Skip to main content
Top
Published in: Rheumatology International 1/2021

01-01-2021 | Systemic Sclerosis | Epidemiology of RMD

Incidence, prevalence and mortality of systemic sclerosis in Italy: a nationwide population-based study using administrative health data

Authors: Jacopo Ciaffi, Maria Francesca Morabito, Piero Ruscitti, Salvatore D’Angelo, Luana Mancarella, Veronica Brusi, Giuseppina Abignano, Valentina Pucino, Roberto Giacomelli, Riccardo Meliconi, Francesco Ursini

Published in: Rheumatology International | Issue 1/2021

Login to get access

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.
Literature
4.
go back to reference Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980). Arthritis Rheum. 23(5):581–590. https://doi.org/10.1002/art.1780230510 Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980). Arthritis Rheum. 23(5):581–590. https://​doi.​org/​10.​1002/​art.​1780230510
5.
go back to reference LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205PubMed LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205PubMed
6.
go back to reference LeRoy EC, Medsger TA Jr (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576PubMed LeRoy EC, Medsger TA Jr (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28(7):1573–1576PubMed
7.
go back to reference van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Muller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747. https://doi.org/10.1002/art.38098CrossRefPubMedPubMedCentral van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Muller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747. https://​doi.​org/​10.​1002/​art.​38098CrossRefPubMedPubMedCentral
8.
go back to reference Lo Monaco A, Bruschi M, La Corte R, Volpinari S, Trotta F (2011) Epidemiology of systemic sclerosis in a district of northern Italy. Clin Exp Rheumatol 29(2 Suppl 65):S10-14PubMed Lo Monaco A, Bruschi M, La Corte R, Volpinari S, Trotta F (2011) Epidemiology of systemic sclerosis in a district of northern Italy. Clin Exp Rheumatol 29(2 Suppl 65):S10-14PubMed
12.
go back to reference Carrara G, Scire CA, Zambon A, Cimmino MA, Cerra C, Caprioli M, Cagnotto G, Nicotra F, Arfe A, Migliazza S, Corrao G, Minisola G, Montecucco C (2015) A validation study of a new classification algorithm to identify rheumatoid arthritis using administrative health databases: case-control and cohort diagnostic accuracy studies. Results from the RECord linkage On Rheumatic Diseases study of the Italian Society for Rheumatology. BMJ Open 5(1):e006029. https://doi.org/10.1136/bmjopen-2014-006029 Carrara G, Scire CA, Zambon A, Cimmino MA, Cerra C, Caprioli M, Cagnotto G, Nicotra F, Arfe A, Migliazza S, Corrao G, Minisola G, Montecucco C (2015) A validation study of a new classification algorithm to identify rheumatoid arthritis using administrative health databases: case-control and cohort diagnostic accuracy studies. Results from the RECord linkage On Rheumatic Diseases study of the Italian Society for Rheumatology. BMJ Open 5(1):e006029. https://​doi.​org/​10.​1136/​bmjopen-2014-006029
14.
17.
go back to reference Airò P, Tabaglio E, Frassi M, Scarsi M, Danieli E, Rossi M (2007) Prevalence of systemic sclerosis in Valtrompia in northern Italy. A collaborative study of rheumatologists and general practitioners. Clin Exp Rheumatol 25 (6):878–880 Airò P, Tabaglio E, Frassi M, Scarsi M, Danieli E, Rossi M (2007) Prevalence of systemic sclerosis in Valtrompia in northern Italy. A collaborative study of rheumatologists and general practitioners. Clin Exp Rheumatol 25 (6):878–880
18.
