Pulmonary rehabilitation in patients with restrictive lung diseases

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1 Pulmonary rehabilitation in patients with restrictive lung diseases B. Salhi Ghent University Hospital Department of Respiratpory Medicine e- mail:

2 1. Introduc+on COPD versus non- COPD 3. Effects of rehabilita+on in pa+ents with restric+ve lung diseases IntersFFal Lung Disease Idiopathic Pulmonary Fibrosis Kyphoscoliosis Sarcoidosis Neuromuscular diseases TB sequelles 3. Conclusions

3 Introduction Pulmonary rehabilitafon programs have shown to improve exercise performance and health status in pafents with COPD. In many aspects, the symptoms of pafents with restricfve disorders are similar to those of COPD pafents. Less is known about rehabilitafon of pafents with non- COPD.

4 Design ProspecFve, non- randomized trial Program Intensive inpafent pulmonary rehabilitafon program of 4 weeks 2x / 45 min. physical therapy 45 min. occupafonal therapy EducaFon RelaxaFon Foster et al, 1990

5 FOSTER STUDY COPD: n = 317 NON COPD: n=32 Pulmonary Fibrosis (n= 7) Bronchiectasis (n= 7) Scoliosis (n= 4) Neuromuscular disease (n= 4) Fibrothorax (n= 3) Others (n= 7) Foster et al, 1990

6 FOSTER STUDY Patient characteristics COPD (n=317) % Non COPD (n= 32) % FVC (L) 1.58 ± ± 16 FEV1 (L) 0.65 ± ± ± ± ± ± 22 FEV1/ FVC 42 ±10 75 ± 14 6MWD (m) 116 ± ± 67 PImax (cmh 2 O) 38 ± ± 18 PEmax(cmH 2 O) 51 ± ± ± ± ± ± 13 Foster et al, 1990

7 FOSTER STUDY Effects of rehabilitation in NON COPD Admission % predicted FVC (L) 1.20 ± ± 16 FEV1 (L) 0.88 ± ±22 Discharge % predicted 1.30 ± ± ± ±19 P= NS P= NS FEV1/ FVC 75 ±14 76 ±14 P= NS 6MWD (m) 84 ± ± 112 P< 0.01 PImax (cmh 2 O) 39 ± ±24 44 ±22 47 ±25 P= NS PEmax (cmh 2 O) 43 ±23 27 ±13 58 ±28 36 ±16 P< Foster et al, 1990

8 FOSTER STUDY 6MWD (m) COPD NON COPD Foster et al, 1990

9 à EffecFveness of rehabilitafon in NON- COPD (GRADE B evidence) Small observafonal or restrospecfve Heterogeneinous pafent populafon No predifined inclusion criteria Impaired and non impaired pafents Ries et al, 2006

10 Design ProspecFve, non- randomized trial Inclusion criteria Diagnosis of a restricfve lung disease (RLD) à (FEV 1 < 60%; FEV 1 / FVC rafo > 70%; TLC< 75%) At least two of the following criteria : Max.workload < 90 Waj 6MWD < 70% pred. QF < 70% pred. PI max < 70% pred. PE max < 70% pred. CRDQ < 100 points or CRDQdyspnea < 20 points Salhi et al, 2010

11 N = 704 N = 673 (95%) COPD or mixed syndrome N = 31 (5%) restricfve syndrome Methods MulFdisciplinary outpafent rehabilitafon program of 6 months 2 h sessions (endurance + strength) Salhi et al, 2010

12 PaFent CharacterisFcs Salhi et al, 2010

13 PaFent CharacterisFcs Salhi et al, 2010

14 PaFent CharacterisFcs Salhi et al, 2010

15 Different diagnosis of patients with restrictive diseases RLD (N= 31) Kyphoscoliosis (11) IntersFFal Lung Disease (11) Neuromuscular disease (7) Sequelae of TB (2) Salhi et al, 2010

16 6MWD * 64m * # 81m (m) intake 3m 6m Salhi et al, 2010

17 Muscle force Intake 3 month 6 month * (% pred.) PImax PEmax QF Salhi et al, 2010

18 QoL (CRDQ) 35 Intake 3 month 6 month 30 Points dyspnea fafgue emofon mastery Salhi et al, 2010

19 Subanalysis ILD CWD Intake (N=11) Intake (N= 20) Age (years) FEV 1 (% pred.) * 54± 19 57± 16 47± 17 46± 18 Wmax (% pred.) 37± ± 16 6MWD (m) 321 ± ± 105 QF (% pred.) 62 ± ± 22 PImax (% pred.) PEmax (% pred.) 43± 18 38± 22 33± 16 43± 25 CRDQ dyspnea (points) 15 ± 5 17 ± 6 Salhi et al, 2010

