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Effectiveness of Pulmonary
Rehabilitation in Restrictive
Lung Disease
Nizar A. Naji, MRCPI; Marian C. Connor, MSc, HDipStats, SMISCP;
Seamas C. Donnelly, MD, FRCPI; Timothy J. McDonnell, MD, FRCPI

.................................................................................................................................................................................................................
..
h BACKGROUND: Pulmonary rehabilitation is effective in improving exercise .. K E Y
.
W O R D S
endurance and quality of life in chronic obstructive pulmonary disease ..
(COPD). However, the efficacy of pulmonary rehabilitation in restrictive . interstitial lung disease .
..
lung disease has not been extensively studied. ..
h METHODS: Forty-six patients with restrictive lung disease (35 interstitial lung .. outcome
diseases, 11 skeletal abnormalities) were admitted to a pulmonary ..
. pulmonary rehabilitation
rehabilitation program; 26 completed the 8-week program and 15 were ..
followed to a 1-year reassessment. Fifteen noncompliant patients were .. restrictive lung disease
excluded and 1 patient with interstitial lung disease died at 8 weeks.
..
..
Pulmonary function tests, exercise endurance, quality of life (Chronic .. skeletal abnormalities
Respiratory Disease Questionnaire, St. George’s Respiratory ..
Questionnaire, Hospital Anxiety and Depression scale and dyspnea) .. survival
were measured at baseline, 8 weeks, and 1 year. ..
..
h RESULTS: Exercise endurance (treadmill) improved at 8 weeks (mean ..
improvement, 10.2 T 7.4 minutes) and at 1 year (mean improvement, ..
8.7 T 12.2 minutes). Shuttle test improved at 8 weeks (mean ..
improvement, 27.2 T 75.9 m) but not at 1 year. Patients using .. From the St. Michael’s Hospital and St.
long-term oxygen therapy (LTOT) had a better improvement in the . Vincent’s University Hospitals (Dr Naji,
.. Ms Connor, and Dr McDonnell); St.
treadmill test (P G .01) at 8 weeks compared with those not using .
LTOT. Thirty-three percent of patients failed to complete the program. .. Vincent’s University Hospital, Elm Park
There was significant improvement in dyspnea and quality of life in . (Dr Donnelly), Dublin 4, Ireland.
.. Statement of submission: All
Chronic Respiratory Disease Questionnaire, St. George’s Respiratory . authors have read and approve the
Questionnaire, and Hospital Anxiety and Depression scale for .
.. submission of the manuscript, and the
depression at 8 weeks compared with baseline; there was a .. manuscript has not been published and
sustained significant reduction in hospital admission days noted . is not being considered for publication
at 1-year postrehabilitation (P G .05). .. elsewhere in whole or part in any
.
h CONCLUSIONS: Pulmonary rehabilitation is effective in improving exercise .. language except as an abstract
endurance and the quality of life and in reducing hospital admissions . Address correspondence to:
.. Dr Timothy J. McDonnell, MD, FRCPI,
in this small group of patients with significant restrictive lung disease. . Department of Respiratory Medicine,
The relatively large dropout number suggests that a standard chronic
..
.. St. Vincent’s University Hospital,
obstructive pulmonary disease program may not be ideal for patients .. Elm Park, Dublin 4, Ireland (email:
with restrictive lung disease. timmcd@iol.ie).

INTRODUCTION tion in restrictive lung disease (RLD; interstitial lung


............................................................................................................. disease [ILD] and skeletal abnormalities [SA]) is limited.
Pulmonary rehabilitation is an established therapeutic Unlike COPD in which respiratory mechanics, ven-
intervention in chronic obstructive pulmonary disease tilatory impairment, hyperinflation, and skeletal muscle
(COPD) which improves exercise tolerance and quality dysfunction limit exercise capacity,4,5 gas exchange im-
of life (QoL) and reduces hospital admission.1Y3 How- pairment is more important in limiting exercise in
ever, evidence of the benefit of pulmonary rehabilita- patients with ILD.6,7 Interstitial lung disease causes

