Best Practice & Research Clinical Rheumatology 35 (2021) 101667

Contents lists available at ScienceDirect

Best Practice & Research Clinical

Rheumatology
journal homepage: www.elsevierhealth.com/berh

Employment outcomes in systemic sclerosis

Jennifer J.Y. Lee a, *, Monique A.M. Gignac b,
Sindhu R. Johnson c
a
Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital,
Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada
b
Institute for Work and Health, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario,
Canada
c
Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital,
Toronto Western Hospital; Institute of Health Policy, Management and Evaluation, University of Toronto,
Toronto, Ontario, Canada

a b s t r a c t
Keywords:
Scleroderma Work disability is highly prevalent in the systemic sclerosis (SSc)
Systemic sclerosis population; yet, it is an area of research that continues to be
Employment underrecognized and underexplored. In this chapter, we review
Work disability the burden of this work disability by exploring the reported
Absenteeism prevalence of work loss, the risk factors associated with reduced
Productivity
work participation, the impact on work productivity outcomes,
Presenteeism
and the economic consequences of work disability in individuals
with SSc. Finally, we discuss the potential challenges in the
workplace and strategies that may foster employment retention in
this population. We subsequently present a conceptual framework
for work disability in the context of SSc, which incorporates our
understanding of the various work disability concepts and the
potential facilitators that may accelerate a worker toward com-
plete work loss.
© 2021 Elsevier Ltd. All rights reserved.

Introduction

Systemic sclerosis (SSc), also referred to as scleroderma, is a chronic autoimmune connective tissue
disease characterized by progressive fibrosis of the skin and widespread vasculopathy [1]. As a het-
erogeneous condition, affected individuals can experience a multitude of clinical features affecting

* Corresponding author. Toronto Scleroderma Program, Suite 2-004, 60 Murray Street, Toronto, Ontario, Canada, M5T 3L9.
E-mail address: jenniferjiy.lee@mail.utoronto.ca (J.J.Y. Lee).

https://doi.org/10.1016/j.berh.2021.101667
1521-6942/© 2021 Elsevier Ltd. All rights reserved.
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

several organ systems. Manifestations may include skin tightening, joint contractures, Raynaud's
phenomenon, ischemic digital ulcers, and interstitial lung disease [2e4]. The psychological impact and
distress of the disease is also high, with studies reporting associations with both mood and anxiety
disorders [5,6]. Consequently, studies have reported reduced health-related quality of life, greater
functional disability, and limited participation in various life roles [7e11]. The onset of SSc coinci-
dentally peaks between 20 and 50 years of age, which coincide with the peak age for optimal work
participation and employment. This naturally places patients with SSc at a significant risk for short-
term and long-term work disability [12,13].
Musculoskeletal diseases and arthritis are the most frequently reported causes of physical disability in
the working population, with greater than 50% of adults with arthritis-related disability remaining out of
the workforce [14e16]. The individual and societal burden of work disability is significant. For instance, in
Canada, it is reported that the estimated economic cost of arthritis is over $4.4 billion each year, with a
significant proportion of indirect costs related to underemployment [17]. Several studies have reported
that the total indirect costs, including the value of potential productivity loss related to unpaid labor, exceed
direct medical costs by two-to threefold in adults with arthritis and systemic autoimmune diseases [18,19].
Much of the previous research on arthritis and employment has been focused on those with
rheumatoid arthritis (RA) and osteoarthritis. There is a gap in terms of our understanding of the impact
of SSc on employment and work-related outcomes. In this review, we synthesize the literature eval-
uating the prevalence of work loss, the risk factors associated with work disability, work productivity
outcomes, and the economic impact of work disability in individuals with SSc. We highlight common
outcomes that are used to measure work disability in patients with SSc. Furthermore, we discuss the
potential challenges in the workplace and comment on strategies that may foster employment
retention in this population.

Work disability in systemic sclerosis

Work disability and related concepts

The definition of work disability can vary across countries and in different studies [20]. The most
commonly utilized definition of work disability is work loss, which is the premature cessation of
employment, which can range from complete work loss (i.e., unemployment), early or premature
retirement, and a temporary giving up of paid work (e.g., short-term or long-term disability). However,
limitations from SSc can also be described as at-work disability or work-related disability and
measured as work activity limitations or work productivity losses. Work activity limitations refer to
difficulties in the execution of work-related tasks and may reflect a reduction in the physical or mental
ability (i.e., mobility and concentration) to execute tasks, or a reduction in the quality or quantity of job-
related tasks [20,21]. Work productivity losses can be defined as a combination of absenteeism and
presenteeism [22,23]. Absenteeism refers to the time missed from work, in this case due to SSc health
reasons, commonly captured as the number of days/hours off from work. Presenteeism is the time or
amount of impaired performance while at work due to health reasons that result in productivity loss. It
can also encompass work activity limitation or can be represented by the loss of time and money [23].
Various methods to gauge worker productivity have been examined [24].

Work disability in systemic sclerosis

Prevalence of work loss

Numerous studies have evaluated the prevalence of work loss (either partial or complete work loss)
in the SSc population (Table 1). Work loss was most commonly identified as using self-reported
employment status in questionnaires. Of the 11,315 patients with SSc in 36 studies, the mean preva-
lence of unemployment was 40.5% (4e92%). The mean age was 52.7 (standard deviation 4.6) years and

