British Journal of Rheumatology 1998;37:196–200
IMPROVEMENT OF PHYSICAL FITNESS AND MUSCLE STRENGTH IN
POLYMYOSITIS/DERMATOMYOSITIS PATIENTS BY A TRAINING PROGRAMME
G. F. WIESINGER, M. QUITTAN, M. ARINGER,* A. SEEBER,‡ B. VOLC-PLATZER,† J. SMOLEN*
and W. GRANINGER*
Department of Physical Medicine and Rehabilitation, *Department of Internal Medicine, Division of Rheumatology,
†Department of Dermatology, Division of Immunology, Allergy and Infectious Disease and ‡Division of General Dermatology,
University of Vienna, Austria
SUMMARY
In the present investigation, the benefit of physical training in patients with inflammatory myopathy was studied. In this
prospective, randomized, controlled study, 14 patients with polymyositis (PM ) or dermatomyositis (DM ) were investigated.
The training, consisting of bicycle exercise and step aerobics, took place over a 6 week period. Baseline and endpoint
measurements included an ‘activities of daily living’ (ADL) score, peak isometric torque (PIT ) generated by muscle groups in
the lower extremities, peak oxygen consumption ( VO max), and creatine phosphokinase (CPK ) levels. There was no significant
2
rise in disease activity in the training group in comparison to the controls. The ADL score for the treatment group, in
comparison to the control group, improved (P < 0.02), PIT rose (P < 0.05) and there was a statistically significant increase in
oxygen uptake relative to body weight (P < 0.05). No rise in inflammatory activity, but significant improvement in muscle
strength, oxygen uptake and well-being, were found in patients with inflammatory myopathy as a result of physical training.
Besides medication, a physical training programme consisting mainly of concentric muscle contractions should therefore be an
integral part of therapy, particularly in view of the cardiopulmonary risk of these patients.
K : Polymyositis, Dermatomyositis, Physical training, Rehabilitation, Rheumatology, Dermatology.
P and dermatomyositis (PM/DM ) are
inflammatory myopathies of unknown aetiology.
Muscle weakness, myalgias and sometimes generalized
fatigue lead to malaise and to decreased use of striated
muscles [1]. In addition, cardiac abnormalities and
pulmonary involvement may restrain cardiovascular
fitness [2–7]. Decreased muscle activity leads to further
atrophy of muscle fibres and a decrease in cardiorespiratory training status. Despite these facts, many physicians recommend a reduction in physical activity since
it is feared that the use of inflamed tissue would further
worsen the inflammatory process [1, 8–10].
In many chronic diseases, a controlled muscle training and exercise programme has been shown to result
in an improvement in the clinical symptoms [11–15].
Therefore, we hypothesized that also in myositis, at
least during stable phases of chronic muscle inflammation, controlled muscle training might be of benefit to
the well-being and cardiorespiratory fitness of the
patients.
In this prospective, controlled study of a physical
exercise programme in chronic myositis patients, we
evaluated its effects on (a) disease activity, (b) wellbeing, (c) muscle strength and (d ) cardiorespiratory
fitness. We show that the training programme does not
have negative effects on disease activity, but led to
significant improvement of the variables investigated
and thus to overall benefit to the patients.
MATERIALS AND METHODS
Study design
Patients. Patients were entered into this prospective,
randomized and controlled study if they met the following criteria.
(1) Established DM or PM with a disease duration of
>6 months.
(2) Clinical activity defined as the presence of proximal
muscle weakness, characterized clinically by a
reduced ability to climb stairs, to cross one leg
over the other, to rise from a squatting position,
to rise from a seated position, to comb the hair;
and/or by the elevation of serum muscle enzyme
values [creatine phosphokinase (CPK ) levels above
the upper limit of normal (70 IU/l ) on three or
more consecutive observations during the preceding 3 month period ].
(3) The drug therapy had to be stable for at least 3
months before the start of the training programme.
