TABLE 1

Summary of all studies included in the review

Study [ref.]	Country	Year	Study type	Clinical diagnosis standards (CDS)	CDS year	Measurement instrument	Population characteristics
Study [ref.]	Country	Year	Study type	Clinical diagnosis standards (CDS)	CDS year	Measurement instrument	Sample size	Male sex	Mean age	Mean FVC%
De Vries et al. [77]	Netherlands	2000	Cohort study	NR	NR	SGRQ WHOQOL	10	40	61.1	79.6
Martinez et al. [43]	Brazil	2000	Cross-sectional study	NR	NR	SF36	34	59	52.3	62.4
Clark et al. [78]	UK	2001	Prospective observational study	ATS/ERS	2000	SF36	50	66	67.7	80.9
De Vries et al. [31]	Netherlands	2001	Cross-sectional study	ATS/ERS	2000	WHOQOL	41	37	63.5	NR
Baddini et al. [38]	Brazil	2002	Cohort study	ATS/ERS	2000	SF36	30	60	58.6	61.9
Jastrzebski et al. [41]	Poland	2005	Cross-sectional study	ATS/ERS	2000	SF36	16	69	48.3	44.0
Nishiyama et al. [79]	Japan	2005	Prospective observational study	ATS/ERS	2000	SGRQ	41	85	64.0	76.6
Tomioka et al. [80]	Japan	2005	RCT	ATS/ERS	2000	SF36	22	NR	70.0	72.5
Tzanakis et al. [81]	Sweden	2005	Cross-sectional study	ATS/ERS	2000	SGRQ	25	80	66.0	68.8
Antoniou et al. [82]	Greece	2006	RCT	ATS/ERS	2000	SGRQ	50	84	66.9	71.4
Tomioka et al. [83]	Japan	2007	Cross-sectional and longitudinal study	ATS/ERS	2000	SF36	46	70	69.9	71.0
Zimmermann et al. [84]	Brazil	2007	Cross-sectional study	ATS/ERS	2000	SGRQ SF36	20	NR	61.5	70.4
Feltrim et al. [39]	Brazil	2008	Cross-sectional cohort study	NR	NR	SGRQ	7	57	54.1	NR
King et al. [85]	Multi-country	2008	RCT (BUILD-1)	ATS/ERS	2002	SGRQ SF36	154	73	65.2	67.8
Krishnan et al. [86]	USA	2008	Cross-sectional study	ATS/ERS	2000	SF36	39	54	67.7	68.9
Nishiyama et al. [87]	Japan	2008	RCT	ATS/ERS	2000	SGRQ	28	75	66.2	67.5
Peng et al. [88]	China	2008	Cross-sectional and longitudinal study	ATS/ERS	2000	SGRQ	68		64.0	66.0
Raghu et al. [89]	USA	2008	RCT	ATS/ERS	2000	SGRQ SF36	87	68	65.2	63.9
Han et al. [90]	USA	2009	Cross-sectional study	ATS/ERS	2000	SGRQ SF12	221	67	63.0	62.9
IPF Clinical Research Network et al. [91]	USA	2010	RCT (STEP-IPF)	ATS/ERS	2000	SGRQ EQ5D SF36	180	83	69.0	56.8
Jastrzebski et al. [42]	Poland	2010	Cross-sectional study	NR	NR	SGRQ SF36	30	80	52.2	41.2
Key et al. [92]	UK	2010	Cross-sectional study	ATS/ERS	2002	LCQ	NR	74	70.8	78.5
Lindell et al. [93]	USA	2010	Mixed method intervention study	ATS/ERS	2000	SF36	21	76	66.2	NR
Lutogniewska et al. [51]	Poland	2010	NR	NR	NR	SGRQ SF36	30	NR	NR	NR
Ozalevli et al. [94]	Turkey	2010	Prospective observational study	ATS/ERS	2000	SF36	15	67	62.8	71.6
Raghu et al. [95]	Multi-country	2010	RCT (BUILD-1)	ATS/ERS	2000	SGRQ	154	73	65.2	67.8
Swigris et al. [74]	USA	2010	Cohort study	ATS/ERS	2000	ATAQ-IPF	95	82	69.3	66.0
Swigris et al. [76]	USA	2010	RCT (BUILD-1)	ATS/ERS	2000	SGRQ SF36	100	73	65.1	66.97
du Bois et al. [96]	Multi-country	2011	RCT	ATS/ERS	2000	SGRQ	822	71	66.0	72.5
Elfferich et al. [49]	Netherlands	2011	Cross-sectional study	other	NR	WHOQOL	49	63	63.1	82.9
Jones et al. [97]	UK	2011	Case control study	ATS/ERS	2002	LCQ	NR	63	72.0	80.4
