacopo Ciaffi [email protected] 1
Medicine and Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli (IOR), Via Pupilli 1, 40136 Bologna, Italy
2
Department of Economics, Statistics and Finance “Giovanni Anania”, University of Calabria, Cosenza, Italy
3
Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L’Aquila, L’Aquila, Italy
two major clinical subtypes, namely limited cutaneous (lcSSc) and diffuse cutaneous SSc (dcSSc) [2]. Although data on its epidemiology are still limited, SSc is considered a rare disease [3]. Divergent fi
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