Exclusion criteria were clinically manifest pulmonary
or cardiac disorders, inclusion body myositis, fever,
neoplasms, physical inability of patients to exercise, or
increase in muscle destruction during the past 3 months
before the start of the training programme, as indicated
by at least a 50% increase in CPK levels over the
baseline value.
Fourteen consecutive patients (nine women and five
men) between 24 and 70 yr of age (median 51), who
met these criteria, were entered into the study. All
patients had a diagnosis of primary inflammatory
muscle disease as defined by the established criteria of
Bohan and Peter [16 ]. In all patients, muscle biopsies,
electromyograms and laboratory studies had been performed to establish the diagnosis. Nine out of the
Submitted 24 February 1997; revised version accepted 25 June
1997.
Correspondence to: W. Graninger, Division of Rheumatology,
Department of Internal Medicine III, University of Vienna, Vienna
General Hospital, Waehringer Guertel 18–20, A-1090, Vienna,
Austria.
© 1998 British Society for Rheumatology
196
WIESINGER ET AL.: AEROBIC CONDITIONING IN POLYMYOSITIS
14 suffered from DM and five had PM. In all patients,
medication was kept constant for the study period.
Nine patients were treated with prednisolone (up to
25 mg/day); four patients received high-dose i.v.
immunoglobulins; two patients had azathioprine,
100 mg/day, and two other patients received cyclosporin A; one patient was treated with a non-steroidal
anti-inflammatory drug only. The characteristics of
patients with DM and PM were similar ( Table I ).
None of the patients had participated in regular physical exercise for 2 yr before inclusion in our study.
Randomization. The study was conducted at the
University Hospital of Vienna following approval by
the local ethical committee. Informed consent was
obtained from all patients before they were randomly
assigned either to the training group ( TG) or to the
control group (CG). Distinct randomization lists were
used. According to the decision of the ethical committee, the patients in the control group were also to be
guaranteed participation in the training programme at
the end of the study, if it should prove beneficial.
Description of exercise regime
Patients in the training group took part in a 6 week
training programme which included a mixed programme of stationary cycling and step aerobics. Each
exercise session lasted for 1 h. Cycling was slowly
increased on an individual basis. Following a 3–5 min
warm-up phase, resistance was increased until a heart
rate of 60% of the previously established maximum
heart rate was reached. If the patient reported increased
muscle tenderness from the preceding exercise, the
resistance was decreased to a previously tolerated
amount. Every training session also included one halfhour of step aerobics. The last 5 min were used for
cool-down and stretching exercises. Because of the
different rate levels, the training load of this exercise
could be adjusted and changed. During the first 2
weeks, training was carried out twice weekly and three
times weekly during the remaining 4 weeks. The training was guided by a physiotherapist and supervised by
the authors regularly. The patients were allowed to
carry out an additional training programme, such as
stationary cycling at home, if they so wished. Patients
in the control group did not undergo any training and
continued their previous way of life.
197
Assessment of efficacy and unwanted effects
Laboratory assessment. Elevated serum levels of
enzymes, such as CPK and aldolase, which leak from
injured skeletal muscle are valuable aids in detecting
active muscle inflammation [17]. In order to determine
the effects of training on the degree of muscle destruction, these two enzymes were measured weekly on
Monday after a weekend recovery phase without
exercise.
Functional assessment. For describing individual abilities and limitations, we used a modified Functional
Assessment Screening Questionnaire. This questionnaire has been well described [18] and used for evaluating disability [19]. The questionnaire was used in a
German translation. Questionnaires for functional
assessment have been validated in many languages,
including German [20, 21], and have proved useful
across many language and cultural barriers [22–24].
The patients had to fill in the questionnaires at the
beginning and at the end of the 6 weeks observation
period. Since the use of visual analogue scale ( VAS)
anchors that clearly indicate extremes appears to have
the greatest sensitivity and is the least susceptible to
bias or distortions in rating [25, 26 ], a 10-cm-long
VAS was used for each question.
Measurement of muscle strength. Muscle strength
was determined in the TG and the CG before and at
the end of the 6 weeks observation period. The muscle
strength of the hip flexors and the knee extensors
on the right and on the left side was analysed by
a computerized isokinetic system (CYBEX 6000
Isokinetic dynamometer, Lumex Inc., New York),
which measures the peak isometric torque (PIT ).
