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Research ArticleSystemic Sclerosis

Systemic Sclerosis and Associated Interstitial Lung Disease in Ontario, Canada: An Examination of Prevalence and Survival Over 10 Years

Janet E. Pope, Kobina Quansah, Shazia Hassan, Soo Jin Seung, Jason Flavin and Martin Kolb
The Journal of Rheumatology September 2021, 48 (9) 1427-1434; DOI: https://doi.org/10.3899/jrheum.201049
Janet E. Pope
1J.E. Pope, MD, Schulich School of Medicine and Dentistry, Western University, St. Joseph Health Care, London;
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  • ORCID record for Janet E. Pope
  • For correspondence: janet.pope@sjhc.london.on.ca
Kobina Quansah
2K. Quansah, MSc, Boehringer Ingelheim (Canada) Ltd., Burlington;
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Shazia Hassan
3S. Hassan, HBSc, S.J. Seung, HBSc, HOPE Research Centre, Sunnybrook Research Institute, Toronto;
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Soo Jin Seung
3S. Hassan, HBSc, S.J. Seung, HBSc, HOPE Research Centre, Sunnybrook Research Institute, Toronto;
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Jason Flavin
4J. Flavin, MA, Boehringer Ingelheim International Gmbh;
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Martin Kolb
5M. Kolb, MD, PhD, Department of Respiratory Medicine, Pathology and Molecular Medicine, McMaster University, Hamilton, Canada.
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  • Figure 1.
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    Figure 1.

    Flow chart of SSc and SSc-ILD included cases. DAD: Discharge Abstract Database; ILD: interstitial lung disease; IKN: Institute for Clinical Evaluative Sciences key number; NACRS: National Ambulatory Care Reporting System; SSc: systemic sclerosis.

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    Figure 2.

    Kaplan-Meier survival curve and risk tables for patients with systemic sclerosis.

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    Figure 3.

    Kaplan-Meier survival curve and risk tables for patients with systemic sclerosis with interstitial lung disease.

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    Table 1.

    Baseline characteristics for prevalent patients with SSc and SSc-ILD over the study period.

    Year2008/92009/102010/112011/12Patients with SSc 2012/132013/142014/152015/162016/172017/18
    n = 497n = 823n = 1073n = 1239n = 1435n = 1590n = 1749n = 1909n = 2007n = 2114
    Age at index date, yrs
        Mean ± SD60.0 ± 14.459.6 ± 14.659.3 ± 14.458.6 ± 14.258.2 ± 14.358.2 ± 14.258.0 ± 14.257.8 ± 14.457.6 ± 14.457.4 ± 14.3
        Median (IQR)60 (51–72)60 (50–71)60 (50–70)59 (49–69)58 (49–69)59 (49–68)58 (49–68)58 (48–68)58 (48–68)58 (48–67)
    Age group, yrs, n (%)
        18–2910 (2.0)18 (2.2)23 (2.2)32 (2.6)48 (3.3)53 (3.3)57 (3.3)64 (3.4)69 (3.4)72 (3.4)
        30–50114 (22.9)199 (24.2)263 (24.5)309 (24.9)354 (24.7)390 (24.5)441 (25.2)493 (25.8)523 (26.1)555 (26.3)
        51–64180 (36.2)299 (36.3)400 (37.3)471 (38.0)533 (37.1)584 (36.7)645 (36.9)702 (36.8)745 (37.1)799 (37.8)
        65+193 (38.8)307 (37.3)387 (36.1)427 (34.5)500 (34.8)563 (35.4)606 (34.7)650 (34.1)670 (33.4)688 (32.5)
    Sex, n (%)
        Female412 (82.9)698 (84.8)914 (85.2)1060 (85.5)1215 (84.7)1353 (85.1)1492 (85.3)1619 (84.8)1698 (84.6)1780 (84.2)
        Male85 (17.1)125 (15.2)159 (14.8)179 (14.5)220 (15.3)237 (14.9)257 (14.7)290 (15.2)309 (15.4)334 (15.8)
    Year2008/92009/102010/112011/12Patients With SSc-ILD 2012/132013/142014/152015/162016/172017/18
    n = 118n = 178n = 227n = 262n = 282n = 288n = 289n = 296n = 281n = 257
    Time to ILD after SSc diagnosis, d
        Mean ± SD964.5 ± 981.5974.4 ± 961.9987.3 ± 910.5971.8 ± 886.9975.7 ± 870.8958.3 ± 861.8935.0 ± 866.2865.0 ± 855.3798.7 ± 846.8732.2 ± 840.1
        Median (IQR)629.5 (107–1632)735.5 (118–1632)810 (176–1609)844.5 (181–1572)848.5 (206–1572)801.5 (222.5–1545)771 (167–1494)611 (144.5–1348)511 (100–1246)390 (68–1129)
    Age at index date, yrs
        Mean ± SD59.1 ± 13.357.8 ± 13.157.8 ± 12.758.4 ± 12.559.0 ± 12.358.9 ± 12.358.4 ± 11.958.5 ± 12.458.1 ± 12.257.9 ± 12.2
        Median (IQR)59 (52–69)57 (50–67)58 (49–67)59 (50–67)60 (51–68)60 (51–68)59 (51–67)59 (50–67.5)59 (50–67)59 (50–66)
    Age group, yrs, n (%)
        18–291–5a1–5a1–5a1–5a1–5a1–5a1–5a1–5a0 (0.00)0 (0.0)
        30–5018–22a42–46a56–60a62–66a62–66a61–65a66–70a71–75a74 (26.33)65 (25.3)
        51–6455 (46.6)77 (43.3)100 (44.1)112 (42.7)119 (42.2)122 (42.4)127 (43.9)124 (41.9)122 (43.4)117 (45.5)
        65+40 (33.9)54 (30.3)66 (29.1)83 (31.7)96 (34.0)100 (34.7)91 (31.5)96 (32.4)85 (30.3)75 (29.2)
    Sex, n (%)
        Female94 (79.7)146 (82.0)183 (80.6)211 (80.5)221 (78.4)233 (80.9)237 (82.0)240 (81.1)224 (79.7)206 (80.2)
        Male24 (20.3)32 (18.0)44 (19.4)51 (19.5)61 (21.6)55 (19.1)52 (18.0)56 (18.9)57 (20.3)51 (19.8)
    • ↵a Exact counts suppressed for privacy reasons. ILD: interstitial lung disease; SSc: systemic sclerosis.