Effectiveness of Pulmonary Rehabilitation in RLD / 237

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alveolar and capillary destruction which decreases Thirty-five patients with ILD (idiopathic pulmonary
compliance, increases work of breathing, increases fibrosis, 28; sarcoidosis, 1; Sjogren, 1; scleroderma, 1;
pulmonary vascular resistance, and limits venous return. lymphangioleiomyomatosis, 1; Systemic Lupus Erythro-
Cardiac output increase is inadequate to support the in- matosis, 1; rheumatoid arthritis, 1; and radiation fibrosis,
creased muscle oxygen requirements during exercise.6 1) and 11 patients with SA (thorocoplasty, 4; kyphosco-
The main predictors of exercise limitation in patients liosis, 3; lobectomy, 2; pnemonectomy, 1; and ankylos-
with ILD are the increase in the resting alveolar/arterial ing spondylitis, 1). Patients were excluded if they had
pressure gradient, decreased diffusing capacity, and re- significant airflow obstruction, were noncompliant,
duced pulmonary function parameters (eg, forced expi- were unable to perform pulmonary function tests, or
ratory volume in the first second).7 Arterial hypoxemia were unable to perform an exercise endurance test.
significantly impairs maximal incremental exercise in Patients were initially admitted to the hospital for 3
those patients,6,8 and supplemental oxygen increases days for baseline assessment and to commence on the
exercise duration and maximal work load.8,9 program. The program consisted of exercise and educa-
Pulmonary function is altered in SA mainly due to the tional components as previously described18 and was
restriction of chest wall movement. Exercise in patients continued postdischarge twice a week over a period of
treated for tuberculosis by thoracoplasty is limited by 8 weeks. Pulmonary function testing, including spi-
ventilatory capacity and this is due to a reduction in both rometry, and diffusion capacity were performed (V max
dynamic lung volumes and respiratory frequency.10 229, Sensor Medics, Yorba Linda, Calif) according to the
However, in another study, the exercise capacity in European Respiratory Society guidelines.19 It was not
patients with ankylosing spondylitis was not influenced possible to perform lung volumes on all patients. The
by the limitation of chest wall movements,11 and pe- treadmill test, measured in minutes,20 and the shuttle
ripheral muscle function seemed to be the most im- test, measured in meters21Y23 were performed.
portant determinant of exercise intolerance in those Health-related QoL was assessed by the Chronic
patients. This suggests that deconditioning may be the Respiratory Disease Questionnaire (CRDQ) and the St.
main factor in the production of the reduced exercise George’s Respiratory Questionnaire (SGRQ). An interviewer-
capacity in at least some patients with RLD due to SA.12 administered questionnaire, CRDQ is composed of 4
In a review of the benefits of pulmonary rehabilita- domains (dyspnea, fatigue, emotional function, and
tion in non-COPD patients, Crouch and Macintyre have mastery).24 The SGRQ is a well-validated questionnaire
suggested that functional improvements of non-COPD for the evaluation of psychosocial impact of respiratory
patients are similar to those obtained by COPD symptoms.25 Anxiety and depression were measured
patients.13Y15 Foster and Thomas studied a 32 heteroge- using Hospital Anxiety and Depression (HAD) scale, a
neous group of non-COPD patients with ILD, bronchi- self-assessment scale that has been demonstrated to be
ectasis, fibrothorax, thoracoplasty, and neuromuscular a reliable instrument for detecting anxiety and depres-
abnormalities. They noted that their improvement with sion in the setting of medical outpatients clinics.26
rehabilitation was only slightly less than that of COPD Hospital admission days, Borg scale for dyspnea, body
patients (P = .3).14 A further study showed that pulmo- mass index, and oxygen use were also assessed.
nary rehabilitation is as beneficial in post-tuberculosis Every patient was assessed on admission to the pro-
thoracoplasty patients as in COPD patients when gram, at 8 weeks, and then at 1 year. The same re-
matched for the severity of their disability.15 A number spiratory physiotherapist conducted the initial and
of reports have recommended that ILD patients be interval assessments.
rehabilitated before transplantation,16,17 but a quantifi-
cation of benefit is lacking.
Statistical Analysis
We reviewed 46 patients with RLD who attended our
rehabilitation center over a period of 8 years to deter- All interval data are expressed as means T SD, and all
mine the effect of pulmonary rehabilitation on exercise ordinal data are expressed as medians (ranges). t tests
tolerance, dyspnea, QoL, and subsequent hospital were used to test differences between interval data
admission rate. We also examined whether the outcome (lung function variables, exercise tests, respiratory bed
of rehabilitation is different in the 2 principal groupsV days), and Wilcoxon signed rank tests were used for
those with ILD and SA. QoL data (CRDQ, dyspnea, SGRQ, HAD). Where
between-group differences (ILD and SA, compliant
METHODS and non-compliant, LTOT or no LTOT) were tested,
............................................................................................................. completely randomized or repeated measures analysis
Four hundred eighty-three patients with chronic lung of variance as appropriate was used for interval data
disease were referred to our pulmonary rehabilitation and Mann-Whitney U tests were used for ordinal data.
center over the past 8 years. Of the 483 patients, 46 Survival analysis were demonstrated by Kaplan-Meier
(9.5%) had RLD and were the subject of this study. cumulative survival.