CYBEX assessment was carried out by the same
person who was unaware of the group to which
individual patients belonged. Bilaterally, the extensors
of the knee in the seated position, and the flexors of
the hip in the supine position, were tested. The patients
had to perform maximal isometric contractions and,
during these measurements, the best of three attempts
by the individual patient was recorded. The results of
the PIT are expressed as the sum of values obtained
for the four muscle groups (hip flexors and knee
extensors on both legs) [27].
The CYBEX system has been used extensively to
assess muscle strength in normal and in some diseased
TABLE I
Clinical characteristics of study patients
Characteristics
Age (yr) (median, range)
Sex (f/m)
PM/DM
CPK level elevated above
upper limit of normal (y/n)
ADL score (mean ± ...)
PIT (Nm) (mean ± ...)
VO max (ml/min/kg) (mean ± ...)
2
Training group (n = 7)
Control group (n = 7)
56 (44–68)
4/3
2/5
7/0
40 (24–70)*
5/2
2/5
4/3
156.6 (±9.7)
633.1 (±100)
17.4 (±1)
142.6 (±19.8)*
435 (±82)*
16.9 (±1.8)*
*No statistically significant differences between the training and control group.
198
BRITISH JOURNAL OF RHEUMATOLOGY VOL. 37 NO. 2
populations, and has been reported to give valid and
reliable results [28]. The detailed method has been
outlined previously and its utility in the assessment of
strength in patients with inflammatory muscle disease
has been described [29].
Exercise testing. Exercise studies were performed
using a symptom-limited, incremental cycle ergometer
protocol. Pedalling at 50–60 r.p.m., the work rate was
increased every 2 min by 25 W from an initial load of
25 W. Ventilatory parameters were collected breath by
breath using a computer-based device (Sensor Medics
2900 System, Sensormedics, Yorba Linda, LA, CA,
USA) and 20 s averages for each parameter were
registered. Patients breathed through a mouthpiece
connected to a mass flowmeter (Sensor Medics, CA,
USA), measuring minute ventilation by the thermal
conductivity technique. Oxygen uptake ( VO ) was
2
measured with a fast-response zirconium oxide
analyser (Servomex-Taylor, Fussex). The anaerobic
threshold was determined by using the V slope technique [30]. Samples of whole blood were taken from
the hyperaemized earlobe at rest, within the last 20 s
of each work rate, and at maximal exercise with a
20 ml capillary to assess lactate concentration ( ESAT
6661, Eppendorf, Hamburg, Germany). The heart rate
and rhythm were recorded continuously at rest and
throughout exercise with a 12-lead electrocardiogram
(Siemens, Germany). Maximal oxygen uptake
( VO max) was defined as the highest O consumption
2
2
obtained during the symptom-limited exercise test [31].
In order to examine the effects of the training, values
for VO max (ml/min/kg) at initiation and at the end
2
of the study were compared.
Statistical analysis
Descriptive statistics (means, ..., median, range)
were performed for all dependent variables. In order
to determine changes in each patient, the differences
in the variable values between baseline and endpoint
were determined. Owing to the small sample sizes and
non-normality of the data of this prospective, randomized, controlled trial, exact non-parametric tests were
used. The exact Wilcoxon signed rank test was used
to test for significant changes in the variable values
before and after the training programme within the
groups. The exact Mann–Whitney U-test was used to
compare these changes between the two groups. Both
were performed using the software system StatXactTurbo (CYTEL Software Corporation, 1992,
Cambridge, MA, USA). A P value of <0.05 was
considered statistically significant.
RESULTS
The clinical characteristics of the training group
(n = 7) and the control group (n = 7) at baseline are
indicated in Table I. The two groups were well balanced
with respect to most baseline characteristics. No statistically significant differences were observed between the
two groups. Medication remained unchanged during
the 3 months before and during the study in all subjects
participating in the trial. Questioning at the end of the
study revealed that none of the control patients had
changed the extent of their daily physical activities.
