TABLE 1

Description of studies included in the final screening

First author [ref.]YearCompleted n (% of initial)Respiratory rehabilitation programmeExercise testPEDro score
Add-on interventionComponents of trainingExercise training sessions n
Additional exercise modalities
 Benton [9]201319 (100)Single set of resistance trainingCy, Tr166MWT4
 Vonbank [10]201224 (100)Strength trainingCy24CPET3
 Bernard [11]199936 (80)Strength trainingCy36CPET+5
 Dourado [12]200924 (73)Strength trainingTr, Cal366MWT4
 Phillips [13]200619 (unclear)Strength trainingCy, Tr, Cal, PE136MWT4
 Mador [14]200424 (unclear)Strength trainingCy, Tr24CPET, 6MWT6
 Würtemberger [15]200124 (100)Strength trainingCy, PENRCPET, 6MWT4
 Alexander [16]200820 (75)Strength trainingCy, Tr166MWT3
 Holland [17]200438 (100)Upper and lower limb strengthCy, Tr126MWT7
 Subin [18]201017 (100)Upper and lower limb strengthTr206MWT4
 Sívori [19]199828 (65)Upper and lower limb strengthCy24CPET, 12MWT4
 Costi [20]200946 (92)Upper and lower limb strengthTr, Cal, St156MWT§8
 Vivodtzev [21]#200617 (100)NMESTr, Cal, St166MWT5
 Rooyackers [22]200324 (100)Eccentric cyclingCy, St50CPET, 6MWT4
 Gloeckl [23]201272 (88)Whole body vibrationCy, Cal, St, PE, Nut156MWT§,ƒ6
NIV
 Johnson [24]200222 (79)Bi-level NIVTr, PE12CPETƒ4
 Toledo [25]200718 (100)Bi-level NIVTr36CPET3
 Reuveny [26]#200519 (79)Bi-level NIVTr16CPET6
 Costes [27]200314 (100)Bi-level NIVCy24CPET, endurance##4
van’t Hul [28]#200621 (72)Inspiratory supportCy24ISWT, endurance8
 Hawkins [29]200219 (66)Proportional assisted ventilationCy18CPET, endurance5
 Bianchi [30]200219 (58)Proportional assisted ventilationCy, St, PE, Nut18CPET, 6MWT5
 Garrod [31]200037 (82)Nocturnal NIVCy, Tr, St, PE16ISWT§,ƒ6
 Duiverman [32]#200862 (86)Nocturnal NIVCy, Tr, St, IMT, PE, Nut36, plus 69 nights of NIV6MWT, endurance, CPET4
Oxygen
 Scorsone [33]201030 (100)Supplementary oxygenCy24CPET, enduranceƒ5
 Emtner [34]#200329 (100)Supplementary oxygenCy, PE21CPET, endurance7
 Garrod [35]#200022 (88)Supplementary oxygenCy, Tr, Cal, PE18ISWT8
 Wadell [36]#200120 (100)Supplementary oxygenTr246MWT6
 Dyer [37]#201247 (85)Supplementary oxygenTr, St, PE14ESWT§,ƒ6
 Rooyackers [38]#199724 (unclear)Supplementary oxygenCy, Cal, St, PE50CPET, 6MWT5
 Bjørgen [39]200912 (79)Supplementary oxygenCy24CPETƒ3
 Ringbaek [40]#201338 (84)Continuous oxygen supplementCy, Tr, PE14ESWTƒ5
Heliox
 Johnson [24]200221 (88)Supplementary helioxTr, PE12CPETƒ4
 Eves [41]200931 (82)Supplementary helioxCy, Tr, Cal, St, PE16CPET, enduranceƒ9
 Scorsone [33]201030 (100)Supplementary helioxCy24CPET, enduranceƒ5
Prescription medications
 Pasqua [42]#201022 (100)TiotropiumCy, Tr, Cal, IMT206MWT3
 Ambrosino [43]2008120 (68)TiotropiumTr246MWTƒ8
 Casaburi [44]200591 (84)TiotropiumTr24Endurance§6
 Creutzberg [45]200363 (100)Anabolic steroidsCy40CPET10
 Ferreira [46]#199817 (100)Anabolic steroidsCy, IMT40CPET, 6MWT##7
 Miki [47]#201229 (100)GhrelinCy, Cal, PE456MWTƒ9
 Burdet [48]#199716 (100)Growth hormoneAero NR36CPET, 6MWT##8
 Miki [49]#201320 (100)GhrelinCy, Cal, PE15CPET¶¶9
 Hornikx [50]201249 (98)Vitamin D supplementCy, Tr, St, PE36CPET¶¶, 6MWT8
 Blanco [51]#201341 (85)SildenafilCy, St36CPET, enduranceƒ, 6MWT8
 Satta [52]199120 (unclear)UbidecarenoneTrNRCPET6