2
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

the mean disease duration was 10.3 (standard deviation 8.5) years. Thirty-five percent of the patients
had the diffuse subtype and 77% were female patients. Definitions of employment status varied, and
most studies did not subcategorize employed individuals further into full-time or part-time employ-
ment. In most studies, it was unclear how studies categorized specific worker populations, including
workers who were employed but on short-term or long-term disability or individuals whose work is
considered self-employed, seasonal, or contract-based.
Only 2 studies reported work loss in the SSc population in comparison with other rheumatic dis-
eases [25,26]. Ouimet et al. compare the frequency of work disability (defined as stopping an occu-
pation due to illness) between a cohort of patients with SSc and a cohort of patients with RA. Fifty-six
percent of people living with SSc were classified as work disabled as compared to 34.6% of people living
with RA, with a statistically significant odds ratio (OR) of 2.38 (p < 0.009), after a mean disease duration
of 11 years [25]. Mau et al. compared work participation of individuals living with SSc with the general
population in Germany and with several chronic inflammatory diseases, including RA, ankylosing
spondylitis (AS), psoriatic arthritis (PsA), Wegener's granulomatosis (WG), and systemic lupus ery-
thematosus (SLE), by determining standardized employment ratios (SERs). The SERs were estimated by
determining the ratio of observed and expected number of individuals employed [26]. SERs were
significantly reduced in all inflammatory conditions as compared to the general population, with
people living with SSc having an SER of 0.70e0.77 in women and 0.84e0.90 in men. Individuals with
SSc, along with RA, SLE, and WG had lower employment rates than people with PsA and AS.
At-work disability outcomes, including worker productivity loss or work activity limitations, in
individuals with SSc have been explored, albeit to a lesser extent than the prevalence of work loss.
Table 2 categorizes the studies according to the measurement tools that were used to explore at-work
or work-related disability other than employment status identified in Table 1. Approximately half of the
studies did not include a specific measurement tool to measure at-work disability, other than patients
asking to self-report whether they considered themselves disabled at work. Certain studies gauged
work-related disability by measuring the frequency, duration, or use of sick leaves [27,28], occupational
changes or transitions [29], professional difficulties [30], number of job disruptions [31], or the
acquisition of disability pensions [27].
Only a few studies incorporated the use of specific measurement tools used to measure at-work
disability and productivity [32]. Some of these measures have been evaluated in similar patient
groups (mainly RA), but further research is needed to determine if these measurement tools are valid,
reliable, and can detect change in the SSc population. Four studies utilized the work productivity and
activity impairment tool (WPAI), which measure the effect of health and symptoms on work pro-
ductivity and nonwork activities. The WPAI is designed to measure both absenteeism and presenteeism
from 0% to 100% over a recall period of 7 days, with greater scores indicating a greater level of
impairment [32]. Morrisoe et al. evaluated the construct validity of the instrument in a cohort of pa-
tients with SSc and found that the productivity outcomes correlated well with other measures that are
relevant to health status, including overall function, fatigue, and pain scores, WHO functional class, and
overall disease activity. In 476 patients with SSc, 155 patients were employed, of whom 16.1% reported
absenteeism and 63% reported presenteeism while at work in the past week due to their health [33]. Of
those who missed work due to SSc, 24 workers reported an average of 32.9% time missed because of
SSc. Of those who experienced work impairment, 95 workers reported an average of 35% of work time
impaired due to SSc. Guillevin et al. adapted the WPAI specifically for SSc-associated digital ulceration,
and determined that having more digital ulcerations resulted in more absenteeism and presenteeism.
Overall, in 2327 patients, 36.7% of patients experienced work impairment due to digital ulcers [34].
Three other studies used different formal measurement tools to evaluate work disability.
Sandqvist et al. utilized the work ability index (WAI) in 57 patients with SSc. The WAI is a self-
administered questionnaire comprising questions around current ability, work ability in relation
to the physical and mental demands of the job, the worker's health status, and psychological
resources [28,35]. The WAI ranges from 7 to 49 points, with higher scores indicating better work
ability. Work ability can be categorized into the following levels: poor (scores 7e27), moderate
(scores 28e36), good (scores 37e43), and excellent (scores 44e49). Sandqvist et al. found 13
(23%) had good/excellent WAI, 15 (26%) had moderate WAI, and 20 (35%) had poor WAI. Patients
with good/excellent WAI perceived milder symptoms (pain, fatigue, and hand function),

3
 Table 1

J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson

Summary of studies that report employment status in individuals with Systemic Sclerosis.

Reference Sample Mean Aged Female, n (%) Mean Disease Diffuse SSc, n Employment/Work Employment Status, n (%) Work Loss Definition
Size (years, SD) Durationd (%) Disability as a
FT PT UE
(years, SD) Primary Outcome

Baker, 2012, 36 47.9 (9.5) 22 (81) 10.2 (7.64) 14 (58) No 19 (70) 7 (26) 1 (4) Not provided
USA [65]
Bassel, 2011, 464 55.8 (12.9) 464 (88) 10.9 (9.4) 59 (13) No 146 (31) NR Not provided
Canada[66]
Benrud-Larson, 142 52.1 (13.7) 129 (91) NR 50 (35) No 32% 29% unemployed, Employment was
2002, USA 21% retired defined as working
[67] outside of the home
Berezne, 2011, 189 54.1 (13.3) 164 (87) 9.3 (8.4) 78 (44) Yes 42 (37) 23 (20) 46 (40) Patients were asked
France[29] to describe their
employment status as
employed, retired,
homemaker, student,
disabled, on sick
leave, or looking for a
job
Buck, 2010, 62 53.0 (11.7) 54 (87) 6.0 (6.4) 30 (48) No 43 (69) N/A NR Not provided
USA [68]
4

Chen, 2008, 40 56.3 (11.9) 32 (80) 4.3 (3.1) 28 (70) No 24 (60) NR Employment defined

