DOI: 10.1002/msc.

1185

LITERATURE REVIEW

Aerobic and resistance exercise in systemic sclerosis: State of

the art
Natália Cristina de Oliveira1 | Leslie Andrews Portes1 | Henrik Pettersson2 |

Helene Alexanderson3 | Carina Boström3

1
Research Group in Physical Exercise, Lifestyle
and Health Promotion. Health Promotion Abstract
Master Program, UNASP ‐ Adventist Introduction: Patients with systemic sclerosis (SSc) experience reduced exercise capacity and
University of São Paulo, São Paulo, SP, Brazil
2
muscle strength compared with healthy subjects. There are also indications of reduced levels of
Unit of Rheumatology, Department of
Medicine Solna, Karolinska Institutet, and,
physical activity.
Functional Area Occupational Therapy & Objective: To present the current knowledge of physical exercise in SSc.
Physiotherapy, Allied Health Professionals
Function, Karolinska University Hospital, and, Results: Most studies presently available [three case studies, one single subject experimental
Department of Neurobiology, Care Sciences design, one study comparing patients with healthy controls, one quasi experimental design (pre–
and Society, Division of Physiotherapy,
post), two clinical trials and two random controlled trials] have included small samples of patients,
Karolinska Institutet, Stockholm, Sweden
3 mostly composed of patients with and without pulmonary involvement. It seems that patients
Department of Neurobiology, Care Sciences
and Society, Division of Physiotherapy, with SSc without pulmonary involvement are able to perform and benefit from aerobic exercises
Karolinska Institutet, and, Functional Area of at least moderate intensity. Exercise tolerance, aerobic capacity, walking distance, muscle
Occupational Therapy & Physiotherapy, Allied
strength and muscle function as well as health‐related quality of life (HRQL) have been found
Health Professionals Function, Karolinska
University Hospital, Stockholm, Sweden to be improved after participation in programmes including aerobic exercise and aerobic exercise
Correspondence combined with resistance exercises. Improvements seem to be only partially retained at follow
Natália Cristina de Oliveira, Research Group in up. Patients with pulmonary involvement may also experience improved muscle strength, physi-
Physical Exercise, Lifestyle and Health
cal and aerobic capacity, as well as HRQL following exercise.
Promotion. Health Promotion Master Program,
UNASP – Adventist University of São Paulo, Conclusions: Patients with SSc without pulmonary involvement can be recommended to be
Estrada de Itapecerica, 5859, São Paulo, SP, as physically active as the general population. Patients with mild pulmonary involvement can be
Brazil. 05858‐001
Email: natalia.silva@ucb.org.br
recommended to be physically active by engaging in exercises of moderate intensity and to
participate in moderate‐load resistance exercises. Health professionals should inform patients
with SSc about the importance of physical activity and avoidance of a sedentary lifestyle.

KEY W ORDS

aerobic exercise, exercise, exercise therapy, rehabilitation, resistance training, systemic

sclerosis

1 | I N T RO D U CT I O N
Systemic sclerosis (SSc) is a rare disease of unknown aetiology with
vasculopathy, fibrosis and autoimmunity (Hinchcliff & Varga, 2008;
Walker et al., 2007). The disease often causes symptoms which not
only affect the skin but also joints, muscles and important internal
organs, such as the heart and lungs. Traditionally, SSc has been sepa-
rated into two major subgroups, diffuse cutaneous SSc (dcSSc) and
the more common form, limited cutaneous SSc (lcSSc) based on the
major differences in skin involvement and the type of organ systems
affected. dcSSc is associated with skin tautness in the proximal parts
of the body (arms, trunk and/or thighs) and interstitial lung disease
(ILD, thickening of the interstitium usually due to autoimmunity or
inflammation), cardiac fibrosis and a worse prognosis. Patients with
lcSSc are characterized by skin tautness of the distal parts of the
extremities and head and have an overall better prognosis, even
though they are at a higher risk of developing pulmonary arterial
hypertension (PAH) due to vasculopathy (Hinchcliff & Varga, 2008;
LeRoy & Medsger, 2001; Matucci‐Cerinic, Kahaleh, & Wigley, 2013;
Steen, 2008; Walker et al., 2007).
Diagnostic criteria for SSc include skin abnormalities (puffy fingers,
swollen digits turning into sclerodactily), vascular alterations

