Dyspnea in Idiopathic Pulmonary Fibrosis: A Systematic Review

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1 Vol. 43 No. 4 April 2012 Journal of Pain and Symptom Management 771 Review Article Dyspnea in Idiopathic Pulmonary Fibrosis: A Systematic Review Christopher J. Ryerson, MD, DorAnne Donesky, PhD, RN, Steven Z. Pantilat, MD, and Harold R. Collard, MD Department of Medicine (C.J.R., S.Z.P., H.R.C.), School of Medicine, and Department of Physiological Nursing (D.D.), School of Nursing, University of California at San Francisco, San Francisco, California, USA Abstract Context. Little is known about the treatment and correlates of dyspnea in idiopathic pulmonary fibrosis (IPF). Objectives. The objective of this systematic review was to summarize the literature regarding the treatment and correlates of dyspnea in IPF. Methods. MEDLINE, EMBASE, and all Evidence-Based Medicine Reviews were searched for publications that evaluated treatment or correlates of dyspnea in IPF. Reference lists and recent review articles also were searched. Results. The heterogeneity of included studies did not permit meta-analysis. Dyspnea improved in studies of sildenafil, pulmonary rehabilitation, and prednisone with colchicine. Additional studies of these three treatments, however, found discordant results. One study suggested that assisted ventilation delivered by facemask improved exertional dyspnea. Oxygen and opioids improve dyspnea in other chronic lung diseases, but data in IPF are limited. Correlates of dyspnea included functional and physiological measures and comorbid diseases. Conclusion. Sildenafil and pulmonary rehabilitation should be considered as potential therapies for dyspnea in selected patients with IPF. Supplemental oxygen and opioids may be additional potential therapies; however, the evidence supporting their use is weak. Additional research should focus on the management of functional status and comorbidities as potential treatments for dyspnea. J Pain Symptom Manage 2012;43:771e782. Ó 2012 U.S. Cancer Pain Relief Committee. Published by Elsevier Inc. All rights reserved. Key Words Dyspnea, idiopathic pulmonary fibrosis, systematic review Address correspondence to: Christopher J. Ryerson, MD, 505 Parnassus Avenue, Box 0111, San Francisco, CA 94143, USA. chris.ryerson@hli.ubc.ca Accepted for publication: April 27, Ó 2012 U.S. Cancer Pain Relief Committee Published by Elsevier Inc. All rights reserved. Introduction Interstitial lung disease (ILD) is a diverse group of conditions that are characterized by inflammation and fibrosis of the pulmonary parenchyma. Idiopathic pulmonary fibrosis (IPF) is one of the most common ILDs, with /$ - see front matter doi: /j.jpainsymman

2 772 Ryerson et al. Vol. 43 No. 4 April 2012 a prevalence of up to 227 per 100,000 among those aged 75 years or older. 1 IPF is characterized by relentless progression, with a three-year mortality of up to 50%. 2,3 Because of these features, IPF has been the focus of most of the clinical research conducted in ILD. IPF is a disease with high morbidity, with 90% of patients reporting dyspnea at the time of diagnosis. 2 The clinical significance of dyspnea in IPF is illustrated by its strong correlation with quality of life (QOL) 4 and mortality. 5 Relief of dyspnea is, therefore, an important goal in the management of IPF. Disease-modifying pharmacotherapy is of marginal benefit, 6,7 highlighting the need for complementary symptom-based management strategies. One such approach is to investigate the treatment of correlates of dyspnea, as some of these correlations also may represent causation. Common management approaches to dyspnea in IPF include pulmonary rehabilitation, supplemental oxygen, and opioids. 8 In this article, we review the evidence for these and other treatments of dyspnea in IPF and provide recommendations for dyspnea management based on the available evidence. We also review the correlates of dyspnea in IPF and suggest that directing treatment toward independent correlates of dyspnea could improve symptoms. Methods Data Sources and Searches We performed a systematic search of the literature using MEDLINE, EMBASE, and all Evidence-Based Medicine Reviews. The search was designed to capture all studies relating to both dyspnea and IPF. Search terms for dyspnea included dyspn* and breath* to return all variations of the terms dyspnea, breathlessness, and shortness of breath. The medical subject heading in each database for pulmonary fibrosis was used to capture all studies relevant to IPF. Additional search terms included IPF, lung fibrosis, fibrosing alveolitis, idiopathic interstitial pneumon*, usual interstitial pneumon*, UIP, and IIP. No limits were applied. Details of the search strategy are available in Appendix I at jpsmjournal.com. We considered articles and conference abstracts published from database inception through November 2010 (MEDLINE from 1950, EMBASE from 1980). In addition, we examined bibliographies of retrieved articles and other major review articles to identify additional articles. The literature search was performed independently by two authors (C. J. R. and D. D.) using predefined criteria. All discrepancies were resolved by iteration and consensus. Study Selection Studies were eligible if they were published in English and reported treatments or correlates of dyspnea in 10 or more adults with IPF. There was no restriction on study design, quality, duration, or type of treatment. Studies were included if the diagnosis of IPF was based on the American Thoracic Society/European Respiratory Society diagnostic criteria or if equivalent criteria were described. 9,10 Studies that included subjects with other forms of ILD were acceptable if subjects with IPF were reported separately. Studies published only in abstract form were included if sufficient information was available to satisfy inclusion criteria. Data Extraction and Quality Assessment Data were extracted from selected studies using data abstraction forms with predefined criteria. Two authors (C. J. R. and D. D.) collected information regarding study design, subject characteristics, and study results as they pertained to the prespecified endpoints. Discrepancies were resolved by iteration and consensus. Trial quality was assessed using the Cochrane Collaboration s tool for assessing methodological quality and risk of bias. 11 Data Synthesis and Analysis We compared baseline study characteristics using Chi-squared or t-tests. For treatment results, we reported the available data for treatment approaches that were identified in the search or were determined a priori to be commonly used in clinical practice (i.e., pulmonary rehabilitation, supplemental oxygen, and opioids). Two authors (C. J. R. and H. R. C.) used the grading system developed by the Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) working group to provide a recommendation (for or against, weak or strong). 12 This system