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

Chinaa [69] as receiving a form of
payment
Chevreul, 2015, 147 53.8 (11.7) 133 (91) 11.5 (10.1) NR No 52 (35) NR Questionnaire asked
France[49] about early
retirement but data
not provided
Decuman, 84 47.8 (8.9) 64 (76) Median: 56.5 12 (14) Yes 38 (45) 46 (55) Unemployment
2012, months classified as
Belgium[70] homemakers, those
unemployed,
students, on early
retirement, and on
sick leave
Guillevin, 2013, 2327 54.5 1954 (84) NR 914 (40) No 784 (34) NR Employment includes
Franceb [34] those who are self-
employed
Hudson, 2009, 643 48.4 (9.4) in 83% in 9.0 (7.7) in 42% in Yes 232 (36) NR Employment
Canada[43] working, 50.2 working, 84% working, 11.0 working, 59% classified as full time,
(8.2) in work in work (8.6) in work in work part time, or self-
disabled disabled disabled disabled employed.
 J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson
Unemployed
individuals, those
reported as retired,
students,
homemakers, and
individuals >65 years
old were excluded.
Jetha, 2019, 110 49 (12.9) 84 (77) 9.3 (7.2) NR Yes 84 (77) 26 (23) NR Only employed
Canada [31] participants included.
Full time
characterized as >35
h/week
Kallen, 2010, 62 53.4 (12.5) 54 (87) 8.0 (5.8) 39 (63) No 20 (33) 7 (12) Homemaker,
USA[71] students, and
patients on disability
were categorized
separately from those
considered employed
or unemployed.
Unemployment
included retirements.
Lopez-Bastida, 147 45.0 (14.1) 146 (85) NR NR No 55 (40) 11 (8) unemployed, Not provided
5

2014, Spain 19 (14) retired

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

[52]
Mansour, 2013, 1145 55.4 (12.1) 986 (86) 11 (9.5) 396 (38) No 432 (47) 52 (6) Employment
Canadab [40] classified as
employed (working),
Disabled/sick leave,
and unemployed
Matsuura, 50 59.6 (11.2) 41 (82) 13.6 20 (40) No 24 (48) NR Not provided
2003, Japan
[72]
Mau, 2005, 802 47.0 (10.0) 667 (83) <5 years: 361 NR Yes SERe in women NR Standardized
Germany[26] (48) with SSc: 0.39-0.90 Employment Ratios
6-10 years: 212 SER in men (SER) calculated as
(28%) with SSc: 0.82-0.94 ratios of observed
>10 years: 186 versus expected
(25%) cases. Results were
split by gender
McCormick, 42 46.3 37 (88) 13 NR Yes 14 (56) 8 (32) Defined as employed
2018, Canada for pay.
[73] Unemployment
defined as not
employed due to
health
(continued on next page)
 Table 1 (continued )

J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson

Reference Sample Mean Aged Female, n (%) Mean Disease Diffuse SSc, n Employment/Work Employment Status, n (%) Work Loss Definition
Size (years, SD) Durationd (%) Disability as a
FT PT UE
(years, SD) Primary Outcome

Medsger, 1978, 489 N/A Males only NR NR No NR 10 (2) Occupation

USA [74] determined by chart
review
Minier, 2010, 80 57.4 72 (90) 6.2 (6.6) 20 (25) Yes 7 (8) 2 (3) NR Not provided
Hungary [50]
Morgan, 2017, 1009 42.4 843 (84) 9.9 (8.7) 566 (57) No 297 (43) 77 (11) Not provided
USA[75]
Morrisroe, 802 50.4 in Employed: 538 Employed: 9.6 Employed: Yes 642 (87) 160 (20) Not provided. Self-
2016, employed, 51.9 (84), (9), 163 (25), report questionnaire
Australiac in unemployed Unemployed: Unemployed: Unemployed:
[41] 132 (83) 11.1 (10.9) 61 (38)
Morrisroe, 476 56.6 138 (87) 9.1 (8.2) 37 (24) Yes 155 (55) NR Not provided. Self-
2017, report questionnaire
Australia[33]
Morrisroe, 476 64.2 398 (87) 11.2 (10.3) 100 (22) Yes 133 (55) 108 (45) Employment in
2018, individuals <65 years
Australia [45] old. Self-report
questionnaire
6

Moser, 1993, 94 55.0 82 (88) 8 (7) NR No 24 (56) NR Not provided. Self-

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

USA [76] report questionnaire
Nguyen, 2010, 87 48.6 72 (83) 8.1 (6.4) 30 (35) Yes 24 (28) 10 (12) NR Not provided. Self-
France [27] report questionnaire
Poole, 2016, 33 47.3 82% 9.7 (7.0) 58% Yes 72% 28% NR Self-report
USA ([55]) questionnaire. Full
time defined as >¼ 35
h/week, Part time less
than 35 hours/week
Poole, 2017, 64 48.9 80% 11.4 (11) NR No 33% NR NR
USA [77]
Sandqvist, 36 Median: 52 100% (Women Median: 9 (2- Limited SSc Yes 16 (44) NR
2005, only) 44 years) only
Sweden [78]
Sandqvist, 44 Median: 52 100% (Women Median: 8 (2- Limited SSc Yes 21 (48) NR
2008, only) 44 years) only
Sweden [44]
Sandqvist, 57 58.0 100% (Women Median: 14 (9- 10% Yes 36 (63) 20 (35)
2010, only) 19 years)
Sweden [28]
 J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson
Schouffoer, 64 55.3 50 (78) 8.5 (6.4) 34 (53) No 22 (34) 42 (66) Employment defined
2011, as paid employment,
Netherlands self-report
[79] questionnaire
Sharif, 2011, 284 48.7 237 (84) 2.5 (1.6) 162 (57) Yes 111 (3) 14 (5) NR Part-time workers
USAb [42] needed to work part-
time but not
attributable to SSc
concerns
Singh, 2012, 162 51.8 131 (81) 7.6 (8.2) 67 (41) Yes 60 (37) 99 (61) Employment defined
USA [36] as working outside
the home
Tedeschini, 78 53.7 72 (92) 7.7 (6.4) 13 (17) Yes Those without the NR
2014, Italy impact of skin
[80] disfigurement employed:
35 (54%), Those with the
impact of skin
disfigurement,
employed: 3 (23%)
Willems, 2014, 537 Median: 55 in France: 96%, France: Median France: 35%, No France: 34%, Netherlands: NR Described as paid
Netherlands France, 60 in Netherlands: 8 (4-15), Netherlands: 21%, Spain: 53%, employment
[81] Netherlands, 49 84%, Spain: Netherlands 9 32.3%, Spain: Switzerland: 42%,
7

in Spain, 56.6 in 80%, (4-15), Spain 8 27.9%, and UK: 37%

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

Switzerland, Switzerland: (3-13), and Switzerland:
and 37.5 in UK 88%, and UK: Switzerland 5 62%, and UK:
90% (3-11), UK: 6 37.2%
(3-12)