Musculoskeletal Care. 2017;1–8. wileyonlinelibrary.com/journal/msc Copyright © 2017 John Wiley & Sons, Ltd. 1
2 DE OLIVEIRA ET
(Raynaud’s phenomenon, SSc pattern in capillaroscopy) and laboratory
findings such as antinuclear, anticentromere and antitopoisomerase‐I
antibodies (Avouac et al., 2011).
The incidence and prevalence of SSc and its subgroups varies
greatly, depending on ethnicity and world regions, with an average
incidence per million of between 4 and 43 individuals, and a prevalence
between 70 and 340 per million has been reported (Barnes & Mayes,
2012). Female gender predominates with a general female/male ratio
of 6 : 1 (Walker et al., 2007).
Pulmonary involvement such as lung fibrosis and PAH is common:
approximately 35–50% of patients with SSc have lung fibrosis, and
over 20% have PAH (Walker et al., 2007). The prevalence of traditional
cardiovascular risk factors in SSc has been reported to be reduced or
similar in comparison with controls (Ali, Ng, & Low, 2015). However,
SSc has featured as an independent risk factor for coronary artery dis-
ease in addition to age, male gender, hypercholesterolaemia, hyperten-
sion, diabetes and SSc‐related factors (including PAH, renal
involvement and disease duration) (Ali et al., 2015).
Also, the prevalence of premature cardiovascular disease is higher
in patients with SSc than in controls (Nordin et al., 2013). Further,
patients have an increased risk of acute myocardial infarction (Chu
et al., 2013), deep vein thrombosis and pulmonary thromboembolism
(Chung et al., 2014), as well as an increased risk of comorbidities such
as osteoporosis (Omair, Pagnoux, McDonald‐Blumer, & Johnson,
2013), anxiety and depression (Nguyen et al., 2014).
Involvement of facial and oral tissues is a typical feature in patients
with SSc, which may cause a reduction of mouth width and furrows.
These represent a great concern for patients, especially those who
have a longer disease duration, as they deal with progressive and
limiting impairments (Paquette & Falanga, 2003). In addition to
skin deformities and disfigurement, pain, fatigue and dyspnoea are
features related to a reduced health‐related quality of life (HRQL) in
patients with SSc (Almeida, Almeida, & Vasconcelos, 2015; Khanna
et al., 2005; Paquette & Falanga, 2003; Stamm et al., 2011) when
compared with a healthy population (Khanna et al., 2005). Patients
with dcSSc also have lower HRQL scores than those with lcSSc
(Khanna et al., 2005).
Patients with SSc, especially those with the dcSSc, may have joint
involvement (Avouac et al., 2010) and present enthesopathy (Kilic, Kilic,
Akgul, & Ozgocmen, 2015). Proximal muscle weakness in the shoulder
and hip–pelvic region is a common feature (Akesson, Fiori, Krieg, van
den Hoogen, & Seibold, 2003; Clements et al., 1978). Lima et al.
(2015) have shown that quadriceps strength is reduced and that fatigue
in this muscle group is increased. Several studies have also found that
patients with SSc have a reduced overall exercise capacity when com-
pared with healthy individuals and general population (Blom‐Bülow,
Jonson, & Bauer, 1983; Cuomo et al., 2010; de Oliveira et al., 2007;
Morelli et al., 2000; Pettersson et al., 2017; Schwaiblmair, Behr, &
Fruhmann, 1996; Sudduth et al., 1993; Villalba et al., 2007). Many
patients have reduced aerobic capacity (Rosato et al., 2014) including
patients without pulmonary involvement (de Oliveira et al., 2007).
Reduced physical activity has been observed in patients with lcSSc
with PAH compared with patients without PAH (Mainguy, Provencher,
Maltais, Malenfant, & Saey, 2011), and in patients with SSc with early
lung involvement (Battaglia et al., 2017). Physical inactivity may lead to
AL.
muscle weakening (Turesson & Matteson, 2007) and impairment in
oxygen transport and consumption (Blom‐Bülow et al., 1983). This
may contribute to a poor HRQL (Hudson et al., 2009; Khanna et al.,
2005; Lima et al., 2015). Other lifestyle factors also have an important
impact on SSc. Smoking may worsen vascular, gastrointestinal and
respiratory outcomes (Hudson et al., 2011), and patients may have
an increased risk of alcohol‐related cancers (Olesen, Svaerke, Farkas,
& Sørensen, 2010).
The heterogeneous clinical course of SSc and the wide array of
available treatment options makes recommendations of pharmacolog-
ical treatment a challenging task. Treatment usually focuses on the
main disease features: digital vasculopathy, PAH, gastrointestinal
involvement, renal crisis, ILD and skin involvement (Kowal‐Bielecka
et al., 2009).
The condition and its treatment may affect work, leisure and
family life, so psychological and social support may be needed (The
Swedish National Board of Health and Welfare, 2016). Patients with
SSc are recommended to avoid stress, cold environments and smoking