go back to reference Airò P, Regola F, Lazzaroni MG, Tincani A, Inverardi F, Fenini MG, Ferre F, Furloni R, Scarsi M (2019) Incidence and prevalence of systemic sclerosis in Valcamonica, Italy, during an 18-year period. J Scleroderma Relat Disord 5(1):51–56. https://doi.org/10.1177/2397198318819908 Airò P, Regola F, Lazzaroni MG, Tincani A, Inverardi F, Fenini MG, Ferre F, Furloni R, Scarsi M (2019) Incidence and prevalence of systemic sclerosis in Valcamonica, Italy, during an 18-year period. J Scleroderma Relat Disord 5(1):51–56. https://​doi.​org/​10.​1177/​2397198318819908​
29.
36.
go back to reference Elhai M, Avouac J, Walker UA, Matucci-Cerinic M, Riemekasten G, Airo P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Balbir Gurman A, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Muller-Ladner U, Kahan A, Distler O, Allanore Y (2016) A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75(1):163–169. https://doi.org/10.1136/annrheumdis-2014-206386CrossRefPubMed Elhai M, Avouac J, Walker UA, Matucci-Cerinic M, Riemekasten G, Airo P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Balbir Gurman A, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Muller-Ladner U, Kahan A, Distler O, Allanore Y (2016) A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75(1):163–169. https://​doi.​org/​10.​1136/​annrheumdis-2014-206386CrossRefPubMed
41.
go back to reference Pelkonen PM, Jalanko HJ, Lantto RK, Makela AL, Pietikainen MA, Savolainen HA, Verronen PM (1994) Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland. J Rheumatol 21(11):2143–2146PubMed Pelkonen PM, Jalanko HJ, Lantto RK, Makela AL, Pietikainen MA, Savolainen HA, Verronen PM (1994) Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland. J Rheumatol 21(11):2143–2146PubMed
44.
go back to reference Caputo M, Pecere A, Sarro A, Mele C, Ucciero A, Pagano L, Prodam F, Aimaretti G, Marzullo P, Barone-Adesi F (2020) Incidence and prevalence of hyperthyroidism: a population-based study in the Piedmont Region, Italy. Endocrine. 10.1007/s12020-020-02222-7 Caputo M, Pecere A, Sarro A, Mele C, Ucciero A, Pagano L, Prodam F, Aimaretti G, Marzullo P, Barone-Adesi F (2020) Incidence and prevalence of hyperthyroidism: a population-based study in the Piedmont Region, Italy. Endocrine. 10.1007/s12020-020-02222-7
45.
go back to reference Gini R, Schuemie MJ, Francesconi P, Lapi F, Cricelli I, Pasqua A, Gallina P, Donato D, Brugaletta S, Donatini A, Marini A, Cricelli C, Damiani G, Bellentani M, van der Lei J, Sturkenboom MC, Klazinga NS (2014) Can Italian healthcare administrative databases be used to compare regions with respect to compliance with standards of care for chronic diseases? PLoS ONE 9(5):e95419. https://doi.org/10.1371/journal.pone.0095419CrossRefPubMedPubMedCentral Gini R, Schuemie MJ, Francesconi P, Lapi F, Cricelli I, Pasqua A, Gallina P, Donato D, Brugaletta S, Donatini A, Marini A, Cricelli C, Damiani G, Bellentani M, van der Lei J, Sturkenboom MC, Klazinga NS (2014) Can Italian healthcare administrative databases be used to compare regions with respect to compliance with standards of care for chronic diseases? PLoS ONE 9(5):e95419. https://​doi.​org/​10.​1371/​journal.​pone.​0095419CrossRefPubMedPubMedCentral
Metadata
Title
Incidence, prevalence and mortality of systemic sclerosis in Italy: a nationwide population-based study using administrative health data
Authors
Jacopo Ciaffi
Maria Francesca Morabito
Piero Ruscitti
Salvatore D’Angelo
Luana Mancarella
Veronica Brusi
Giuseppina Abignano
Valentina Pucino
Roberto Giacomelli
Riccardo Meliconi
Francesco Ursini
Publication date
01-01-2021
Publisher
Springer Berlin Heidelberg
Published in
Rheumatology International / Issue 1/2021
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-020-04720-3

Other articles of this Issue 1/2021

Rheumatology International 1/2021 Go to the issue