Overall, the training programme was well tolerated.
The changes in endpoint results as a percentage of
baseline for patients in the training and control groups
are shown in Fig. 1. There was no statistically signific-
F. 1.—Endpoint results of creatine phosphokinase (CPK ), peak isometric torque (PIT ), peak oxygen consumption ( VO max) and ‘activities
2
of daily living’ (ADL) score are depicted as a percentage of the baseline for both the training (A) and the control group (B). The P values
(endpoint vs baseline; Wilcoxon signed rank test) indicate significant improvement of PIT (P < 0.05), VO max (P < 0.05) and ADL score
2
(P < 0.02) for the training group. For PIT, VO max and ADL score in the control group, and for CPK in both groups, changes did not reach
2
statistical significance.
WIESINGER ET AL.: AEROBIC CONDITIONING IN POLYMYOSITIS
ant change in CPK and aldolase (data not shown)
during the observation periods either in the CG (mean
−13.9%; ... 14) or in the TG (mean −6%; ...
8.5) (in spite of the training programme).
However, after the 6 weeks of training, there was a
significant (P < 0.02) improvement in ADL rating in
the patients who participated in the training (mean
20.5%; ... 4.1), but not in the control group (mean
2.9%; ... 11.1). There was also a significant group
difference for the ADL score (P < 0.02) after (but not
before) the observation period.
Moreover, muscle strength when measured with PIT
improved significantly (P = 0.03) in the training group
(mean 29.4%; ... 6.9), in contrast to the control
group (mean 11.1%; ... 11.6). Again, after the 6
weeks training programme, the group difference was
significant (P < 0.05).
Finally, after the training, the maximal VO
2
(ml/min/kg) uptake had increased in the training group
(mean 12%; ... 4.7; P < 0.05), but not in the control
group (mean −2.6%; ... 6.4), and a significant
group difference in maximal VO (ml/min/kg) uptake
2
was found between trained and control patients
(P = 0.03).
DISCUSSION
In this prospective, randomized, controlled investigation, we reveal that a training programme in patients
with DM or PM increased muscle strength, increased
O consumption during exercise, increased the ADL
2
and did not worsen muscle inflammation. Thus, with
respect to disease activity, the most important conclusion of this study is the safety of physical training in
patients with chronic inflammatory myopathy. Almost
equally important, this investigation reveals that physical training in inflammatory muscle disease is beneficial with regard to both muscle strength and patients’
well-being, as well as patients’ fitness.
Whether the training affects inflamed or noninvolved muscle fibres, or both, is unclear. In the light
of the focal involvement of muscles in inflammatory
myopathies, it can be assumed that an adequate training of healthy muscle fibres may lessen the strain on
inflamed fibres. In addition, a greater biochemical
efficiency of unaffected muscle fibres could be operative [27].
Since we know that eccentric contraction of the
muscles is associated with ultrastructural indications
of damage [32] to a much greater extent than concentric contractions [33–36 ], we recommend training consisting mainly of concentric muscle contractions for
patients with DM/PM. Exercise such as cycling and
running or walking on level ground consists mainly of
concentric contractions, in which the active muscle
shortens.
The present study is in line with previous observations in uncontrolled studies of separate myositis
patients using a non-systematic programme design [27,
28]. Here, a systematic programme in conjunction with
systematic evaluation was employed, and training
199
intensity was adjusted to fit the individual needs of the
patients.
A sedentary lifestyle is an important health risk
factor that has been well documented in the physically
abled population. It is particularly associated with an
increased risk of cardiovascular and coronary heart
disease [37–39]. A physically active lifestyle, in contrast, has been shown to be effective in lowering allcause mortality in the able-bodied population [40].
The present study leads us to conclude that a physical
training programme should be recommended for
patients with chronic DM/PM as an adjunct to drug
therapy. Training, however, must be carried out under
medical supervision and must be adjusted to fit the
needs of the patients.
A
The authors wish to thank Mrs M. Knötig for
technical assistance.
R
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