FT: Full Time, PT: Part Time, UE: Unemployed

NR: Not Reported
UK: United Kingdom USA: United States of America
All studies are cross-sectional in design except for the following noted:
a
Chen et al. is a case-control study
b
Guillevin et al., Mansour et al., Sharif et al. are prospective cohort studies
c
Morrisroe et al. is a retrospective cohort study
d
Age and Disease duration reported in mean (SD), unless specified
e
SER: Standardized Employment Ratios
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

exhibited better competence at the workplace, and had the possibility of adapting to their work
environment. Singh et al. utilized a variation of the Work Productivity Survey (WPS), which was
initially developed for RA, by modifying the word “arthritis” with “scleroderma” [36]. The WPS
measures the impact of the disease on the productivity of employment work, household work,
and daily activities over a recall period of 1 month. The tool provides the number of days of work
absence, the number of days with reduced work productivity, and the degree of interference on
work productivity. Of the 60 patients employed outside of the home, patients reported absen-
teeism of 2.6 days per month, and had 2.5 days per month of productivity reduced by half. Jetha
et al. included the Workplace Activity Limitations Scale (WALS) to evaluate the difference in
workplace activity according to SSc workers with and without workplace support [31]. The WALS
is an 11-item self-reported questionnaire designed to ask questions around work-related activity
limitations, including ambulating to, from, and at the workplace; sitting/standing; lifting;
reaching; and meeting job demands [37]. Responses ranged from 0 to 33, with higher scores
reflecting more difficulty. Jetha et al. determined in 110 SSc workers that those with workplace
support had significantly lower WALS scores than those with unmet needs [31].

Risk factors associated with work disability IN SSc

Table 3 and Fig. 1 summarize the demographic, disease-related, psychosocial, and work-related risk
factors that were significantly associated with higher work disability in the SSc population. We specify
whether the reported risk factors impacted work loss or at-work disability. Most commonly reported
risk factors for both work loss and at-work disability across several studies included lower education
[25,36,38e42], longer disease duration [29,39,43], diffuse skin involvement/increasing skin score
[28,39,41,43e45], worse functional status particularly related to the hands (including contractures and
ulcers) [27e29,41,42,44], and higher depression scores [27,36,46]. The majority of studies evaluated the
association of various demographic and disease-related risk factors, while work-related risk factors
were the least explored, despite the recognition of their importance in studies of other various
rheumatic conditions [47] (see Table 4).
Only six studies explored the impact of the work context, of which 5 of 6 studies evaluated the
impact of work context risk factors on at-work disability [28,31,38,39,41,44]. Reported work-
related factors that influence at-work disability may include the type of work, physical/psy-
chological demands of the work, element of job strain or control, job satisfaction, disease
disclosure, and the availability and access to job modifications and accommodations. Sandqvist
et al. conducted a series of studies, which evaluated the role of work context factors on worker
ability and productivity, such as duration at the workplace, type of profession, perceived work
stress, physical/cognitive demands, empowerment in the workplace, social support at the
workplace, and job accommodations [28,38,44]. Overall, individuals with better work ability
scores had more mentally demanding jobs, had the opportunity to easily adapt and accommodate
work needs, had support from colleagues, and felt more empowered in the workplace [28].
Furthermore, Jetha et al. determined that workers with SSc who had more workplace activity
limitations, but who were supported in the workplace (received job accommodations (i.e., special
equipment), job modifications (i.e., change in job tasks or flextime), or benefits (i.e., wellness
programs or extended health benefits)) had lower productivity losses, including absenteeism,
presenteeism, and job disruptions [31].

Conceptual framework of work disability in SSc

A framework for unifying the concepts that comprise work disability in SSc described previously is
presented in Fig. 2. Facets of work productivity may begin after diagnosis. It may progress from pre-
senteeism to absenteeism, which evolves to partial work loss and/or complete work loss. This may
occur anytime in the disease course and can be facilitated by disease, psychosocial, and work context
factors.

8
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

Table 2
Studies reporting At-Work Disability or Work-Related Disability in Systemic Sclerosis.