238 / Journal of Cardiopulmonary Rehabilitation 2006;26:237/243

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RESULTS assessment. Four (8.7%) patients with ILD had died at 1
............................................................................................................. year (Figure 2) from respiratory failure. Two patients
Baseline Characteristics with SA did not attend the 1-year assessment and
subsequently were excluded and regarded as noncom-
Of 46 patients (19 ILD, 7 SA), 26 (56.5%) completed the pliant. Eight (17.4%) patients, all ILD, are still in the
8-week program, and their baseline characteristics are program and have not finished the 1-year program. Six
shown in Table 1. Fifteen (32.6%) ILD patients and 4 SA patients with ILD and 1 patients with SA who finished
(8.7%) were noncompliant and subsequently excluded the 1-year program died at different times over a period
from the analysis. Compliance was measured as atten- of 90 months due to different causes. Seven patients
dance at 24 exercise sessions and at required reassess- with ILD and 4 with SA are still alive. The most common
ments at 8 weeks and 1 year. One patient with ILD died cause of death in 8 of 11 ILD and 2 SA patients was
at 8 weeks from respiratory failure secondary to severe respiratory failure.
pneumonia (see Figure 1).
There was no statistically significant difference in Results at 8 Weeks
baseline forced expiratory volume in 1 second, forced
vital capacity, the ratio of forced expiratory volume in 1 At 8 weeks, there was statistically significant difference
second and forced vital capacity, and diffusing capacity in exercise endurance, treadmill test, (P G .0001) and
of lung for carbon monoxide (DLCO) between the ILD shuttle test (P G .01), in the compliant group that
and SA groups. Baseline scores of CRDQ and SGRQ attended the 8-week assessment (Table 2) The data in
indicated a moderate reduction in QoL in these patients. this table compare the 26 patients who completed the
The median HAD/anxiety score of 7.5 (1, 12) in ILD 8-week program with the same 26 patients at baseline
patients represented a group at high risk of becoming and similarly compare the 15 who completed the 1-year
anxious, whereas the depression score was not indica- program with those same 15 at baseline. There was
tive of risk of depression. DLCO was consistent with a statistical significant difference in CRDQ (dyspnea) and
moderate to severe impairment in gas transfer (Table 1). QoL dataVCRDQ, SGRQ, and HAD for depression,
compared with baseline. There was no statistical sig-
Survival nificant difference in the percentage change from base-
line to 8 weeks in exercise endurance (treadmill test),
At 1 year, 15 (57.7%) of 26 patients who had completed shuttle test, SGRQ, dyspnea, CRDQ, or HAD results be-
the 8-week program (11 ILD, 4 SA) completed the final tween the 2 groups (ILD and SA), which indicate that
............................................................................................................. both groups had similar benefit from the 8-week
Table 1 & BASELINE CHARACTERISTICS program (Table 3).
OF THE 26 PATIENTS WHO ATTENDED Comparing LTOT with non-LTOT users, patients who
THE 8-WEEK REASSESSMENT were using LTOT had a better percentage change from
Variable ILD SA P baseline in treadmill test (P G .01) than those who were
Age, y 66.5T11.3 69T10.3 .70 not on LTOT. Patients who were using LTOT (n = 13)
FEV1%pred. 68.2T22.1 53.3T14.5 .13 had a 160% improvement from baseline treadmill score
FVC%pred. 66.7T20.7 53.1T13.4 .25
at 8 weeks compared with those who were not using
LTOT (n = 13)Vthose not using LTOT had an improve-
FEV1/FVC 79.8T12.6 81.6T12.6 .90
ment of 54.6% from baseline measurements (Table 4).
DLCO%pred. 42.5T14 55.3T20.1 .1
All patients had rest and exercise oximetry together with
DLCO/VA 62.8T18.7 80.7T23.2 .6
overnight oximetry and arterial blood gases to de-
Shuttle, m 206T108 220T78 .60 termine their oxygen requirements before the com-
Treadmill, min 13.3T10.4 12.3T6.2 .80 mencement of the rehabilitation program. Oxygen
CRDQ 16(12.6,22.6) 14.9(12.8,15.6) .90 requirements for those on LTOT ranged from 4 to 8 L/min,
Dyspnea 3.4(1.8,5.5) 2.2(1.8,3) .20 given with a portable system via nasal prongs, as
SGRQ 48(27.6,67.9) 36.7(28.9,45.9) .40 required, to maintain an oxygen saturation of Q90%
HAD (anxiety) 7.5(1,12) 2.5(1,4) 1.00 during exercise.
HAD (depression) 5.5(1,6) 2.5(1,4) .50
Hospital admission days 13.5T15.1 10.4T9.7 .90 Results at 1 Year
(year prerehabilitation) Of the 26 patients who finished the 8-week program, 15
BMI, kg/m2 26.7T4.9 23.8T3.4 .20 completed 1 year (10 ILD and 5 SA)Vsee Table 2. The
ILD indicates interstitial lung disease; SA, skeletal abnormalities; FEV1, forced improvement in treadmill test was maintained at 1 year
expiratory volume in 1 second; FVC, forced vital capacity; DLCO, diffusing (P G .02). The improvement in shuttle test, dyspnea, and
capacity of lung for carbon monoxide; VA, alveolar volume; CRDQ, Chronic
Respiratory Disease Questionnaire; SGRQ, St. George’s Respiratory QoL parameters that has been achieved at 8 weeks was
Questionnaire; HAD, Hospital Anxiety and Depression; BMI, body mass index. not maintained at 1 year