20 6MWD 450 * 54m 69m Distance (m) 350 * 79m 102m ILD CWD 250 intake 3 months 6 months

21 Quadriceps Force 90 ILD CWD * % pred intake 3 months 6 months B. Salhi et al, 2010

22 Dyspnea 25 ILD CWD CRDQd (points) * * 10 intake 3 months 6 months B. Salhi et al, 2010

23 Spruit et al, 2013

24 1. Introduc+on COPD versus non- COPD 2. Effects of rehabilita+on in restric+ve lung diseases IntersFFal Lung Disease Idiopathic Pulmonaryn Fibrosis Kyphoscoliosis Sarcoidosis 3. Conclusions

25 6MWD: 46 m * ProspecFve Non randomized trial N= N 4 weeks InpaFent program N= 402 ILD pafents Intake 308 m 354m aqer rehabilitafon Hupmann et al, 2013

26 The mean change in 6MWD depends on baseline 6MWD Hupmann et al, 2013

27 ILD 6MWD Dowman et al, 2014

28 ILD QoL Holland et al., Cochrane Library 2008 Dowman et al, 2014

29 1. Introduc+on COPD versus non- COPD 2. Effects of rehabilita+on in restric+ve lung diseases IntersFFal Lung Disease Idiopathic Pulmonary Fibrosis Kyphoscoliosis Sarcoidosis 3. Conclusions

30 N= 90 ProspecFve non randomized open trial 8- week outpafent program 6 months follow up Kozu et al, 2011

31 PaFent CharacterisFcs IPF (N=45) Mean (SD) Age (years) 68 ± 8 67 ± 5 Gender (M) BMI (kg/m 2 ) 21 ± 3 21 ± 2 COPD (N=45) P < 0,05 FEV 1 (%pred.) 80 ± ± 12 * FVC (%pred.) 69 ± ± 23 * FRC (%pred.) 65 ± ± 23 * TLC (%pred.) 64 ± ± 15 * DL, CO(%pred.) 39 ± ± 24 * QF (kg)/ body weight( kg) (%) 35 ± ± 15 * 6MWD (m) 308 ± ± 109 Wmax (Waj) 42 ± ± 23 SF- 36 PF (points) 36 ± ± 22 MRC 3 ± 1 3 ± 1 Kozu et al, 2011

32 Quadricepsforce/ body weight IPF COPD * * % * intake 8 weeks 6 months Kozu et al, 2011

33 Dyspnea (MRC) 5 IPF COPD 4 points 3 2 * * * 1 0 intake 8 weeks 6 months Kozu et al, 2011

34 Dyspnea in IPF pa+ents (MRC) Kozu et al, 2014

35 6MWD 400 IPF COPD Δ: 53 m * * 350 * m 300 Δ: 16m intake 8 weeks 6 months Kozu et al, 2011

36 MCID 6MWD in IPF pa+ents 28 m MCID Kenn et al, 2013

37 Long term effects on 6MWD in ILD pafents Holland et al., Cochrane Library 2008

38 Long term effects on dyspnea in ILD pafents Holland et al., Cochrane Library 2008

39 Long term effects in ILD pafents Reyerson et al, 2013

40 RelaFonship between 6MWD and risk of mortality Singh et al, 2014

41 1. Introduc+on COPD versus non- COPD 2. Effects of rehabilita+on in restric+ve lung diseases IntersFFal Lung Disease Idiopathic PulmonaryFibrosis Kyphoscoliosis Sarcoidosis 3. Conclusions

42 RCT 3 months OutpaFent program Cejudo et al, 2014

43 PaFent CharacterisFcs of KyphoscolioFc pafents Rehabilita+on (N=16) Control (N=11) Age (years) 61 ± 9 64 ± 9 FEV 1 (%pred.) 29 ± ± 10 FVC (%pred.) 32 ± ± 12 TLC (%pred.) 41 ± ± 15 RV (%pred.) 59 ± ± 16 SWD (m) 188 ± ± 98 PImax (%pred.) 37 ± ± 10 PEmax (%pred.) 48 ± ±14 CRDQd (points) 2,7 ± 0,9 2,7 ± 0,7 Cejudo et al, 2014

44 Effect of 3 months rehabilita+on on SWD in pa+ents with KS CONTROL Δ 23 m 220 Δ 75 m * m REHABILITATION Intake 3 months Clejudo et al 2014