Measure Studies and Outcomes

VAS of work productivity or
impairment
Work Disabled (Y/N)
Disability Pension or Sick Leave
Duration
Work Productivity and Activity
Impairment (WPAI)
Work impairment over 7 days
Work Ability Index (WAI)
Score from 7 to 49
Work Productivity Survey
Productivity over 1 month
Professional Difficulties
Work transitions
Job Disruptions
Workplace Activity Limitations Scale
Berezne et al (n¼189) [29]: 0e10 (Major decrease) VAS for work productivity in
the previous month due to SSc. Mean VAS: 3.4
Matucci-Cerinic et al (n¼1459) [82]: 0e10 VAS (expressed as %) for decreased
work productivity in the previous month due to SSc digital ulcers (DU). Mean
VAS: 17.6% (No DU), 12.3% (Episodic DU), 33.4% (Recurrent DU), and 48.3%
(Chronic DU)
Average work disability percentage of 11 studies (n¼44 to 1009): 40%
Nguyen et al (n¼87) [27]: Self-reported duration of receiving a disability
pension. Of the 31 patients with disability pension and mean (SD) duration of
disability pension (years): 5.5 (6.9)
Sandqvist et al (n¼32) [28]: WD calculated net days of sick leave and disability
pension. Mean (SD) WD in limited SSc in 3 years: 103 (130), in diffuse SSc: 190
(151)
Guillevin et al (n¼2327) [34]: Adapted WPAI to DU. Overall work impairment
(expressed as a %) was calculated as the sum of work time missed and lost
productivity at work. Overall work impairment: 36.7%
Jetha et al (n¼110) [31]: % of cohort with >1 day of absenteeism: 50%. Those
with poor workplace support had a significantly higher proportion of patients
with absenteeism. Mean Presenteeism (0-10): 2.8 (2.7). Those with poor
workplace support had a significantly higher presenteeism.
McCormick et al (n¼42) [18]: Absenteeism: not reported and Presenteeism: 10
(83%)
Morrisroe et al (n¼476) [45]: Mean absenteeism (% of work time missed): 5.3%,
Mean % of work time missed in those who missed work: 32.9% and Mean
presenteeism (% work time impaired): 22.2%, Mean % of work time impaired in
those who experience work impairment: 35.1%
Sandqvist et al (n¼57) [36]: Median (IQR) WAI: 32 (16.8,37.0)
Singh et al (n¼162) [36]: Mean (SD) absenteeism (days missed): 2.6 (6.3), Mean
(SD) presenteeism (days with productivity reduced >50%): 2.5 (6.1)
Peres et al (n¼104) [30]: Professional difficulties due to SSc: 65 (62.5%)
Berezne et al (n¼189) [29]: 39 (34.5%) had changed their profession since SSc
Decuman et al (n¼84) [56]: 47 (56%) had a health-related work transition
Nguyen et al (n¼87) [27]: 27(31%) had occupational changes
Jetha et al (n¼110) [31]: Mean job disruptions (0e7): 1.6 (1.7). Those with poor
workplace support had significantly higher job disruptions.
Jetha et al (n¼110) [31]: Mean workplace activity limitation score of 8.1 (5.7,
range 0e36). Most difficult workplace acts included working with hands,
crouching, bending/kneeling, lifting, and carrying/moving objects.

Economic impact of work disability in SSc

Ten studies, which included SSc cohorts from 11 countries, quantified the substantial economic
impact attributable to indirect costs of having the disease (Table 3). All studies were cross-sectional
study designs. Work or productivity losses were frequently estimated through self-reported ques-
tionnaires and an economic evaluation was most approached through the human capital approach
utilizing the societal perspective. The human capital approach involves determining an individual's
rate of pay over a period of time and then the economic impact is determined by calculating the
amount of income that would be forgone because of ill health [48]. Definitions of indirect costs varied
from the study and included hours lost from productivity, early retirement, or the duration of short-
term or long-term disability leaves.
Of the six studies that evaluated both direct and indirect total costs, four studies reported higher
total indirect costs relative to total direct costs [19,49e51]. Total direct costs include direct
healthcare costs (this includes drugs, hospitalizations, emergency room visits, and visits to medical
specialists), direct non-healthcare formal costs (number of hours of care by professional caregivers),
and direct non-healthcare informal costs (number of hours of care by caregivers). Bernatsky et al.

9
 Table 3

J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson

Risk factors associated with work disability in systemic sclerosis.

Reference Sample Work Disability Outcome Risk Factors

Size (Work Loss and/or At-Work
Demographic Disease-Related Psychosocial Work Context
Disability)

Berezne, 2011, France([29]) 78 Work Loss Older Age Longer disease duration, worse More perceived individual
functional status/physical function handicap (MACTAR)
(KPS, HAQ, and SF-36), and worse
hand function (Cochin Hand
Function Scale)
Buck, 2010, USA( [68]) 62 Work Loss Less Self Efficacy (Arthritis
Self-Efficacy Scale)
Decuman, 2011, 84 At-Work Disability Lower education Higher skin score (modified Rodnan More manual workers than
Belgium([39]) level Skin Score), longer disease employees, reduced
duration, higher disease activity willingness to undergo
score, and more disability (HAQ-DI, training
SHAQ, SF-36)
Guillevin, 2013, 2327 Work loss and At-work More digital ulcers
France([34]) disability
Hudson, 2009, Canada([43]) 643 At-work disability Longer disease duration, diffuse
skin involvement, worse physician
global score, more pain, more
10

comorbidities (SCQ), worse physical

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

function (HAQ), and more fatigue
Jetha, 2019, Canada [31] 110 At-work disability Unmet workplace support
needs (Need of job
accommodations,
modifications, or benefits is
more than use)
Mansour, 2013, 1145 Work loss Lower education
Canada([40]) level
Mau, 2005, Germany([26]) 802 Work loss Female with a
longer disease
duration
Morrisroe, 2016, 1587 Work Loss Lower education Diffuse skin involvement, Physical work
Australia([41]) level Raynaud's, GI manifestations, joint
contractures, pulmonary
hypertension, sicca, digital
amputation, higher skin score,
more hand contractures, lower
6MWD, and worse physical
function (WHO functional class)
 J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson
Morrisroe, 2018, 476 Work Loss and At-work Older age Diffuse skin involvement, synovitis,
Australia([45]) disability small joint contractures of the hand,
and pulmonary hypertension
Nguyen, 2010, France([27]) 87 At-work disability Myalgia, worse functional status More depression (HAD)
(KPS), higher global disability
(HAQ), more hand disability (CHFS),
more mouth disability (MHISS)
Ouimet, 2008, Canada([25]) 61 At-work disability Lower education Higher disability (HAQ)
patients level
with SSc
Peres, 2017, France([30]) 104 At-work disability Arthralgias
Sandqvist, 2008, 44 At-work disability Higher skin score, more fatigue, Worse satisfaction with
Sweden([44]) more breathlessness, worse hand occupation and lower
function, and worse self-rated capacity to perform
health (SF-36) occupations
Sandqvist, 2010, 57 At-work disability More fatigue, more pain, more Lower life satisfaction Lower satisfaction with
Sweden([28]) stiffness, and more ulcers occupation, lower
empowerment in the
workplace (Spreitzer's
empowerment scale), less
mentally demanding, less
adaptations at the
11