King et al. [98]	Multi-country	2011	RCT (BUILD-3)	ATS/ERS	2000	SF36 EQ5D	581	70	63.8	74.5
Kozu et al. [99]	Japan	2011	Prospective non-randomised open trial	ATS/ERS	2000	SF36	45	82	67.5	68.6
Kozu et al. [100]	Japan	2011	Prospective non-randomised, and uncontrolled study	ATS/ERS	2000	SF36	65	71	67.5	65.3
Mishra et al. [101]	India	2011	RCT	ATS/ERS	2002	SGRQ	NR	100	70.7	61.4
Rammaert et al. [102]	France	2011	Prospective observational study	ATS/ERS	2000	SGRQ SF36	NR	62	67.1	67.0
Swigris et al. [103]	USA	2011	Prospective observational study	ATS/ERS	2000	SF36	21	86	71.5	73.0
Verma et al. [45]	Canada	2011	Cross-sectional study	ATS/ERS	2000	SGRQ SF36	137	66	59.4	61.7
Horton et al. [50]	USA	2012	RCT	other	NR	SGRQ CQLQ	23	78	67.6	70.4
Nishiyama et al. [104]	Japan	2012	Retrospective cohort study	ATS/ERS	2000	SGRQ	87	89	66.3	75.0
Noth et al. [105]	USA	2012	RCT (ACE-IPF)	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D SF36	145	73	67.0	58.8
Raghu et al. [106]	USA	2012	RCT (PANTHER-IPF)	ATS/ERS/JRS/ALAT	2011	SGRQ SF36	155	75	68.4	70.7
Shulgina et al. [107]	UK	2012	RCT	ATS/ERS	2000	SGRQ EQ5D	181	72	71.6	70.7
Swigris et al. [108]	USA	2012	RCT (STEP-IPF)	ATS/ERS	2005	SGRQ SF36	180	83	69.0	56.8
Lechtzin et al. [109]	USA	2013	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ CQLQ	20	75	67.6	70.4
Mermigkis et al. [110]	Greece	2013	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SF36	23	78	68.2	76.2
Tzouvelekis et al. [111]	Greece	2013	Non-randomised Clinical Trial	ATS/ERS/JRS/ALAT	2011	SGRQ	14	86	64.4	74.2
Alhamad [112]	Saudi Arabia	2014	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SF36	58	57	62.9	65.2
Gaunaurd et al. [71]	USA	2014	RCT	ATS/ERS/JRS/ALAT	2011	SGRQI	21		68.6	60.5
IPF Clinical Research Network et al. [113]	USA	2014	RCT (PANTHER-IPF)	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D SF36	264	78	67.7	72.8
Kozu et al. [114]	Japan	2014	Prospective cross-sectional study	ATS/ERS/JRS/ALAT	2011	SF36	65	71	67.5	65.3
Lubin et al. [115]	USA	2014	Cohort study	ATS/ERS	2000	SF36	102	75	70.0	70.0
Morsi et al. [33]	Egypt	2014	Cross-sectional study	NR	NR	SGRQ	36	22	53.0	46.3
Richeldi et al. [116]	Multi-country	2014	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1061	79	66.8	79.6
Rifaat et al. [34]	Egypt	2014	Prospective observational study	ATS/ERS/JRS/ALAT	2011	SGRQ	30	27	54.4	51.9
Vainshelboim et al. [117]	Israel	2014	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	32	66	67.3	68.2
Bors et al. [118]	USA	2015	Prospective, cross-sectional study	ATS/ERS/JRS/ALAT	2011	SF36	46	63	64.6	61.7
Huang et al. [119]	China	2015	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	76	93	60.3	77.3
Mermigkis et al. [120]	Greece	2015	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SF36	55	NR	70.3	NR
Nathan et al. [121]	Multi-country	2015	RCT (CAPACITY)	ATS/ERS	2000	SGRQ	338	73	66.5	74.7
Raghu et al. [122]	Multi-country	2015	RCT	ATS/ERS	2000	SGRQ	126	80	65.0	67.2
Vainshelboim et al. [123]	Israel	2015	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	28	66	67.3	68.2