3 Vol. 43 No. 4 April 2012 Dyspnea in Idiopathic Pulmonary Fibrosis 773 grades the strength and quality of the available evidence, and these grades may change as more and better quality data become available. For correlates of dyspnea, we report ranges for the correlation coefficients (r values). Data were grouped for some variables (e.g., actual and percent predicted forced vital capacity) to facilitate reporting and interpretation. The sign of the correlation coefficient was reversed for variables associated with the baseline dyspnea index, the dyspnea component of the Chronic Respiratory Questionnaire, and the oxygen cost diagram because decreasing scores on these metrics correspond to increasing dyspnea severity. Thus, for the remainder of this review a positive correlation coefficient indicates that an increase in the value of a variable corresponds to increased dyspnea. Results The initial search revealed 3490 peerreviewed citations; 122 citations were identified for full-text review (Fig. 1). Of these, 26 studies satisfied inclusion criteria. Examination of reference lists and other major review articles yielded three additional studies. A total of 29 studies were included in the final review, including 14 studies that evaluated potential treatments of dyspnea 6,13e25 and 15 studies that evaluated correlates of dyspnea. 26e40 The heterogeneity of the selected studies precluded meta-analysis. Dyspnea severity was measured with nine metrics (Appendix II at jpsmjournal.com). Only the dyspnea component of the Composite Clinical, Radiographic, and Physiological scoring system was designed specifically for use in an ILD population. 41 Most metrics were developed in populations with chronic obstructive pulmonary disease (see references in Appendix II). Treatment of Dyspnea Fourteen studies evaluated the treatment of dyspnea in IPF (Table 1). Study quality was variable, with the Cochrane methodological quality score ranging from 3 to 6 out of 6 for the seven randomized trials. The evidence for potential treatments of dyspnea is summarized below. Based on available evidence, recommendations for the treatment of dyspnea in IPF are provided in Fig. 2. Disease-Modifying Treatments No potential disease-modifying treatment (i.e., treatment aimed at modifying the underlying pathogenesis of IPF) has been shown to reliably Fig. 1. Study selection (*studies could be excluded for multiple reasons during full-text review).