workplace, less support

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

from others, and less
influence over outcomes at
work
Sandqvist, 2015, 32 At-work disability Lower education Less number of years at
Sweden([38]) level workplace, The use of sick
leave the month before
disease onset
Sharif, 2011, USA([42]) 284 Work loss Older age More cardiac involvement, small Worse mental function (SF-
Not Caucasian, Not joint contractures, lower lung 36 MCS), Less social
married, Lower function (Lower FVC, DLCO), GI support (ISEL), Poor Coping
education level involvement, more pain, more (IBQ) and more
dyspnea, more fatigue (Fatigue helplessness (AHI)
Severity Scale), worse functional
status/physical function (SF-36 PCS,
HAQ)
Singh, 2012, USA([36]) 162 At-work Disability Lower education Lower patient global assessment, More depression (CES-D-
level worse functional status (HAQ DI), 10)
more fatigue (FACIT-F)
Tedeschini, 2014, Italy( 78 Work loss More depression (BDI) and
[80]) more psychosocial impact
of disfigurement

Bolded are statistically significant in multivariable analyses.

J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

determined in 457 Canadian patients with SSc that the total cost was estimated at $18,453 per
patient, of which only 27% was attributable to direct costs. The remaining was related to the cost of
lost productivity related to unpaid labor and potential productivity related to paid labor [19]. Pa-
tients with diffuse disease had higher annual costs than limited patients with SSc. Lopez-Bastida
et al. conducted two studies evaluating the cost of SSc in Spain and in other European countries,
and showed variability in the proportion of direct and indirect costs according to country. In some
countries like Spain, the direct cost was higher than the indirect costs because of the incorporation
of direct non-healthcare expenses [52,53]. Most studies concluded that the costs associated with
SSc was substantial and that both direct non-healthcare costs and indirect costs were often
considerable yet hidden.
Three studies commented on the economic burden of SSc as compared to other rheumatic
diseases. Kawalec et al. evaluated the gross value per worker of Polish patients with SLE, SSc, and
sarcoidosis, and determined that patients with SLE and SSc had comparable work disability costs.
The cost of indefinite long-term disability per patient was the highest for SSc. Both SSc and SLE had
proportionally more costs attributed to long-term disability (defined as typically 18 months or
longer), than sarcoidosis where more costs were generated because of sick leave, which is the
shortest form of absenteeism [54]. Similarly, McCormick et al. also determined similar and higher
costs when comparing patients with SLE and SSc, and slightly lower work disability costs, albeit still
significant than adults without systemic autoimmune rheumatic diseases, in the Sjogren's syn-
drome population. Minier et al. compared SSc costs with matched patients with RA and PsA in
Hungary and reported higher total costs in the SSc population, although indirect costs between
patients with SSc and RA were comparable, but there appeared to be significantly more direct costs
associated with SSc [50].

Potential challenges at the workplace in individuals with SSc

Several qualitative studies explored the common challenges that people with SSc experience at the
workplace, including their overall experience with employment after SSc disease onset, barriers that
interfere with work productivity, along with protective factors that promote or influence their de-
cisions to maintain employment [55e59]. Fig. 3 illustrates the commonly reported barriers and fa-
cilitators to employment according to the qualitative studies.
Several studies highlighted the negative impact of physical symptoms attributable to SSc, in
particular, fatigue, limited mobility, and intolerance to cold temperatures. Mendelson et al. described
that their entire study population of 32 employed SSc individuals complained about ubiquitous fatigue
that not only impacts their ability at work, but their inability to have enough energy to maintain
balance and prioritize other aspects of their life, including routine household and life activities [57].
Sandqvist et al. commented similarly that participants often mentioned the importance of utilizing
their energy wisely, along with the importance of having additional support in the home [58].
Furthermore, given the increased frequency of Raynaud's phenomenon in this population, finding
strategies to maintain warmth on their way to work or within the workplace was an important
consideration.
Protective factors that facilitate employment included having various forms of accommodations,
including within the job changes (i.e., change in work hours), major job changes (i.e., changing to a
different position in the workplace, which could be performed with better ability), work environment
changes (i.e., the use of assistive devices), or ability to get time off (i.e., paid sick leave). Participants
sampled from the qualitative interviews generally reported a positive experience with the disclosure of
illness to employers and coworkers, which suggest disclosure improved transparency at the workplace
and improved their ability to access accommodations. However, Sumpton et al. reported in 30 adult
patients with SSc that the lack of knowledge and invisibility of the illness as perceived by the public as
well as some medical professionals is a source of difficulty [60]. Similarly, other general rheumatology
studies suggest that healthcare professionals may not be as proactive in asking about the impact of
disease on work or recognize the impact of disease on occupational goals [61,62]. This may limit the
opportunity for providers to initiate appropriate referrals for various therapies that may facilitate work,

12
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667
Fig. 1. Risk factors associated with work disability in systemic sclerosis.
13
 Table 4

J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson

Work disability cost outcomes.