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    Table 2.

    Prevalent SSc and SSc-ILD cases.

    Fiscal YearPrevalent SSc Cases by Diagnosis Year, nCumulative Prevalent Cases of SSca, nPrevalent SSc-ILD Cases by Diagnosis Year, nCumulative Prevalent n Cases of SSc-ILDa, n
    2008/9497497118118
    2009/1038182375178
    2010/11334107370227
    2011/12281123962262
    2012/13290143550282
    2013/14267159044288
    2014/15271174939289
    2015/16272190943296
    2016/17257200738281
    2017/18261211420257
    • ↵a Cumulative prevalence is the sum of patients that are alive with the indication at the start of that year who are recorded that year and were diagnosed in any of the eligible cohort years. For example, cumulative prevalent cases in 2010 refers to all patients alive on April 1, 2010, diagnosed between 2008 and 2010. ILD: interstitial lung disease; SSc: systemic sclerosis.

    • View popup
    Table 3.

    Overall prevalence of patients with SSc and SSc-ILD per 100,000 persons.

    Fiscal Year 2017/18SSc, n = 2114SSc-ILD, n = 257SSc Cases With ILD, %
    Overall, n19.12.312.0
    Age group, yrs, n
        18–293.60.0–
        30–5014.11.712.1
        51–6428.84.214.6
        65+29.43.210.9
    Sex, n
        Female31.23.611.5
        Male6.21.016.1
    • ILD: interstitial lung disease; SSc: systemic sclerosis.

Additional Files

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    • 201049DataSuppl.pdf
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1 Sep 2021
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Systemic Sclerosis and Associated Interstitial Lung Disease in Ontario, Canada: An Examination of Prevalence and Survival Over 10 Years
Janet E. Pope, Kobina Quansah, Shazia Hassan, Soo Jin Seung, Jason Flavin, Martin Kolb
The Journal of Rheumatology Sep 2021, 48 (9) 1427-1434; DOI: 10.3899/jrheum.201049

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Systemic Sclerosis and Associated Interstitial Lung Disease in Ontario, Canada: An Examination of Prevalence and Survival Over 10 Years
Janet E. Pope, Kobina Quansah, Shazia Hassan, Soo Jin Seung, Jason Flavin, Martin Kolb
The Journal of Rheumatology Sep 2021, 48 (9) 1427-1434; DOI: 10.3899/jrheum.201049
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Keywords

INTERSTITIAL LUNG DISEASE
PREVALENCE
SURVIVAL
SYSTEMIC SCLEROSIS

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