Effectiveness of Pulmonary Rehabilitation in RLD / 239

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Figure 1. Diagram showing patient numbers at baseline, 8 weeks, and 1 year during this study.

There was also a significant reduction in the hospital SA. Two SA and 3 ILD patients did not attend their
admission days in the year postrehabilitation compared 1-year assessment, although they had completed the
with first year (10 days in the year prerehabilitation 8-week program and the initial reassessment. There was
compared with 2.8 days postrehabilitation, P G .05). no statistical difference in the baseline characteristics
Data on previous hospital admissions and admissions of forced vital capacity, HAD, QoL, gender, LTOT, or
postrehabilitation were obtained from the patients’ support (living alone or not) between the compliant
medical records in our own hospital and the various and noncompliant group. However, there was a statisti-
other hospitals attended by the patients. cally significant difference in the baseline (shuttle) exer-
Removing the 8 patients who are still actively cise capacity test (P G .02) but no significant difference
involved with rehabilitation and the 5 who died within (P G .7) in the treadmill test between compliant and
the first year, there were 15 (10 ILD, 5 SA) compliant noncompliant groups. There was also a statistical
patients versus 14 (9 ILD, 5 SA) noncompliant in the difference in DLCO (P G .005) between the compliant
sample population at 1 year. There were more non- (mean 53%pred) and the noncompliant group (mean
compliant patients in the SA group (45%) than those 39%pred.).
with ILD (26%). Most noncompliance occurred in the There were 13 deaths over a period of 8 years (11 ILD
first 8 weeks (n = 10). Seven patients had ILD and 3 had and 2 SA)V6 (17.1% of ILD group) patients with ILD
died in the first year due to respiratory failure. None died
in the SA group in the first year. Seven (15.2% of total
group) patients died after 1 year (5 ILD, 2 SA). There was
no statistically significant difference in spirometry,
DLCO, exercise endurance, shuttle test, dyspnea, and
QoL parameters at baseline between survivors and
nonsurvivors in either ILD or SA group. However, the
patients who did not survive spent more time in hospital
as an inpatient in the year before rehabilitationV22.8
versus 7.1 days (P G .005). Body mass index was not
different between survivors and nonsurvivors.