Reference, Year, Sample Size Definitions Currency Direct Costs Indirect Costs (Related to Work
Country Disability)

Bernatsky et al., 457 Modification of the Standard Health Assessment 2007 CDN
2009, Canada Questionnaire. Productivity losses due to
[19] disease arise from inabilities to perform work in
the labor market (paid labor).
Chevreul et al., 147 Cost analysis using bottoms-up approach. Costs 2012 Euros Annual Direct cost per patient: 8452 Total Indirect Costs: 10,526 EUR
2015, France [49] were divided into direct healthcare costs, direct
non-healthcare formal costs, direct non-
healthcare informal costs, indirect costs.
Indirect costs are described as loss of
productivity linked to work absence and early
retirement
Kawalec et al., 500 Human Capital Approach using gross domestic 2012 Euros NR
2015, Poland [54] product per capita, gross value added per
worker and gross income per worker in 2012.
Analyzed according to sick leave, short-term
work disability, long-term work disability (fixed
14
Average direct cost per patient: $5038 Average value of lost productivity
CAD (95% CI: $4400e5676)
EUR (14,480)
Direct non-healthcare formal cost:
1606 EUR (8663)
Direct non-healthcare informal cost:
1875 EUR (6982)
related to labor: $5345 CAD (95% CI:
$4598e6092)
Average of lost productivity related to
unpaid labor: $8070 CAD (95% CI: 7167
e8973)
(15,145)
Productivity loss: 2403 EUR (7990)
Early retirement: 8123 EUR (14,312)
Sick leave (Cost per patient): 842 EUR
Short-term work disability: 216 EUR
Long-term work disability (Fixed
period): 2025 EUR
Long-term work disability (Unlimited

period), long-term work disability (Unlimited period): 1454 EUR

Best Practice & Research Clinical Rheumatology 35 (2021) 101667

period), and total costs over a 1-year period
Lopez-Bastida et al., 589 Bottoms-up approach. Costs were split
2016, Spain [53] according to direct healthcare costs, direct non-
healthcare costs (formal and informal care), and
labor productivity losses. Labor productivity
loss defined as sick leave or early retirement,
estimated for the year 2011 using human
capital-based approach
Lopez-Bastida et al., 147 Average annual cost per patient. Labor
2014, Spain [52] productivity losses determined by determining
reductions in working time (temporary and
permanent sick leave or early retirement)
McCormick et al., 42 Excess hours of paid and unpaid productivity
2018, Canada loss as associated costs. Excess refers to
[83] adjusted differences in lost productive time and
monetary value between the diseased group
and the general population. Paid productivity
loss split into absenteeism and presenteeism.
Total: 4537 EUR
2012 Euros Direct cost (Range from 7 countries): Labor productivity cost:
1413 EUR to 17,300 EUR 1701 EUR to 14,444 EUR
Direct non-healthcare cost: 1875 EUR
to 4684
2011 Euros Direct healthcare cost: 8235 EUR Labor productivity cost per patient:
Direct non-healthcare cost: 5503 EUR 7303 EUR
2015 CDN NR Average weekly costs for time lost
from paid work: $158/week. When
including presenteeism, mean costs
were $255/week. Annual productivity
loss (from paid work) $18,236;
additional $3582 lost productivity costs
each year over and above their control
group

Minier et al., 2010, 80 Average annual cost per patient. Costs were
Hungary [50] split according to direct medical, direct non-
medical, and indirect costs. Costs of sick leave,
part-time job, and full disability due to SSc were
calculated using the human capital approach
method.
Morrisroe et al., 476 Weekly and annual costs were estimated for
2018, Australia unemployment and lost productivity from a
[45] societal perspective. Annual costs were
calculated by multiplying the productivity loss
(in hours) by the average hourly earnings,
which was calculated by dividing the average
weekly earnings by the average working hours
per week.
Singh et al., 2012, 162 Loss of productivity at work was translated to
USA [36] USD per month. Calculated estimated earnings
lost due to work missed per month. Referred to
US median weekly earnings of employees aged
25 years and older by sex and the level of
education
Wilson et al., 1997, 183 Costs were split to direct and indirect costs
USA [51] (ARAMIS Data) annually. Indirect costs were calculated using
15
the human capital approach. Work loss:
calculated based on 2.1 work loss days/year. Job
loss: calculated based on 10% of unemployment
due to SSc, Disability: calculated based on 18.2%
being on disability, sick leave: calculated based
on 0.6% being on sick leave. To estimate 1994
productivity losses, 1992 employment and
earnings were inflated by 1.069
CDN: Canadian Dollars, USD: US Dollars, and AUD: Australian Dollars.
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson
2006 Euros Total direct cost: 3300 Euros Total indirect costs: 5390 Euros
Total direct non-medical cost: 929
Euros
2015 AUD NR Annual cost from lost earnings:
AUD$77963.60 per patient (Full-time
workers), AUD$59571.20 per patient
(Both full- and part-time workers).
For employed patients, annual costs
from lost productivity per patient:
AUD$8024.20.
2010 USD NR Mean Income loss in employed
patient with missed work: $897 (range
$127e2792) per month. Patients with
SSc-related disability (39.4%) had a
mean income loss of $3576.90 (SD
$1302.80) per month.
1994 USD Total direct cost: 462 million Total indirect cost: 998 million
Work loss (In employed patients):
28.04 million/year
Best Practice & Research Clinical Rheumatology 35 (2021) 101667
Job loss: 164.7 million/year
Disability: 599.4 million/year
Sick leave: 13.6 million/year
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667
Fig. 2. Conceptual model of work disability in systemic sclerosis.
16
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667
Fig. 3. Barriers and facilitators to employment and worker productivity.
17
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

such as occupational therapy, physiotherapy, vocational assessments, or vocational rehabilitation

programs.

