Table 1 Summary of studies included

Akhtar 2013 [33]

To assess the presence of depressive symptoms

Prospective study

Wakefield Self-assessment of Depression Inventory score ≥ 15 screening tool

Outpatients (n = 118)

IPF

High resolution computed tomography, Lung biopsy

Not available

Depression 49.2%

Alhamad 2008 [18]

Describe the clinical course and prognosis of IPF among Middle Eastern patients, and to attempt to identify variables that would predict prognosis.

Retrospective study Chart reviews, telephone interviews

Hospital patients (n = 61)

IPF

ATS/ERS criteria

FVC 64.8 (21.6)^a

Dyspnoea 93%; Cough 82%; Weight loss 12%

Araki 2003 [17]

To investigate the outcome of IPF in elderly patients whose pathological diagnosis corresponded to usual interstitial pneumonia on autopsy findings.

Retrospective study

MRC dyspnoea scale, medical records

Patients older than 65y, based on histological findings on autopsy, complete medical records (n = 86)

UIP, IPF

Lung biopsy: Histological findings consistent with UIP, IPF

VC 72.6 (25.2)

DLCO 62.8 (30.1)

Dyspnoea 54.7% Cough 93.2%

Bajwah 2012 [19]

To compare the palliative care needs, treatments, and end-of-life preferences of PIF-ILD patients

Retrospective study Medical records

Outpatients Hospital; Ages 37–99 (n = 45)

PIF-ILD

ATS/ERS criteria

Not available

Dyspnoea 93%; Cough 60%; Fatigue 29%; Insomnia 6%; Depress ion/anxiety 22%; Anorexia/weight loss 18%; Chest pain 29%; Generalized pain 9%; Dyspepsia 4%; Polyuria/polydipsia 4%; Diarrhea 2%; Dysphagia 2%

Bandeira 2009 [20]

To determine prevalence of GERD and to evaluate its clinical presentation

Prospective study General questionnaire, Quality of Life Scale for Gastroesophageal Reflux Disease

Outpatients (n = 28)

IPF

ATS/ERS criteria in 11 patients, lung biopsy 17 patients

FVC 66.6 (16.0) DLCO 44.5 (22.0)

Heartburn 29%; Nocturnal heartburn 14%; Regurgitation 40%; Nocturnal regurgitation 18%; Epigastric pain 18%; Dysphagia 11%; Cough 77%; Nocturnal cough 37%; Dysphonia 11%; Chest pain 25%

D’Ovidio 2005 [13]

To determine the prevalence of gastroesophageal reflux in lung transplant candidates

Interviews and Esophageal manometer.

Outpatients (n = 26)

IPF

Not specified

FVC median (range) 67 (33–96) DLCO median (range) 40 (13–77)

Heartburn Regurgitation Dysphagia 65%

Hashemi Sadraei 2013 [21]

To evaluate the clinical characteristics of IPF patients from The National Research Institute of Tuberculosis and Lung Diseases

Retrospective descriptive study Medical records and interviews

(n = 132)

IPF

Clinical presentation, radiographic and or/pathological findings ATS criteria

Not available

Breathlessness 68.2%; Cough 60.6%; Chest pain 8.3%; Fatigue 7.6%

Hoppo 2012 [14]

To determine the prevalence of GERD and assess the proximity of reflux events in patients with histologically proven IPF

Retrospective study

(n = 35)

IPF

Lung biopsy

Not available

Cough 74%; Heartburn 25%; Regurgitation 25%

Jeon 2006 [22]

To investigate the prognostic factors at initial presentation and the causes of death in Korean patients with IPF

Retrospective study Medical records

Outpatients (n = 88)

IPF

Surgical lung biopsy compatible with UIP, ATS criteria

FVC 74.0 (19.2) DLCO 65.2 (21.4)

Exertional dyspnoea 89%

Lancaster 2009 [31]

To analyze obstructive sleep apnea in clinically stable patients with IPF

Epworth sleepiness scale (ESS) ≥10 consistent with daytime sleepiness

(n = 35)

IPF

ATS criteria (2000)

FVC 68.8 (13.7)^a

Daytime sleepiness 25%

Lindell 2010 [34]

To test the ability of a complex intervention (PRISM) to decrease symptom burden, stress and improve HRQoL perceptions of patients with IPF and their carers.

Nested mixed method design (experimental, qualitative) Beck Anxiety Inventory, Beck Depression Inventory-II

Outpatients (n = 37)

IPF

Biopsy and/or High resolution computed tomography

70% FVC > 55 15% FVC 50–55 15% FVC < 50%

Anxiety 58% Depression 4 (10%)

Mermigkis 2009 [32]

To describe sleep quality associated to daytime consequences in IPF

Cross-sectional control study Epworth Sleepiness Scale Pittsburgh Sleep Quality Index Functional Outcomes in Sleep Questionnaire Fatigue Severity Scale Polysomnography Interview

Outpatients (n = 15)

IPF

ATS/ERS criteria or lung biopsy

FVC 77.4 (21.2) DLCO 56.3 (17.8)

Daytime sleepiness 20%; Snoring 40%; Insomnia 46.6%; Witnessed apnoea’s 13.3%

Mermigkis 2007 [28]

