Background
Methods
Aim
Design
Search strategy
Selection
Study population
Types of studies included
Types of outcomes included
Data extraction
Data analysis
Results
Overview of included studies
Author/Year | Aim | Study Design and symptom assessment used | Participants (n) | Diagnosis | Diagnosis method | Baseline % predicted lung function mean (SD) | Symptoms prevalence |
---|---|---|---|---|---|---|---|
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% |
Symptom prevalence
Respiratory symptoms
Depression
Digestive tract symptoms
Sleep related symptoms
Anorexia, weight loss, fatigue
Pain
Other symptoms
Discussion
Symptoms | PIF-ILD | Cancer | AIDS | CHF | COPD | ESRD |
---|---|---|---|---|---|---|
Pain | 9% | 30–94% | 30–98% | 14–78% | 21–77% | 11–93% |
Depression | 10–49.2% | 4–80% | 17–82% | 6–59% | 17–77% | 2–61% |
Anxiety | 22–58% | 3–74% | 13–76% | 2–49% | 23–53% | 7–52% |
Fatigue | 7.6–29% | 23–100% | 43–95% | 42–82% | 32–96% | 13–100% |
Breathlessness | 54.7–98% | 16–77% | 43–62% | 18–88% | 56–98% | 11–82% |
Insomnia | 6–46.6% | 3–67% | 40–74% | 36–48% | 15–77% | 1–83% |
Nausea | 13% | 2–78% | 41–57% | 2–48% | 4% | 8–52% |
Diarrhea | 2% | 1–95% | 29–53% | 12% | 8–36% |