45 Effects of 3 months rehabilita+on on muscle force in pa+ents with KS REHABILITATION CONTROL Cejudo et al, 2014

46 Effect of 3 months rehabilita+on on dyspnea in pa+ents with KS 5 CRDQd (points) REHABILITATION CONTROL * 1 0 Intake 3 months Clejudo et al 2014

47 1. Introduc+on COPD versus non- COPD 2. Effects of rehabilita+on in restric+ve lung diseases IntersFFal Lung Disease Idiopathic Pulmonary Disease Kyphoscoliosis Sarcoidosis 3. Conclusions

48 Conclusions PaFents with restricfve lung diseases share a number of common manifestafons. In pafent with a restricfve lung disease pulmonary rehabilitafon results in beneficial effects on exercise capacity, dyspnea, muscle strenght and QoL. To date there is no reliable evidence of the durafon of the effects of rehabilitafon in pafents with restricfve lung diseases.

49 Thank You Ghent University Hospital Ghent, Belgium Bihiyga.

50

51

52 Subanalysis ILD CWD Intake (N=11) 3 months (N=11) 6 months (N= 10) Intake (N= 20) 3 months (N= 18) 6 months (N= 16) Wmax (% pred.) 37 ± ± 25* 52 ± 27# 46 ± ± 19* 57 ± 24# * 6MWD (m) 321 ± ± 184* 428± 211 # 427 ± ± 108* 482 ± 100# QF (% pred.) 62 ± ± ± ± ± 27* 79 ± 36# CRDQ dyspnea (points) 15 ± 5 20 ± 7* 21 ± 7# 17 ± 6 19 ± 5* 23 ± 6# Salhi et al, 2010

53 Effects of 8 week rehabiliafon and 6 months follow up IPF COPD Intake (N= 36) 8 weeks (N=36) 6 months (N= 30) Intake (N= 40) 8 weeks (N= 40) 6 months (N= 37) 6MWD (m) 323 ± ± 122* 320± ± ± 99* 367 ± 95# QF/ body weight (% ) 37 ± ± 19* 37 ± 18 43,8 ± 13,4 54 ± 15,7* 51,8 ± 16,2# Dyspnea MRC (points) 3 ± 1 2,5 ± 1* 2,9 ± 1 3 ± 1 2,3 ± 0,9* 2,4 ± 0,9# SF- 36 PF (points) 39 ± ± ± ± ± 20* 45 ± 20# Kozu et al, 2011

54 MCID Singh et al, 2014

55 Effects of 3 months rehabita+on Cejudo et al, 2014

56 RCT 3 months 2x/ week OutpaFent program Jackson et al, 2014

57 PaFent CharacterisFcs of IPF pafents Rehabilita+on (N=11) Mean (SD) Control (N=10) Age (years) 71 ± 6 66 ± 7 BMI (kg/m 2 ) 31 ± 3 33 ± 2 MVV (L/min) 69 ± ± 26 FVC (%pred.) 60 ± ± 14 TLC (%pred.) 58 ± 8 60 ± 12 DL, CO(%pred.) 44 ± ± 11 6MWD (m) 361 ± ± 109 Jackson et al, 2014

58 Effect of 3 months rehabilta+on on 6MWD in pa+ents with IPF 375 REHABILITATION 350 6MWD (m) CONTROL Intake 3 months Jackson et al 2014

59 Effect of 3 months rehabilta+on on maximal exercise capacity in IPF pa+ents (ml/min Jackson et al 2014

60 Total work performance and training intensity per training session in COPD and IPF pafents COPD COPD IPF IPF Kozu et al, 2011

61 1. Introduc+on COPD versus non- COPD 2. Effects of rehabilita+on in restric+ve lung diseases IntersFFal Lung Disease Idiopathic Pulmonary RehabilitaFon Kyphoscoliosis Sarcoidosis 3. Conclusions

62 IntroducFon Effects of rehabilitafon in restricfve lung diseases IntersFFal Lung Disease Idiopathic Pulmonary RehabilitaFon Kyphoscoliosis Sarcoidosis Conclusion

63 Design r = 0.61 p = r = 0.70 p = prospecfve controlled obersevafonal trial Methods 25 pafents with sarcoidosis were compared with 21 healthy subjects Spruit et al., 2005

64 Muscle force and exercise capacity in pa+ents with sarcoidosis compared to healthy subjects Sarcoidosis Healthy r = 0.61 p = r = 0.70 p = PImax QF 6MWD VO 2 max PI Spruit et al., 2005

65 Effects of 3 months pulmonary rehabilita+on RehabilitaFon Control Spruit et al., 2005

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