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

Ciaffi, Jacopo; Morabito, Maria Francesca; Ruscitti, Piero; D’Angelo, Salvatore; Mancarella, Luana; Brusi, Veronica; Abignano, Giuseppina; Pucino, Valentina; Giacomelli, Roberto; Melicòni, Riccardo; Ursini, F.

doi:10.1007/s00296-020-04720-3

Cited by 21 publications

(13 citation statements)

References 40 publications

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“…Usually, studies focus on the mortality of one group of RD patients, and the whole burden may be underrepresented. Mortality of systemic CTDs (especially SLE and SSc) [2,14,15,[32][33][34][35][36][37][38][39][40][41][42][43][44][47][48][49][50][51][52][67][68][69][70][71][72][73] and SV [2,14,15,[53][54][55][56][57][58][59][60][61][74][75][76] are covered better, as well as mortality of patients with RA and PsA [2,14,[16][17][18][19]…”

Section: Discussionmentioning

confidence: 99%

“…The data on myositis, Sjogren's syndrome, and especially AS and polymyalgia rheumatica mortality are still scarce and fragmented [2,14,15,30,31,45,46,[62][63][64][65][66]77,91]. Myositis, SSc, SLE, and vasculitis are the diseases with the highest reported SMRs (ranging from 2.4 to 9.0 for myositis, from 1.34 to 5.7 for SSc, from 1.48 to 5.25 for SLE, and from 0.5 to 3.7 for vasculitis) [2,14,15,[32][33][34][35][36][37][38][39][40][41][42][43][44][47][48][49][50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65]. SMRs for RA ranged from...…”

Section: Discussionmentioning

confidence: 99%

“…Usually, studies focus on the mortality of one group of RD patients, and the whole burden may be underrepresented. Mortality of systemic CTDs (especially SLE and SSc) [ 2 , 14 , 15 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 47 , 48 , 49 , 50 , 51 , 52 , 67 , 68 , 69 , 70 , 71 , 72 , 73 ] and SV [ 2 , 14 , 15 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 74 , 75 , 76 ] are covered better, as well as mortality of patients with RA and PsA [ 2 , 14 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 2...…”

Section: Discussionmentioning

confidence: 99%

“…The data on myositis, Sjogren’s syndrome, and especially AS and polymyalgia rheumatica mortality are still scarce and fragmented [ 2 , 14 , 15 , 30 , 31 , 45 , 46 , 62 , 63 , 64 , 65 , 66 , 77 , 91 ]. Myositis, SSc, SLE, and vasculitis are the diseases with the highest reported SMRs (ranging from 2.4 to 9.0 for myositis, from 1.34 to 5.7 for SSc, from 1.48 to 5.25 for SLE, and from 0.5 to 3.7 for vasculitis) [ 2 , 14 , 15 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 ]. SMRs for RA ranged from 1.04 to 1.89, for PsA—from 0.82 to 1.59, for AS—from 0.72 to 1.87, for Sjogren’s syndrome—from 1.1 to 2.11.…”

Section: Discussionmentioning

confidence: 99%

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Mortality in Inflammatory Rheumatic Diseases: Lithuanian National Registry Data and Systematic Review

Dadonienė

Charukevič

Jasionytė

et al. 2021

IJERPH

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Despite significant improvement in survival, rheumatic diseases (RD) are associated with premature mortality rates comparable to cardiovascular and neoplastic disorders. The aim of our study was to assess mortality, causes of death, and life expectancy in an inflammatory RD retrospective cohort and compare those with the general population as well as with the results of previously published studies in a systematic literature review. Patients with the first-time diagnosis of inflammatory RD during 2012–2019 were identified and cross-checked for their vital status and the date of death. Sex- and age-standardized mortality ratios (SMR) as well as life expectancy for patients with inflammatory RDs were calculated. The results of a systematic literature review were included in meta-standardized mortality ratio calculations. 11,636 patients with newly diagnosed RD were identified. During a total of 43,064.34 person-years of follow-up, 950 death cases occurred. The prevailing causes of death for the total cohort were cardiovascular diseases and neoplasms. The age- and sex-adjusted SMR for the total cohort was calculated to be 1.32 (1.23; 1.40). Patients with rheumatoid arthritis if diagnosed at age 18–19 tend to live for 1.63 years less than the general population, patients with spondyloarthritis—for 2.7 years less, patients with connective tissue diseases—for almost nine years less than the general population. The findings of our study support the hypothesis that patients with RD have a higher risk of mortality and lower life expectancy than the general population.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Usually, studies focus on the mortality of one group of RD patients, and the whole burden may be underrepresented. Mortality of systemic CTDs (especially SLE and SSc) [ 2 , 14 , 15 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 47 , 48 , 49 , 50 , 51 , 52 , 67 , 68 , 69 , 70 , 71 , 72 , 73 ] and SV [ 2 , 14 , 15 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 74 , 75 , 76 ] are covered better, as well as mortality of patients with RA and PsA [ 2 , 14 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 2...…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Mortality in Inflammatory Rheumatic Diseases: Lithuanian National Registry Data and Systematic Review

Dadonienė

Charukevič

Jasionytė

et al. 2021

IJERPH

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“…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 ]. SSc is considered a rare disease [ 2 ] and its pathogenesis is poorly understood, with a possible role of genetic, hormonal, environmental and occupational factors [ 1 , 3 , 4 ]. The extent of skin involvement defines two major clinical subtypes, namely limited cutaneous (lcSSc) and diffuse cutaneous SSc (dcSSc) [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Whole body insulin sensitivity is increased in systemic sclerosis

et al. 2023

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Objectives In the present study, we aimed to evaluate whole-body insulin sensitivity in systemic sclerosis (SSc) patients and to compare the results with controls with no autoimmune rheumatic disease (non-ARD) and with patients affected by rheumatoid arthritis (RA). Methods In all patients and controls, oral glucose tolerance test (OGTT) was performed according to the World Health Organization (WHO) recommendations. Plasma glucose and insulin concentrations were measured at time 0 and then after 30, 60, 90, and 120 minutes. Whole-body insulin sensitivity (ISI), insulinogenic index (IGI), oral disposition index (ODI), and insulin resistance (HOMA-IR) were estimated accordingly. Results A total of 41 SSc patients were evaluated and, for comparison, 41 individuals with RA and 82 non-ARD control patients were recruited. OGTT yielded a proportion of normotolerant individuals among SSc patients higher than in RA controls (p = 0.040) but lower than in the non-ARD group (p = 0.028). The ISI was significantly higher in SSc patients compared with RA controls (p <0.001) and with non-ARD patients (p <0.001). Significant differences emerged also when analysing the HOMA-IR, which was lower in SSc patients than in RA (p <0.001) and non-ARD (p <0.001) groups. Additionally, IGI was lower in SSc patients compared with RA (p = 0.011) and with non-ARD controls (p <0.001), whereas ODI was not significantly different between groups. Conclusions Interestingly, we found that SSc patients are more insulin sensitive than those with RA and even than individuals without inflammatory diseases. In contrast, no significant difference was found in terms of β-cell function.

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Systemic Sclerosis

Orteu,

Denton

2024

Rook's Textbook of Dermatology

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

Cited by 21 publications

References 40 publications

Mortality in Inflammatory Rheumatic Diseases: Lithuanian National Registry Data and Systematic Review

Mortality in Inflammatory Rheumatic Diseases: Lithuanian National Registry Data and Systematic Review

Whole body insulin sensitivity is increased in systemic sclerosis

Systemic Sclerosis

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