Atkins et al. [63]	UK	2016	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D KBILD	77	59	76.4	84.3
Bahmer et al. [124]	Germany	2016	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ SF12	41	75	67.1	75.4
Costabel et al. [125]	Multi-country	2016	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1061	79	66.8	79.6
Ferrara et al. [61]	Sweden	2016	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	KBILD	71	70	68.3	77.5
Hopkins et al. [47]	Canada	2016	Retrospective population-based study	ICD	NR	HUI2	8295	54	NR	NR
Kolb et al. [126]	Multi-country	2016	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1081	78	65.5	78.1
Kotecha et al. [127]	UK	2016	Cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ	75	58	76.4	83.6
Richeldi et al. [128]	Multi-country	2016	RCT (TOMORROW-1, INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1231	79	66.5	79.7
Taniguchi et al. [129]	Multi-country	2016	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	322	80	66.0	81.2
Yount et al. [48]	USA	2016	Prospective cohort study	Patient referred	NR	ATAQ-IPF PROMIS	220	70	61.0	NR
Yu et al. [46]	China	2016	RCT	CTS	NR	SGRQ	NR	NR	NR	NR
Arizono et al. [130]	Japan	2017	Prospective observational study	ATS/ERS	2002	SGRQ	22	64	70.5	72.2
Azuma et al. [131]	Japan	2017	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	126	81	68.3	81.9
Birring et al. [64]	Multi-country	2017	RCT	ATS/ERS/JRS/ALAT	2011	KBILD LCQ	24	63	67.6	73.0
Cheng et al. [132]	Canada	2017	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ	77	69	68.7	73.2
Collard et al. [133]	Multi-country	2017	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1061	79	66.8	79.6
Dowman et al. [52]	Australia	2017	RCT	NR	NR	SGRQI	61	67	71.4	75.9
Furukawa et al. [134]	Japan	2017	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ	182	85	65.6	79.7
Glaspole et al. [53]	Australia	2017	Observational study	NR	NR	SGRQ	422	67	71.3	81.0
Koyama et al. [135]	Japan	2017	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	Bartel Index	47	89	75.5	75.4
Kreuter et al. [136]	Germany	2017	Observational study	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D	623	77	69.6	67.5
Lee et al. [137]	South Korea	2017	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ	112	84	70.0	85.0
Magnani et al. [35]	Italy	2017	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	PGWBI	18	45	66.5	62.4
Matsuda et al. [138]	Japan	2017	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ	121	82	66.8	81.1
Matsuda et al. [139]	Japan	2017	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ COPD	106	85	67.1	81.9
Natalini et al. [140]	USA	2017	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	SF36	50	78	70.8	70.3
Root et al. [141]	USA	2017	Observational study	ATS/ERS/JRS/ALAT	2011	SF36 LCQ	188	56	65.8	73.9
Sokai et al. [142]	Japan	2017	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ SF36	52	85	72.0	86.1
Swigris et al. [143]	Multi-country	2017	RCT (TOMORROW-1)	ATS/ERS	2000	SGRQ	426	75	65.1	81.3