To describe the clinical and polysomnographic features of SRBD and to identify predictors of OSA in IPF patients

Retrospective study Cleveland Clinic Sleep Disorders Questionnaire, Epworth Sleepiness scale, Polysomnography

Outpatients (n = 18)

IPF

ATS/ERS criteria

FVC 65.7 (10.4) DLCO 49.9 (15.3)

Excessive daytime sleepiness 77.7%; Snoring 88%; Daytime fatigue 61%; Witnessed apnoea’s 44.4%

Ohno 2007 [23]

Not specified

Retrospective Clinical personal records

(n = 1322) Patients covered by public insurance

IIP

Medical records: 12% pathological diagnosis from lung biopsy, rest clinical findings (respiratory function test, images, serology)

Not available

Cough 94%; Exertional dyspnoea 98%

Patti 2005 [15]

To determine the prevalence of GERD, the clinical presentation of GERD and reflux profiles in patients with IPF

Patients rated severity of symptoms 5 point scale (0 = no symptom to 4 = disabling symptom)

Outpatients (n = 18)

IPF

Not specified

Not available

Heartburn 55%; Regurgitation 33%; Cough 83%

Raghu 2006 [29]

To assess the prevalence and clinical symptoms of GER in patients with IPF and compare findings to patients with intractable asthma manifesting symptoms of GER.

Prospective study 24 h oesophageal pH probe, oesophageal manometry, symptom questionnaire form

Outpatients (n = 65)

IPF

ATS criteria

FVC 59.9 (20.0)^a DLCO 34.8 (15.7)^a

Belching 51%; Heartburn 47%; Regurgitation 16%; Abdominal pain 7%; Bloating 27%; Chest pain 24% Choking 13%; Globus 13%; Hoarseness 31%; Liquid dysphagia 7%; Solid dysphagia 16%; Odynophagia 4%; Nausea 13%

Ryerson 2012 [35]

To investigate the prevalence of clinically meaningful depress ion at baseline, characterize the association of depression with patient and disease specific variables, and describe the natural history of depress ion over a period of 6 months

Cohort

Outpatients (n=)52

ILD (21 with IPF)

ATS/ERS criteria

FVC 74.3 (18.5) DLCO 50.8 (16.3)

Depression 24%

Schoenheit 2011 [24]

To generate in depth insights regarding the patient journey, including symptoms triggers to seeking medical care, referral patterns, initial diagnoses, follow up and current disease management.

Qualitative Interviews conducted in the participants at home

Outpatients (n = 45)

IPF

Physician confirmed diagnosis

Not available

Exertional dyspnoea 68%; Cough 59%; Fatigue 28%; Chest pain 6%; Weight loss 2%

Sweet 2007 [16]

To determine the prevalence of distal and proximal reflux, the oesophageal manometric profile and whether or not reflux symptoms could be used to screen for reflux

Retrospective Study Standardized interview with a physician or technician. Patients rated severity of symptoms 5 point scale (0 = no symptom to 4 = disabling symptom)

Outpatients (n = 30)

IPF

Pathological findings in 25 patients, ATS/ERS criteria in 5 patients

Not available

Heartburn 48%; Regurgitation 43%; Dysphagia 30%

Tobin 1998 [25]

To investigate the possible association of GER and IPF

Qualitative study Structured interview

Outpatients (n = 17)

UIP

Lung biopsy compatible with UIP

DLCO mean (range) 35.9 (9–62)

Cough 100%

Von Plessen 2003 [26]

To study the incidence and prevalence of physician diagnosed and hospitalized cryptogenic fibrosing alveolitis in a well-defined adult population in Norway

Retrospective study Registration form, hospital registers (2 physicians extracted the information)

Hospital patients 158 incident cases (1984–1998) and 61 prevalent cases (until 31.12. 1998)

CFA

Progressive dyspnoea, crackles on auscultation and bilateral shadowings on chest X-ray with no exposure to a known fibrogenic agent

83 and 80% of incident and prevalent cases TLCO < 80% predicted

Incident cases dyspnoea 87%; Prevalent cases 79%

Aksu 2014 [30]

To investigate the possibility that IPF is involved in the pathogenic of GERD

Prospective study

Outpatients (N = 21)

IPF

Pulmonary function tests (spirometry, carbon monoxide diffusion capacity, alveolar volume), study of BAL fluid (cell count and lymphocyte subsets, IL-1 β, TNF-α)

FVC 94.9 (11.2)^a TLCO 114.1 (16.7)

Reflux symptoms 52.4% Severe dysphagia 23.8% Epigastric pain 91%

Huang 2014 [27]

To describe the clinical features and prognosis of microscopic polyangiitis (MPA) patients whose initial respiratory presentation was pulmonary fibrosis

Retrospective study Hospital computer-assisted search

Hospital patients MPA cases (N = 67)

IPF patients (N = 19)

Radiological findings (CT), clinical manifestations consistent with UIP pattern according to the ATS/ERS/JRS/ALAT statement 2011

DLCO range 30–76

Of IPF patients: Cough 84.2% Sputum 68.4% Hemoptysis 21.1% Dyspnoea 78.9%