DISCUSSION
.............................................................................................................
This study suggests that pulmonary rehabilitation is
Figure 2. Kaplan-Meier cumulative survival plot. effective in a small group of 15 patients with RLD who

240 / Journal of Cardiopulmonary Rehabilitation 2006;26:237/243

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..................................................................................................................................................................................................................................
Table 2 & RESULTS OF PRP AT 8 WEEKS AND 1 YEAR COMPARED WITH BASELINE
Baseline (n = 46) At 8 Weeks (n = 26) At 1 Year (n = 15)
Variable Measurement Measurement P Measurement P
Treadmill test, min 12 T 9.5 22.7 T 11.7 G.0001 20.3 T 12.5 G.02
Shuttle test, m 183.7 T 108.8 234.6 T 115.7 G.1 209.3 T 120.8 G.80
Dyspnea (CRDQ) 2.8(1.4,5.5) 4(2.2,6.5) G.003 3.9(1.2,4.6) G.30
CRDQ 15.7(9.5,22.6) 19.1(11.5,27.1) G.002 17.9(10.7,25.1) G.60
SGRQ 48.3(21.5,82) 39.5(17.4,69.4) G.10 47.1(18.3,70.8) G.60
HAD (anxiety) 7(0,13) 5.5(0,12) G.2 8(3,14) G.70
HAD (depression) 5(1,12) 4(1,9) G.02 5(2,8) G.30
RBD 10 T 12.2 2.8 T 6.4 G.04
PRP indicates pulmonary rehabilitation (program); CRDQ, Chronic Respiratory Disease Questionnaire; SGRQ, St. George’s Respiratory Questionnaire; HAD,
Hospital Anxiety and Depression; RBD, respiratory bed days.
Statistical tests (paired t tests) were performed between the 26 patients who commenced at baseline and reached 8 weeks and similarly between the
15 patients who commenced at baseline and reached 1 year.

completed both the required attendances and reassess- nificant financial saving to the Irish health service with
ments. There was an improvement at 8 weeks and a the estimated average cost of the respiratory admission
sustained improvement at 1 year, as shown by the in- of A2,889 (1999) and a total cost of the respiratory
crease in exercise tolerance, reduced dyspnea, and admissions of A132.1 million (1999) and A190 million
improved QoL. This improvement gained during reha- in 2001.31
bilitation is most marked during the intensive phase of At baseline, there was no statistically significant dif-
the program, that is, the first 8 weeks. This improve- ference in PFT, DLCO, exercise endurance, shuttle test,
ment tended to diminish after the intensive phase of the dyspnea, and QoL parameters between survivors and
program, probably related to the progressive nature of nonsurvivors in both ILD and SA groups. However, the
the disease and perhaps patients’ compliance. This is patients who did not survive spent more time in
comparable to the gradual fall in exercise tolerance in hospital as inpatients in the year before rehabilitation
patients with COPD, which has been shown in previous (P G .005). The compliant patients did significantly
studies, demonstrating that the initial improvement in better in the shuttle test at baseline assessment com-
exercise tolerance diminished over the subsequent 12 pared with the noncompliant patients. However, there
and 18 months.27Y30 At 1 year, rehabilitation continued was no significant difference in the other exercise or
to be also beneficial in improving exercise endurance QoL parameters. Noncompliant patients had a poorer
(treadmill) compared with 8 weeks, but improvement in DLCO at baseline than compliant patients.
shuttle test and CRDQ dyspnea was not statistically Patients with chronic conditions may frequently
significant. demonstrate poor compliance with the treatment and
The current study shows that there was significant follow-up.32 These conditions are also progressive and
reduction in the rate and duration of admission to the irreversible and demonstrate failure to sustain at 1 year
hospital in the year postrehabilitation similar to COPD some of the improvement in the physiological and QoL
patients. Reducing admission days would result in a sig- measurements achieved.

