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

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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.

Table 1.

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

Year	2008/9	2009/10	2010/11	2011/12	Patients with SSc 2012/13	2013/14	2014/15	2015/16	2016/17	2017/18
	n = 497	n = 823	n = 1073	n = 1239	n = 1435	n = 1590	n = 1749	n = 1909	n = 2007	n = 2114
Age at index date, yrs
Mean ± SD	60.0 ± 14.4	59.6 ± 14.6	59.3 ± 14.4	58.6 ± 14.2	58.2 ± 14.3	58.2 ± 14.2	58.0 ± 14.2	57.8 ± 14.4	57.6 ± 14.4	57.4 ± 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 (%)
18–29	10 (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)
30–50	114 (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)
51–64	180 (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 (%)
Female	412 (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)
Male	85 (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)

Year	2008/9	2009/10	2010/11	2011/12	Patients With SSc-ILD 2012/13	2013/14	2014/15	2015/16	2016/17	2017/18
	n = 118	n = 178	n = 227	n = 262	n = 282	n = 288	n = 289	n = 296	n = 281	n = 257
Time to ILD after SSc diagnosis, d
Mean ± SD	964.5 ± 981.5	974.4 ± 961.9	987.3 ± 910.5	971.8 ± 886.9	975.7 ± 870.8	958.3 ± 861.8	935.0 ± 866.2	865.0 ± 855.3	798.7 ± 846.8	732.2 ± 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 ± SD	59.1 ± 13.3	57.8 ± 13.1	57.8 ± 12.7	58.4 ± 12.5	59.0 ± 12.3	58.9 ± 12.3	58.4 ± 11.9	58.5 ± 12.4	58.1 ± 12.2	57.9 ± 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 (%)
18–29	1–5^{^a}	1–5^{^a}	1–5^{^a}	1–5^{^a}	1–5^{^a}	1–5^{^a}	1–5^{^a}	1–5^{^a}	0 (0.00)	0 (0.0)
30–50	18–22^{^a}	42–46^{^a}	56–60^{^a}	62–66^{^a}	62–66^{^a}	61–65^{^a}	66–70^{^a}	71–75^{^a}	74 (26.33)	65 (25.3)
51–64	55 (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 (%)
Female	94 (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)
Male	24 (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.

Table 2.

Prevalent SSc and SSc-ILD cases.

Fiscal Year	Prevalent SSc Cases by Diagnosis Year, n	Cumulative Prevalent Cases of SSc^{^a}, n	Prevalent SSc-ILD Cases by Diagnosis Year, n	Cumulative Prevalent n Cases of SSc-ILD^{^a}, n
2008/9	497	497	118	118
2009/10	381	823	75	178
2010/11	334	1073	70	227
2011/12	281	1239	62	262
2012/13	290	1435	50	282
2013/14	267	1590	44	288
2014/15	271	1749	39	289
2015/16	272	1909	43	296
2016/17	257	2007	38	281
2017/18	261	2114	20	257

↵^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.