Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease

Figures & data

Table 1 Quality assessment criteria

Rating^a	Quality criteria^b	Availability criteria^c
High	Majority of studies considered to be of adequate or good methodological quality	Data available for the majority of studies Reported data consistent across the majority of studies CI data available for the majority of studies
Medium	Variation in the methodological quality of studies	Data available for some studies Reported data relatively consistent across studies CI data infrequently reported
Low	Major limitations or risk of bias identified for the majority of studies	Data available for few studies Inconsistencies in reported data across the majority of studies Few or no CI data for the majority of studies

Notes: ^aRating assigned for each dimension (prevalence, incidence, demographic profile, and survival and mortality) and region (Europe and North America). ^bQualitative assessment of the methodologic quality and individual risk of bias was performed considering criteria from the Newcastle–Ottawa scale, the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist, and expert review. ^cQualitative assessment of the quantity of available data.

Abbreviation: CI, confidence interval.

Figure 1 Flow diagram of publications identified for inclusion.

Abbreviations: ILD, interstitial lung disease; SSc, systemic sclerosis.

Table 2 Overview of quality assessment results

Epidemiology data	Disease	Quality rating^a	Availability rating^b
Prevalence:^c Europe	SSc	High	High
SSc-ILD	Medium	Medium
Prevalence:^c North America	SSc	Medium	High
SSc-ILD	High	Medium
Incidence: Europe	SSc	High	High
SSc-ILD	Medium	Low
	SSc	Medium	Medium
Incidence: North America	SSc-ILD	N/A	None
Patient demographics
Age: Europe	SSc	High	High
SSc-ILD	Medium	Medium
Age: North America	SSc	High	High
SSc-ILD	High	High
Gender: Europe	SSc	High	High
SSc-ILD	High	High
Gender: North America	SSc	High	High
SSc-ILD	High	High
Ethnicity: Europe	SSc	High	Medium
SSc-ILD	Medium	Low
Ethnicity: North America	SSc	High	High
SSc-ILD	Low	Low
Survival and mortality: Europe	SSc	High	High
SSc-ILD	Low	Medium
Survival and mortality: North America	SSc	High	High
SSc-ILD	Medium	Medium

Notes: ^aQualitative assessment of the methodologic quality and individual risk of bias was performed considering criteria from the Newcastle–Ottawa scale, the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist, and expert review. ^bQualitative assessment of the quantity of available data. ^cPer 100,000 individuals for patients with SSc and as the proportion (%) of SSc patients who had, or were developing, ILD for patients with SSc-ILD.

Abbreviations: ILD, interstitial lung disease; N/A, not applicable; SSc, systemic sclerosis.

Figure 2 Prevalence of SSc across Europe and North America (per 100,000 individuals).^{1,2,7,16,22,25,26,28,29,31,40,57,59–64}

Table 3 Prevalence of systemic sclerosis

References	Prevalence per 100,000individuals (95% CI)	Study period	Date of prevalence estimate	Location	Diagnostic criteria used
Europe
Allcock et al^Citation59	8.8 (6.8–10.8)	2000	January 2000	Northeast England	ACR ^Citation1980^Citation65 and LeRoy et al ^Citation1988^Citation66
Alamanos et al^Citation29	15.4 (12.0–18.8)	1981–2002	December 2002	Northwest Greece	ACR ^Citation1980 and LeRoy et al ^Citation1988
Le Guern et al^Citation40	15.8 (12.9–18.7)	2001	December 2001	Seine–Saint-Denis County, France	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001^Citation67
El Adssi et al^Citation60	13.2 (11.6–15.4)	2006	June 2006	Lorraine, France	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001
Radić et al^Citation61	15.6 (11.8–19.4)	2008	2008	Split-Dalmatia County, Croatia	ACR ^Citation1980
Airò et al^Citation22	33.9 (15.5–52.3)	2004	December 2004	Valtrompia, Italy	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001
Arias-Nuñez et al^Citation7	27.7 (21.1–35.8) 14.9 (10.1–21.2)	1988–2006	December 2006	Lugo, Spain	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001 ACR ^Citation1980
Eaton et al^Citation62	25 (ND) 23 (ND)	1977–2006	October 2006	Denmark	ICD-8 and ICD-10 codes ICD-10 codes
Andréasson et al^Citation57	23.5 (ND) 30.5 (ND)	2006–2010	December 2010	Skåne, Sweden	ACR ^Citation1980 ACR-EULAR 2013^Citation68
Hoffmann-Vold et al^Citation2	9.9 (8.8–11.2) 7.2 (6.3–8.3)	1999–2009	December 2009	Southeast, Norway	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001 ACR ^Citation1980
Vonk et al^Citation16	8.9 (ND)	2005–2006	January 2007	the Netherlands	ACR ^Citation1980 and LeRoy & Medsger ^Citation2001
North America
Furst et al^Citation25	13.5 (12.4–14.5) 18.4 (17.3–19.5)	2003–2008	2003 2008	USA	ICD-9 codes
Bauer et al^Citation1	39.9 (27.9–52.0)	1980–2010	December 2010	MN, USA	ACR ^Citation1980 and LeRoy et al ^Citation1988
Mayes et al^Citation31	24.2 (21.3–27.4) 27.6 (24.5–31.0)^a	1989–1991	ND	Detroit, USA	ACR ^Citation1980
Helmick et al^Citation63	27.6 (24.5–31.0)	2003–2005	December 2005	USA	ACR ^Citation1980
Robinson Jr. et al^Citation64	30.0 (ND)	2001–2002	ND	USA	ICD-9 codes
Bernatsky et al^Citation26	44.3 (41.1–47.6)	1989–2003	December 2003	Quebec, Canada	ICD-9 codes
Avina-Zubieta et al^Citation28	21.3 (ND)	1990–2007	December 2007	British Columbia, Canada	ICD-9 and ICD-10 codes

Note: ^aCapture-recapture analysis.