..................................................................................................................................................................................................................................
Table 3 & BASELINE AND 8 WEEKS CHANGES FOR BOTH GROUPSVINTERSTITIAL LUNG
DISEASE AND SKELETAL ABNORMALITIES
ILD SA
Variable Baseline 8 Weeks Baseline 8 Weeks
Shuttle, m 171 T 102 232 T 118 224 T 126 243 T 118 (NS)
Treadmill, min 11.9 T 10.5 20.8 T 11.9 12.3 T 5.4 27.9 T 11.7 (NS)
Dyspnea (CRDQ) 3(1.4,5.5) 3.4(2.4,6.5) 1.8(1.2,2.3) 3.4(2.2,5.4) (NS)
CRDQ 15.6(9.7,22.6) 17.2(14.6,27.1) 11.3(9.7,13.7) 19.5(14.6,24.3) (NS)
SGRQ 48.1(23,82) 26.4(17.4,69.4) 33.5(28.9,38.6) 38.7(24.6,45) (NS)
HAD-A 7(1,13) 1(0,12) 2(1,5) 5(0,12) (NS)
HAD-D 4(1,12) 1(0,9) 3(1,4) 3(1,5) (NS)
ILD indicates interstitial lung disease; SA, skeletal abnormalities; CRDQ, Chronic Respiratory Disease Questionnaire; SGRQ, St. George’s Respiratory
Questionnaire; HAD, Hospital Anxiety and Depression; NS, not significant.

Effectiveness of Pulmonary Rehabilitation in RLD / 241

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............................................................................................................. pulmonary rehabilitation program with the potential for
Table 4 & TREADMILL RESULTS IN significant financial saving to the healthcare system.
MINUTES AT BASELINE AND 8 WEEKS This benefit resulted from a significant improvement in
DEPENDING ON THE USE OF LONG-TERM exercise endurance, dyspnea sensation, and QoL and
OXYGEN THERAPY was associated with a significant reduction in require-
% Change ment for hospital admission 1 year postrehabilitation.
Group Baseline 8 Weeks P From Baseline P Studies of the value of pulmonary rehabilitation in pa-
LTOT 9.6 T 8.8 22.2 T 12.1 G.04 160% tients with RLD are complicated by the heterogeneous
No LTOT 15.4 T 9.7 23.3 T 11.7 .8 64.6% nature of the disorders associated with RLD and the
% Change G.01 variation in the rate of disease progression. This study
between may be too small to adequately tease out differences
groups between the responses of patients in the 2 groups with
LTOT indicates long-term oxygen therapy. RLD to pulmonary rehabilitation, however. Important
additional questions remain such as the following: When
LTOT: Patients who were on LTOT had a better is the optimal time to initiate pulmonary rehabilitation in
percentage change from baseline in treadmill test than patients with RLD? Does severity of disease make a
those who were not on LTOT. However, it did not show difference to outcome? Should ILD patients be offered a
any impact on shuttle test and other QoL variables. This separate program to SA patients. Perhaps, offering this
observation is consistent with previous studies that therapy earlier in the disease process, with content more
showed that supplemental oxygen in patients with ILD specific to patients with RLD, would be more beneficial
increases exercise duration and maximal work load9 with a probable potential for less dropouts.
and that the increase in exercise time is significantly
correlated with oxygen saturation due to a reduction of
blood lactic acid with oxygen therapy.33 Many patients References
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