Wapenaar et al. [65]	Multi-country	2017	Prospective cohort study	ATS/ERS	2000	SGRQ KBILD EQ5D	108	78	70.5	72.5
Atkins et al. [66]	UK	2018	Prospective observational study	ATS/ERS/JRS/ALAT	2011	KBILD	39	69	75.1	82.7
Bacci et al. [144]	USA	2018	Cross-sectional qualitative study and secondary quantitative analysis of clinical trial data	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D	30	60	67.7	70.2
Capparelli et al. [70]	Argentina	2018	Cohort study- instrument validation	ATS/ERS/JRS/ALAT	2011	SGRQI	23	78	71.9	68.9
da Fontoura et al. [40]	Brazil	2018	Retrospective cohort study	NR		SF36	31	60	57.1	49.0
Grufstedt et al. [145]	Sweden	2018	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ COPD	87	62	70.1	84.21
Kolb et al. [146]	Multi-country	2018	RCT (INSTAGE)	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D	273	79	70.2	67.0
Mavroudi et al. [147]	Greece	2018	Case control study	ATS/ERS	2000	SF36	19	58	69.8	75.6
Nishiyama et al. [148]	Japan	2018	Prospective observational study	ATS/ERS/JRS/ALAT	2011	SGRQ	31	74	72.3	73.8
Nolan et al. [53]	UK	2018	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	KBILD	65	89	72.0	73.2
Raghu et al. [149]	USA	2018	RCT (WRAP-IPF)	ATS/ERS/JRS/ALAT	2011	SGRQ	58	82	69.9	75.4
Rajala et al. [150]	Finland	2018	Prospective observational study	ATS/ERS/JRS/ALAT	2011/2015	RAND36	92	73	75.0	78.0
Rosas et al. [151]	USA	2018	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	58	81	67.6	71.1
Swigris et al. [152]	Multi-country	2018	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ EQ5D	1081	79	66.8	79.6
Szentes et al. [54]	Germany	2018	Prospective observational study	NR	NR	KBILD EQ5D	268	86	63.2	77.4
Takeda et al. [153]	Japan	2018	Prospective cohort study	ATS/ERS/JRS/ALAT	2015	COPD	25	84	74.4	80.6
Vancheri et al. [154]	Multi-country	2018	RCT (INJOURNEY)	ATS/ERS/JRS/ALAT	2011	EQ5D	104	86	68.9	84.0
Wuyts et al. [155]	Multi-country	2018	Prospective observational study	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D	277	77	69.6	80.6
Bosi et al. [156]	Italy	2019	Prospective observational study	ATS/ERS/JRS/ALAT	2011	SGRQ	47	76	68.7	75.5
Chehere et al. [157]	France	2019	Prospective cohort study	ATS/ERS	2001	SF36	19	75	65.0	75.0
Eken et al. [158]	Turkey	2019	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	SGRQ	40	78	65.5	86.6
Justice et al. [159]	USA	2019	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	14	88	70.8	NR
Kalafatis et al. [69]	Sweden	2019	Observational study	ATS/ERS/JRS/ALAT	2011	KBILD	348	72	72.0	70.2
Kimman et al. [57]	Netherlands	2019	Prospective cohort study	NR	NR	PESaM EQ5D	188	76	69	NR
Kreuter et al. [55]	Germany	2019	Observational study	NR	NR	SGRQ EQ5D	424	77	68.7	36.1
Nolan et al. [56]	UK	2019	Prospective cohort study	NR	NR	KBILD	209	61	70.0	71.9
Pan et al. [44]	China	2019	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	SGRQ ATAQ-IPF	20	70	58.3	NR
Prior et al. [73]	Denmark	2019	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQI SGRQ KBILD	150	81	72.9	87.2