Vocational rehabilitation interventions in SSc

Vocational rehabilitation is one approach to address job loss associated with chronic diseases and to
optimize job retention. It commonly refers to a rehabilitation program with defined interventions that
are aimed at reintegrating patients with chronic disease back into the work force (return to work) or
sustaining work force participation [63]. Interventions may be variable but often include individual or
group training sessions that are focused on increasing knowledge (about the disease and legal rights),
gaining an understanding of work-related problems and barriers, increasing activities that result in job
accommodations, and increasing feelings of control and self-efficacy at the workplace [64].
No studies have evaluated the potential role or impact of vocational rehabilitation in the SSc
population. De Buck et al. performed a systematic review of vocational rehabilitation programs per-
formed in patients with various rheumatic diseases, most commonly in RA, and identified six studies,
which evaluated the effectiveness of a multidisciplinary intervention over a period of 2e84 months
[64]. Interventions, follow-up duration, and methods to measure effectiveness of the intervention were
often highly variable. Five studies reported a positive change in job status following a vocational
rehabilitation program at a success rate, which ranges from 52% to 69%. However, only 2 studies re-
ported a greater than 6-month follow-up, of which one study reported a loss of effectiveness in the
program by a 1-year follow-up. Similarly, Varekamp et al. performed a systematic review of vocational
rehabilitation programs in various chronic diseases such as chronic kidney failure, multiple sclerosis,
and inflammatory bowel disease and found that while methodological rigor is often lacking, there
appears to be some evidence to support programs that train patients to request work accommodations
and improve feelings of self-efficacy and self-confidence when dealing with work [64]. Thus, more
research is needed on whether a formal vocational rehabilitation program is effective in this patient
population.

Summary

Work disability is an important concern in systemic sclerosis, where work loss, productivity loss,
and economic impact is substantial. Work disability is multifaceted and can be explored by
measuring several outcomes, including work loss as well as work activity limitations and worker
productivity losses. Our conceptual model of work disability in SSc highlights that work disability
may begin after diagnosis, can take many forms, and can evolve. We emphasize the importance of
not only disease-related factors that may need to be optimized to prevent or reduce work disability
but other risk factors, particularly work-related risk factors. Vocational rehabilitation programs are
one possible strategy considered in various chronic disease populations as a method to foster job
retention. Research is needed to determine whether these programs are effective in the SSc pop-
ulation. We recommend that clinicians should proactively evaluate the impact of the disease on
occupational goals and proactively make appropriate referrals for therapies that may foster work
retention.
SF-36 ¼ Short Form-36, SHAQ ¼ Scleroderma Health Assessment Questionnaire, HAQ QI ¼ Health
Assessment Questionnaire Disability Index, MACTAR ¼ McMaster-Toronto Arthritis Patient Preference
Disability Questionnaire, SCQ ¼ Self-Administered Comorbidity Questionnaire, 6MWD ¼ 6 min walk
distance, WHO ¼ World Health Organization, KPS ¼ Karnofsky Performance Status, CHFS ¼ Cochin
Hand Function Scale, MHISS ¼ Mouth Handicap in SSc Scale, HAD¼ Hospital Anxiety and Depression
Scale, FVC ¼ Forced vital capacity, DLCO ¼ Diffuse capacity of the lung for carbon monoxide, ISEL ¼
Interpersonal Support Evaluation List, IBQ ¼ Illness Behavior Questionnaire, AHI ¼ Arthritis Help-
lessness Index, FACIT-F ¼ Functional Assessment of Chronic Illness Therapy e Fatigue Scale, and BDI ¼
Beck Depression Inventory.

18
J.J.Y. Lee, M.A.M. Gignac and S.R. Johnson Best Practice & Research Clinical Rheumatology 35 (2021) 101667

Practice Points

" Work disability is highly prevalent in the SSc population. In comparison to other chronic
rheumatic diseases, SSc has a relatively high work disability burden. This burden is reflected
in the higher proportion of patients with work loss, the significant indirect costs reflected in
economic analyses, and in the experiences provided by SSc patients within qualitative
studies.
" Risk factors associated with higher rates of work disability include lower education, longer
disease duration, diffuse skin involvement/increasing skin scores, worse functional status
particularly related to the hands (including contractures and ulcers), and higher depression
scores. Few work-related risk factors have been explored, but possible risk factors in the work
place may include a physically demanding job, lower satisfaction/empowerment at the
workplace, less support from colleagues, and limited accommodations.
" Protective factors that may facilitate employment or productivity may include job satisfaction
with better support at the workplace, increased workplace accommodations (both in tasks
and in schedule), and the ability to transition to a job within the same workplace that may
require less physical stress. Commonly reported barriers to employment and work produc-
tivity include ubiquitous fatigue as well as cold environments.
" Medical practitioners should proactively discuss the impact of SSc on employment and on
occupational goals. If applicable, providers should initiate appropriate referrals for therapies
that may facilitate work retention such as occupational therapy, physiotherapy, psycho-
therapy, vocational assessments, and vocational rehabilitation programs.

Research Agenda

" Most research in SSc has focused on work loss as opposed to the evaluation of worker
productivity outcomes, such as absenteeism and presenteeism. Studies are needed where
established productivity measurement tools are used so that it can provide a source of
comparison between different patient population groups and other diseases. Research is
needed to ensure that the productivity measurement tools are valid, reliable, and are sen-
sitive to change in this particular patient population.
" Research is needed to determine non-disease related risk factors associated with work
disability in SSc. In particular, more studies are needed to evaluate the impact of work
context related factors on work disability.

Funding

Dr. Johnson is supported by the Gurmej Kaur Dhanda Scleroderma Research Award, Scleroderma
Association of British Columbia and the Canadian Institutes of Health Research. Dr. Lee was supported
by the Ministry of Research and Innovation; Ministry of Economic Development, Trade and Employ-
ment. Early Researcher Award, and the Canadian Institute of Health Research (CIHR) Canada Graduate
Scholarship e Masters (CGS-M) Award.

Declaration of Competing Interest

None to disclose.

References

[1] Denton CP, Khanna D. Systemic sclerosis. Lancet (London, England) 2017;390:1685e99.

19