(Boström, 2014; The Swedish Scleroderma Study Group, 2015).
Individual physical exercise programmes including information about
how to live with the disease can be designed by health professionals
such as physiotherapists, physical educators and occupational thera-
pists (Boström, 2014; The Swedish Scleroderma Study Group, 2015).
Reports of adverse effects related to participation in exercise
programmes in patients with SSc are rare. In a study of 53 patients
with SSc (Schouffoer et al., 2011), one patient experienced progres-
sively painful skin (although no thickening or inflammation of the skin
was observed), and another patient presented with Achilles tendon
rupture during circuit training. One patient with severe ILD in the
study by Alexanderson, Bergegård, Björnådal, and Nordin (2014) did
not tolerate intensive aerobic exercise, and had dyspnoea and cough
at attempts to increase exercise loads. In a recent study with a sample
of 220 patients with SSc (Rannou et al., 2016), two patients reported
fatigue, one patient reported hip pain after aerobic exercise and
another patient reported calf pain.
Exercise‐induced oxygen desaturation may occur in patients with
SSc and ILD (Someya, Mugii, Hasegawa, Yahata, & Nakagawa, 2014).
Reduced diffusion lung capacity for carbon monoxide (DLCO) and
reduced predicted lung volumes seem to predict oxygen desaturation
in exercise testing (Someya et al., 2014). PAH and exercise‐induced
PAH are common features in SSc (Gargani et al., 2013; Saggar et al.,
2010; Suzuki et al., 2015; Voilliot et al., 2014). Exercise‐induced PAH
is mainly related to increased left ventricular filling pressure and
exercise pulmonary vascular resistance (Voilliot et al., 2014), and may
reduce peripheral capillary oxygen saturation (SpO2) (Yasunobu, Oudiz,
Sun, Hansen, & Wasserman, 2005). Peak exercise pulmonary artery
systolic pressure is affected by age, ILD, and right and left ventricular
diastolic dysfunction (Gargani et al., 2013).
A study with 11 patients with SSc with pulmonary involvement
who underwent maximal exercise testing demonstrated that exercise
may intensify the already increased basal level of systemic inflamma-
tion and oxidative stress (Hargardóttir et al., 2010), suggesting that
an immune response might be triggered in these patients.
Although exercise capacity and HRQL can be improved with
pulmonary rehabilitation and aerobic exercise, ideally combined with
DE OLIVEIRA ET AL.
strength exercise in patients with ILD (irrespective of the cause),
patients with lung fibrosis may experience severe dyspnoea and
exertional desaturation (Markovitz & Cooper, 2010).
Programmes of patient education and self‐management of SSc in
distance learning format (e‐health) may be useful to reach a large
number of patients, and seem to be effective in controlling pain,
fatigue, depression and improving function in daily activities (Piga
et al., 2014; Poole, Skipper, & Mendelson, 2013). Nonetheless,
compliance is usually lower (79% in Poole et al., 2013 and 80% in
Piga et al., 2014) than observed in face‐to‐face exercise programmes
(100% in de Oliveira, dos Santos Sabbag, de Sá Pinto, Borges, & Lima,
2009 and Alexanderson et al., 2014).
Rehabilitation with a multidisciplinary team is recommended for
patients with SSc (Poole, 2010), and may improve handgrip strength
and hand function (Willems et al., 2015). Mouth exercises have been
shown to improve eating, speaking, oral hygiene and dental treatments
(Cazal et al., 2008; Maddali‐Bongi et al., 2011; Naylor, Douglass, & Mix,
1984; Pizzo, Scardina, & Messina, 2003; Willems et al., 2015). In addi-
tion to this, there is an increasing body of evidence supporting the
inclusion of various types of physical exercise as important therapeutic
tools in the treatment of SSc. However, they have not all been studied
to the same extent (Willems et al., 2015). In this review, we will discuss
the main research findings concerning physical exercise in SSc patients.
2 | AEROBIC EXERCISE
2.1 Studies including patients without pulmonary
| Antonioli et al. (2009) enrolled 16 patients with SSc (seven
involvement
The first prospective study of patients with SSc in an aerobic exercise
programme was conducted in 2009 (de Oliveira et al., 2009). Seven
female patients without pulmonary involvement and seven healthy
controls took part in an 8‐week, twice‐weekly, aerobic exercise
programme (30 min of treadmill walking). This moderate‐intensity
aerobic exercise (prescribed after a cardiopulmonary exercise test,
with heart rate maintained within anaerobic threshold and 10% before
respiratory compensation point) was efficient in significantly improving
patients’ exercise tolerance, aerobic capacity and effort oxygen
saturation, and no dropouts or adverse effects were observed.
3 | AEROBIC EXERCISE IN COMBINATION