Abbreviations: ACR, American College of Rheumatology; CI, confidence interval; EULAR, European League Against Rheumatism; ICD, International Statistical Classification of Diseases and Related Health Problems; ND, not defined.

Figure 3 Proportion (%) of SSc patients who had, or were developing, ILD. Where a range is presented, the light grey bar represents the lower limit and the dark grey bar represents the upper limit.

Abbreviations: ILD, interstitial lung disease; SSc, systemic sclerosis.

Table 4 Annual incidence of systemic sclerosis

References	Annual incidence per 100,000 individuals (95% CI)	Study period	Location	Diagnostic criteria used
Europe
Alamanos et al^Citation29	1.1 (0.2–2.0)	1981–2002	Northwest Greece	ACR 1980 and LeRoy et al 1988
Arias-Nuñez et al^Citation7	2.3 (1.6–2.5) 1.2 (0.9–1.6)	1988–2006	Lugo, Spain	ACR 1980 and LeRoy & Medsger 2001 ACR 1980
Hoffmann-Vold et al^Citation2	0.6–1.1 (ND)	1999–2009	Southeast Norway	ACR 1980 and LeRoy & Medsger 2001
Vonk et al^Citation16	0.8 (ND)	2005–2006	The Netherlands	ACR 1980 and LeRoy & Medsger 2001
Andréasson et al^Citation57	1.4 (ND) 1.9 (ND)	2006–2010	Skåne, Sweden	ACR 1980 ACR-EULAR 2013
North America
Mayes et al^Citation31	1.9 (1.2–3.0) 2.1 (ND)^a	1989–1991	Detroit, USA	ACR 1980
Bauer et al^Citation1	2.4 (1.8–3.0) 1.4 (0.9–1.8)	1980–2010	MN, USA	ACR 1980 and LeRoy et al 1988 ACR 1980
Furst et al^Citation25	5.6 (5.2–6.0)	2003–2008	USA	ICD-9 codes
Avina-Zubieta et al^Citation28	3.6 (ND)	1990–2007	British Columbia, Canada	ICD-9 and ICD-10 codes

Note: ^aCapture-recapture analysis.

Abbreviations: ACR, American College of Rheumatology; CI, confidence interval; EULAR, European League Against Rheumatism; ICD, International Statistical Classification of Diseases and Related Health Problems; ND, not defined.

Table 5 Mean age of patients with systemic sclerosis

References	Mean age, years (±SD or interquartile range)	Study period	Location	Diagnostic criteria used
Europe
Alamanos et al^Citation29	50.2 (15.7)^a	1981–2002	Northwest Greece	ACR 1980 and LeRoy et al 1988
Arias-Nuñez et al^Citation7	59.8 (±13.3)^a	1988–2006	Lugo, Spain	ACR 1980 and LeRoy & Medsger 2001
Hoffmann-Vold et al^Citation2	47 (ND)^a	1999–2009	Southeast Norway	ACR 1980 and LeRoy & Medsger 2001
Strickland et al^Citation15	51.6 (±13.7)^a	1999–2010	Bath, UK	ACR 1980 and LeRoy & Medsger 2001
Epailly et al^Citation32	33.5 (ND)^a	ND	France	ND
Czirják et al^Citation33	56.8 (±12.2)	1983–2005	Hungary	ND
Foti et al^Citation24	50.8 (±12.5)	2006–2013	Catania, Italy	ND
North America
Bauer et al^Citation1	49.1 (39.8–67.6)^a	1980–2010	MN, USA	ACR 1980 and LeRoy et al 1988
Mayes et al^Citation31	46.1 (±15.8)	1989–1991	Detroit, USA	ACR 1980

Note: ^aMean age at diagnosis.

Abbreviations: ACR, American College of Rheumatology; ND, not defined; SD, standard deviation.

Table 6 Gender of patients with systemic sclerosis

References	Female:male	Study period	Location	Diagnostic criteria used
Europe
Alamanos et al^Citation29	9:1	1981–2002	Northwest Greece	ACR 1980 and LeRoy et al 1988
Arias-Nuñez et al^Citation7	3.9:1	1988–2006	Lugo, Spain	ACR 1980 and LeRoy & Medsger 2001
Czirják et al^Citation33	6.2:1	1983–2005	Hungary	ND
Hoffmann-Vold et al^Citation2	3.8:1	1999–2009	Southeast Norway	ACR 1980 and LeRoy & Medsger 2001
Lo Monaco et al^Citation69	9.7:1	1999–2007	Ferrara, Italy	ACR 1980 and LeRoy & Medsger 2001
Strickland et al^Citation15	7.2:1	1999–2010	Bath, UK	ACR 1980 and LeRoy & Medsger 2001
Le Guern et al^Citation40	11.5:1	2001	Seine–Saint-Denis County, France	ACR 1980 and LeRoy & Medsger 2001
Kaliterna et al^Citation70	5.2:1	2007–2009	Split-Dalmatia County, Croatia	ACR 1980
North America
Bauer et al^Citation1	8.1:1	1980–2010	MN, USA	ACR 1980 and LeRoy et al 1988
Mayes et al^Citation31	4.6:1	1989–1991	Detroit, USA	ACR 1980
Mandhadi & Lakshminarayanan et al^Citation13	15:1	2004–2014	CT, USA	ACR-EULAR 2013

Abbreviations: ACR, American College of Rheumatology; EULAR, European League Against Rheumatism; ND, not defined.

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