Ryerson et al. [160]	Multi-country	2019	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1060	79	66.8	79.6
Sinha et al. [36]	UK	2019	Prospective cohort study	NR	NR	KBILD	57	33	62.0	80.0
Wallaert et al. [161]	France	2019	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	VSRQ	61	75	66.8	68.6
Witt et al. [162]	Multi-country	2019	Prospective observational study	ATS/ERS/JRS/ALAT	2011	SF36	258	73	67.3	62.7
Xu et al. [163]	China	2019	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	101	54	64.3	80.8
Yuen et al. [164]	USA	2019	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	20	65	69.8	66.0
Bloem et al. [37]	Netherlands	2020	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	EQ5D QoL-RIQ	59	45	73.0	83.2
Case et al. [165]	USA	2020	Retrospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D SF12	662	75	70.0	69.9
Janssen et al. [166]	USA	2020	RCT	ATS/ERS/JRS/ALAT	2011	SGRQ	22	71	90.9	73.4
Kreuter et al. [167]	Multi-country	2020	RCT (INPULSIS-1, INPULSIS-2)	ATS/ERS	2000	SGRQ	1061	79	66.8	79.6
Moor et al. [67]	Netherlands	2020	RCT	ATS/ERS/JRS/ALAT	2018	PESaM EQ5D KBILD	90	91	71.0	80.1
O'Brien et al. [168]	USA	2020	Cross-sectional study	ATS/ERS/JRS/ALAT	2011	SGRQ EQ5D SF12	859	74	70.0	69.7
Prior et al. [72]	Denmark	2020	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQI SGRQ KBILD	150	81	72.9	87.2
Richeldi et al. [169]	Multi-country	2020	RCT (INPULSIS-1, INPULSIS-2, INSTAGE)	ATS/ERS ATS/ERS/JRS/ALAT	2000 2011	SGRQ	1197	79	67.1	78.0
Tzouvelekis et al. [68]	Greece	2020	Prospective cohort study	ATS/ERS/JRS/ALAT	2011	SGRQ KBILD	98	83	70.8	77.0

All means are calculated pooled means. ACE-IPF: Anti-Coagulant Effectiveness in Idiopathic Pulmonary Fibrosis; ATS/ERS: American Thoracic Society/European Respiratory Society; ATS/ERS/JRS/ALAT: American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society; ATAQ-IPF: A Tool to Assess Quality of life in IPF; BUILD: Bosentan Use in Interstitial Lung Disease; CQLQ: The Cough Quality of Life Questionnaire; CTS: Chinese Thoracic Society; EQ5D: EuroQol; FVC: Forced vital capacity; HUI2: Health Utilities Index Mark 2; IPF: idiopathic pulmonary fibrosis; K-BILD: King's Brief Interstitial Lung Disease; LCQ: Leicester Cough Questionnaire; NR: not reported; PANTHER Prednisone, Azathioprine, and N-acetylcysteine in Participants With IPF; PESaM: Patient Experiences and Satisfaction with Medications Questionnaire; PGWBI: The Psychological General Well-Being Index; PROMIS: Participant Reported Outcomes Measurement Information System; QoL-RIQ: Quality-of-Life for Respiratory Illness; RAND36: Research and Development 36 item health survey; RCT: Randomised controlled trial; SF12: Short Form-12; SF36 Short Form-36; SGRQ: St George's Questionnaire; SGRQI: IPF-specific version of the SGRQ; STEP-IPF: Sildenafil Trial Of Exercise Performance In Idiopathic Pulmonary Fibrosis; VSRQ: Visual Simplified Respiratory Questionnaire; WHOQoL: WHO Quality of Life Questionnaire; WRAP-IPF: Treatment of IPF With Laparoscopic Anti-Reflux Surgery.