WITH RESISTANCE EXERCISE
3.1 | Patients without pulmonary involvement
In a single subject experimental design (SSED), Alexanderson et al.
(2014), enrolled four SSc patients (three women and one man, two of
them with lung fibrosis) in an 8‐week, three times per week, exercise
programme that consisted of aerobic and muscle endurance exercises.
In the first week patients performed ergometer cycling for 10 min at an
intensity of ‘light exertion’ (assessed by a visual intensity scale). The
following week involved 5 min of a higher intensity exercise (13 on
the Borg scale) and in the following 6 weeks participants increased
intensity to 15 on the Borg scale, with a total biking time of 30 min
3
per session. After the aerobic exercise participants performed
endurance‐based resistance training for shoulder and hip flexors
aiming at performing the maximal possible number of repetitions.
During the first weeks, participants performed about 50% of the
number of repetitions performed during the initial testing (functional
muscle endurance test) (Alexanderson et al., 2006), then increasing
slowly. One of the two patients without ILD improved predicted
VO2max. Both participants without ILD significantly improved muscle
endurance. The programme was well tolerated; no dropouts or adverse
effects were observed.
3.2 | Patients with and without pulmonary
involvement
Maddali Bongi et al. (2009) studied a group of 20 patients with SSc,
half of whom had pulmonary involvement (ILD and/or pulmonary
hypertension). They were randomized to a control group or to an
interventional group involving hand and face rehabilitation plus one
of the following global rehabilitation techniques: hydrokinesytherapy
or land‐based exercises. After 9 weeks of treatment twice a week
(intensity not included) with no dropouts, patients in the interventional
group displayed significantly better hand mobility and hand function
compared to baseline. However, when assessed after 9 weeks of
follow‐up, improvements were only partially retained. The groups were
not statistically different regarding the presence of pulmonary involve-
ment, and the authors did not present data from patients separately,
but no treatment‐related adverse effects were reported.
of them with ILD) in ten individual sessions of 30 min of therapy
(including warm up, cool down, training of motor functions, respiratory
exercises, walking, finger stretching and occupational therapy). The
authors did not include the intensity of exercises. Patients were also
instructed to exercise at home. The control group was composed of
17 other patients with SSc (nine of them with ILD) who received
standard care. After 4 months with no withdrawals, patients in the
interventional group had significantly better hand mobility, exercise
tolerance and HRQL. The main differences among patients with
pulmonary involvement and patients who did not present with this
condition were, as expected, a reduced baseline DLCO and a clinically
significant compromise of HRQL (determined by airway symptoms).
Quality of life significantly improved after the period of observation
in most patients.
The multidisciplinary programme developed by Schouffoer et al.
(2011) also included patients with lung involvement. Thirteen out of
28 patients with SSc in the interventional group and 12 out of 25
patients with SSc in the control group had ILD. The 12‐week
programme involved 3‐weekly multidisciplinary group sessions com-
prising general exercises, hand/mouth exercises and education. In
addition, patients participated in individual physical therapy sessions
once a week and received instructions to exercise at home (at least
6 days per week). Patients in the interventional group had a greater
and significant improvement in mouth opening, muscle strength, walk-
ing distance and function in daily activities than patients who received
standard care. Adherence rate in the intervention group was 86%.
Although no subgroup analysis was performed comparing patients
4 DE OLIVEIRA ET
with and without ILD, the authors highlighted that patients
with pulmonary involvement significantly increased walking distance,
but did not present improvement in peak oxygen consumption
as previously observed in patients without this condition (de Oliveira
et al., 2009).
Another programme involving aerobic and resistance exercise was
developed by Pinto et al. (2011). The researchers submitted 11
patients (eight of them with no evidence of pulmonary involvement)
to a 12‐week concurrent training programme, with twice weekly
sessions. Patients with SSc performed 30 min of resistance exercises
(four sets of 8–12 maximal repetitions for the main muscle groups, five
exercises for upper and lower extremities) followed by 20 min of
treadmill exercise, at the corresponding heart rate of approximately