 Valderramas [53]200964 (94)Hypertonic salineTr, Cal, St246MWTƒ,##8
Nutrition
 Laviolette [54]201020 (92)Whey proteinCy, St24Endurance6
 Sugawara [55]201226 (83)MEIN (whey protein)Tr, Cal, IMT, PENR6MWT8
 Steiner [56]200360 (70)Carbohydrates, protein and fatTr, Cal, PE14ISWTƒ, ESWT8
 Gurgun [57]#201330 (100)Carbohydrates, protein and fatCy, Tr, St16ISWT, 6MWT4
 Broekhuizen [58]200580 (78)Polyunsaturated fatCy, Tr, PENRCPET+7
 Deacon [59]200880 (80)Creatine monohydrateCy, Tr, St, PE21ISWTƒ, ESWT7
 Faager [60]200623 (100)Creatine monohydrateCy, Cal, St, PE16ESWT5
 Fuld [61]200525 (66)Creatine monohydrateCy, Cal, St, PE16CPET, ISWTƒ7
 Menier [62]200160 (100)Branched-chain amino acidCy, Cal, St30CPET3
 Borghi-Silva [63]200616 (unclear)L-carnitineTr, IMT18CPET, 6MWT§5
Breathing exercises
 Magadle [64]200727 (87)Inspiratory muscle trainingCy, Tr, St1086MWT7
 Mador [65]200529 (76)Inspiratory muscle trainingCy, Tr, Cal, PE24CPET, 6MWT5
 Larson [66]199928 (unclear)Inspiratory muscle trainingCy80CPET5
 Berry [67]199616 (94)Inspiratory muscle trainingTr, St36, plus 96 IMT sessionsCPET, endurance, 12MWT5
 Wanke [68]199442 (unclear)Inspiratory muscle trainingCy32, plus 56 IMT sessionsCPET4
 Weiner [69]199224 (unclear)Inspiratory muscle trainingCy, St72Endurance, 12MWT§5
 Dekhuijzen [70]#199140 (100)Inspiratory muscle trainingCy, Tr, St100CPET, 12MWT5
 Kunikoshita [71]200615 (100)Inspiratory muscle trainingTr, Cal18CPET4
 Sykes [72]200537 (93)Inspiratory muscle trainingCy, St40CPET, 6MWT7
 Goldstein [73]198911 (100)Inspiratory muscle trainingAero NRExercise training++ plus 40 IMT sessionsEndurance, 6MWT5
 Collins [74]200833 (79)Ventilation feedbackCy, Tr36CPET, endurance5
van Gestel [75]201240 (100)Controlled breathingCy, St106MWT6
Other
 Carrieri-Kohlman [76]199651 (100)Coaching versus monitoringTr12CPET, 6MWT5
 Zanotti [77]201220 (100)OsteopathyCy, St, PE206MWT9
 Alexander [78]201227 (unclear)Harmonica playingCy, Tr166MWT4
de Godoy [79]200330 (100)PsychotherapyCy, Tr, Cal, St246MWT5
 Sharifabad [80]201063 (100)Written disclosure therapyCy, Tr, St, PE246MWT4
 Norweg [81]200533 (89)Activity training/lecturesTr, Cal15, plus ∼1 h·week−16MWT3
 Deering [82]201141 (100)AcupunctureTr14ISWT6
  • NIV: noninvasive ventilation; Cy: cycling; Tr: treadmill; 6MWT: 6-min walk test; CPET: cardiopulmonary exercise testing; Cal: calisthenics; PE: psychological or educational support; NR: not reported; 12MWT: 12-min walk test; St: strengthening; NMES: neuromuscular electrical stimulation; Nut: nutritional support; endurance: cycle endurance test; ISWT: incremental shuttle walk test; IMT: inspiratory muscle training; ESWT: endurance shuttle walk test; Aero NR: no aerobic training details reported. #: exemplar studies of patient-centred care; : study included two appropriate interventions; +: statistically significant effect favouring intervention for peak work rate outcome; §: statistically significant effect favouring intervention; ƒ: outcomes with adequate statistical power, according to original publication; ##: statistically significant effect favouring control; ¶¶: statistically significant effect favouring intervention for peak oxygen uptake outcome; ++: training duration of 4 weeks, frequency not reported. Bold indicates statistically significant effects.