70% of VO2 peak. Patients significantly improved strength and muscle
function, resting heart rate, workload and time of exercise testing. No
withdrawals were observed during the intervention.
A recent randomized controlled trial (Rannou et al., 2016) enrolled
the largest sample of patients with SSc so far (n = 220). It was also a
long‐term study (12 months follow‐up). Around 18% of participants
had pulmonary fibrosis, and about 8% of patients had pulmonary
hypertension. Patients were randomized into a control group or a
personalized physical therapy programme for 1 month (three times
per week, 3 h each session, including strength and aerobic exercises,
plus exercises to increase range of motion and minimize other
limitations) followed by daily home sessions for 12 months (30 min).
The control group received usual care (that could include ambulatory
physical therapy). Patients were evaluated after 1, 6 and 12 months.
Reduction in disability was higher in the experimental group after the
first month, along with better hand mobility, amelioration of
microstomia and pain reduction, but these differences became lower
or null after a year. In the first month, only 14% of the patients with
SSc did not adhere to the programme, and 15% of patients had a poor
adherence after 12 months.
3.3 | Patients with pulmonary involvement
Two of the four participants included in the SSED exercise study by
Alexanderson et al. (2014) had ILD, as described earlier. The participant
with severe ILD [forced vital capacity (FVC) of 50% of expected value]
did not tolerate intensive aerobic exercise. Severe dyspnoea and
coughing occurred at all attempts to increase loads from moderate to
high intensity. Exercise was stopped and a lung screening revealed
increased bronchial obstructivity and fluid in the lungs. However,
mycophenolate mofetile treatment was stopped 4 months before
entering the study, which was considered the main reason for the
increased disease activity and not the exercise itself.
A case report described a woman with SSc and PAH with an
FVC of 85% (Shoemaker, Wilt, Dasgupta, & Oudiz, 2009) enrolled in
a 6‐week, three times per week ergometer cycling programme. Loads
(resistance of the cycle ergometer) started on 50% of peak workload,
progressing up to 80% of predicted heart rate. The participant
improved maximal oxygen uptake by 4% and about 102 m in a 6‐min
walk test. Both improvements were considered clinically relevant.
The exercise programme was well tolerated and the only adverse
event was a systolic hypertensive response in some sessions.
AL.
Nevertheless, adjustment in the intensity of the exercise allowed the
participant to continue.
In another case report, a patient with SSc and ILD was enrolled in a
walking exercise plus leg muscle strengthening programme for
27 weeks (1 h, five times per week); walking distance without severe
hypoxia increased after the programme, although the distance
achieved in a 6‐min walk test remained the same (Mugii, Someya, &
Hasegawa, 2011).
A patient with acute quadriparesis secondary to SSc and
associated myositis underwent a physical and occupational therapy
programme of three or more hours per day, six times per week
(Chernev, Gustafson, & Medina‐Bravo, 2009). The programme
included stretching, supine weight exercises, step‐ups, upper body
ergometry and upper body progressive weightlifting exercises. The
authors reported that the patient progressed extremely well, and no
further muscle damage was observed with an increased intensity of
the exercises.
4 | DISCUSSION
Patients with SSc exhibit a good exercise tolerance and studies
indicate effectiveness of exercise programmes in increasing tolerance
and aerobic capacity (Alexanderson et al., 2014; de Oliveira et al.,
2009; Schouffoer et al., 2011), muscle strength (Alexanderson et al.,
2014; Pinto et al., 2011), hand mobility (Antonioli et al., 2009; Maddali
Bongi et al., 2009; Mancuso & Poole, 2009), function in daily activities
(Maddali Bongi et al., 2009) and HRQL (Antonioli et al., 2009; Maddali
Bongi et al., 2009), even in patients with some degree of lung
involvement.
In patients with SSc and pulmonary involvement, evidence
indicates that they may benefit from participation in exercise
programmes (Alexanderson et al., 2014; Chernev et al., 2009; Maddali
Bongi et al., 2009; Mugii et al., 2011; Pinto et al., 2011; Schouffoer
et al., 2011; Shoemaker et al., 2009), although improvements in oxygen
consumption have not yet been observed in these patients.
Literature regarding the effects of exercise in SSc is still scarce,
with small numbers of participants in most studies, an absence of
control groups, and information regarding the precise exercise dose
not always being included. There are only two randomized controlled
trials (Rannou et al., 2016; Schouffoer et al., 2011), and only one of
these is a long‐term study (Rannou et al., 2016). Studies so far do not
include differences in response to exercise regarding gender or the
degree of pulmonary involvement. Also, when participating in experi-
mental exercise programmes, patients are usually orientated to main-
tain their usual medication (Alexanderson et al., 2014; Maddali Bongi
et al., 2009; Mugii et al., 2011; de Oliveira et al., 2009; Pinto et al., 2011).
Chronic systemic inflammation might account for deterioration in
function in daily activities and affect the ability of patients with SSc
to engage in physical activity. Pulmonary involvement, common in
patients with SSc, may also contribute to exercise intolerance (Morelli
et al., 2000). A sedentary lifestyle, in turn, might contribute to inflam-
mation, establishing a ‘vicious cycle’ (Benatti & Pedersen, 2015).
Aerobic and resistance exercise may exert long‐term effects by
improving physical capacity (aerobic capacity, muscle strength,
DE OLIVEIRA ET AL.
function in daily activities and mobility) and body composition and
reduce cardiovascular risk factors and fatigue, and improve HRQL
(Mancuso & Poole, 2009). It is possible that regular exercise is as an
important adjuvant tool in improving overall symptoms and the course
of SSc (Perandini et al., 2012).
4.1 | General precautions for physical exercise in SSc
Patients with SSc should have their blood pressure and heart rate
monitored during exercise, and particluar attention should be paid to
the cool‐down period, since rapid falls in cardiac output can lead to
syncope in patients with PAH (Markovitz & Cooper, 2010). Exercise
must be stopped if chest pain, lightheadedness or palpitations occur
(Markovitz & Cooper, 2010).
Patients with SSc and severe pulmonary hypertension may be
enrolled in an exercise programme if the nature and the intensity of
exercise is considered (Nici et al., 2006). These patients are optimally
monitored by telemetry, especially if they are known to have
experienced cardiac dysrhythmias (Markovitz & Cooper, 2010). In
these patients, activities that increase intrathoracic pressure (such as
high‐intensity exercises) should be avoided, as they may lead to
circulatory collapse and syncope (Markovitz & Cooper, 2010).
In summary, in patients without pulmonary involvement, increased
aerobic capacity, muscle strength and function in daily activities can
result from aerobic exercises at moderate to high intensity, combined
or not with resistance exercises (Alexanderson et al., 2014; de Oliveira
et al., 2009). Any type of aerobic and muscle strength exercises may
reduce disability and increase muscle strength, physical capacity and
quality of life in patients with SSc with or without pulmonary involve-
ment (Antonioli et al., 2009; Maddali Bongi et al., 2009; Pinto et al.,
2011; Rannou et al., 2016; Schouffoer et al., 2011). Patients with pul-
monary involvement may improve physical strength through aerobic
and muscle strength exercises. Aerobic exercise at moderate intensity
may increase physical and aerobic capacity, as well as quality of life
(Alexanderson et al., 2014; Mugii et al., 2011; Shoemaker et al., 2009).
5 | C O N CL U S I O N S
The current literature involving aerobic and resistance exercises in
patients with SSc demonstrates that improvements in the aerobic
capacity of patients with SSc are subtle when a concurrent training
programme is used in comparison with aerobic exercise alone. Patients
with SSc also exhibit a good response to resistance exercises, by
improving physical strength and demonstrating a good exercise
tolerance. There is a need for large multi‐centre randomized controlled
studies including patients with SSc with various degrees of pulmonary
involvement to further establish the effects of exercise in these
patients. Studies show a good adherence of patients with SSc to exer-
cise programmes with no major adverse effects.
Patients with SSc without pulmonary involvement can be
physically active at least at moderate intensity as recommended for
people in general. Regarding patients with pulmonary involvement, as
long as the condition is not severe, both aerobic and resistance exer-
cises, supervised and individually tailored, can be recommended.
5
Health professionals can support patients, informing them about the
importance of avoiding sedentary behaviour and harmful lifestyle
habits (Boström, 2014; The Swedish Scleroderma Study Group,
2015), as well as monitoring patients’ physical activity levels, aerobic
capacity and muscle strength at least once a year, as recommended
for other rheumatic diseases (Bertsias et al., 2008).
ACKNOWLEDGMENTS
We thank Mrs Monica Holmner, patient partner, for her valuable
comments on the manuscript.
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