4 Study n Intervention (Study Design) Potential disease-modifying therapies Azuma et al., 107 Pirfenidone 600 mg PO TID for nine months (RCT) Demedts et al., Acetylcysteine 600 mg PO TID for one year, added to prednisone and azathioprine (RCT) Fiorucci et al., 30 Prednisone 1 mg/kg/d, prednisone 0.5 mg/kg/ d þ cyclophosphamide 100 mg/d, prednisone 0.5 mg/kg/d þ colchicine 1 mg/d for 78 weeks (open study, no control) King et al., 154 Bosentan 125 mg PO BID for minimum 52 weeks (RCT) King et al., Interferon gamma 200 mcg SC 3x/wk for median 64 weeks (RCT) Raghu et al., 330 Interferon gamma 200 mcg SC 3x/wk for minimum 48 weeks (RCT) Raghu et al., 87 Etanercept 25 mg SC 2x/wk for 48 weeks (RCT) Selman et al., 56 Colchicine 1 mg/d þ 1998 a,19 prednisone 1 mg/kg/d, D-penicillamine 600 mg/ d þ prednisone 1 mg/kg/ d, D-penicillamine þ colchicine þ prednisone, prednisone for 104 weeks (open study, no control) Non-disease-modifying therapies Collard et al., 11 Sildenafil for 12 weeks (open study, no control) Jackson et al., 29 Sildenafil 20 mg PO TID for weeks (RCT) Table 1 Summary of Trials Evaluating the Response of Dyspnea to Treatment in IPF % Surgical Biopsy Mean Age % Male % Ever Smoked Mean FVC (% Predicted) Mean DLCO (% Predicted) Dyspnea No effect on dyspnea (Chronic Respiratory Questionnaire, dyspnea subscale) No effect on CRP dyspnea score NR NR NR NR Improved CRP dyspnea score 2.1 points in prednisone þ colchicine arm (P ¼ 0.001) Results Primary Endpoint No effect on change from baseline to lowest SpO 2 during a six-minute steady-state exercise test Improved VC 0.18 L (P ¼ 0.02), DLCO 0.75 mmol/min/kpa (P ¼ 0.003) No effect on total CRP score NR No effect on BDI/TDI or Borg dyspnea index after six-mwt No effect on six-mwd No effect on UCSD SOBQ No effect on overall survival time NR No effect on BDI/TDI No effect on progressionfree survival NR No effect on BDI/TDI No effect on FVC, DLCO, P(A-a)O NR 38 NR No effect on four-point Not specified dyspnea scale No effect on modified Borg dyspnea index during six-mwt > No effect on Borg dyspnea score before or after six-mwt six-mwd improved 49 m (P < 0.05) No effect on six-mwd 774 Ryerson et al. Vol. 43 No. 4 April 2012

5 Vol. 43 No. 4 April 2012 Dyspnea in Idiopathic Pulmonary Fibrosis 775 No effect on six-mwd NR Improved UCSD SOBQ 6.58 points (P ¼ 0.006); no effect on Borg dyspnea score IPF Network, 180 Sildenafil 20 mg PO TID for weeks (RCT), followed by open-label extension of additional 12 weeks Not specified NR Improved Borg dyspnea score during bicycle exercise test (P < 0.05) Moderno 10 Proportional assisted et al., ventilation during exercise, CPAP, no ventilatory assistance (crossover study) NR NR No effect on BDI/TDI Not specified Nishiyama 28 Pulmonary rehabilitation 2x/ et al., wk for 10 weeks (randomized trial, unclear control) Not specified NR Improved MRC 0.9 points (P ¼ 0.003) rehabilitation for 12 weeks (open study, no control) Ozalevli et al., 17 Home-based pulmonary IPF ¼ idiopathic pulmonary fibrosis; FVC ¼ forced vital capacity; DLCO ¼ diffusing capacity of carbon monoxide; PO ¼ orally; TID ¼ three times daily; RCT ¼ randomized controlled trial; SpO2 ¼ oxygen saturation by pulse oximetry; CRP ¼ clinical radiologic and physiologic scoring system; NR ¼ not reported; BID ¼ twice daily; VC ¼ vital capacity; BDI/TDI ¼ baseline/transitional dyspnea index; MWD ¼ minute walk distance; MWT ¼ minute walk test; UCSD SOBQ ¼ University of California San Diego Shortness of Breath Questionnaire; P(A-a)O2 ¼ alveolararterial oxygen gradient; CPAP ¼ continuous positive airway pressure; MRC ¼ Medical Research Council dyspnea scale. a Did not use ATS/ERS diagnostic criteria. improve dyspnea. 6,13e19 The combination of prednisone and colchicine was studied in two unblinded trials. 14,19 One trial randomized 30 subjects to receive 78 weeks of prednisone alone, prednisone plus cyclophosphamide, or prednisone plus colchicine. 14 Dyspnea score improved in the 10 subjects who received prednisone and colchicine but worsened in the other treatment arms (P ¼ 0.001). A second unblinded study found no improvement of dyspnea in 19 subjects after treatment with prednisone and colchicine for 104 weeks. 19 No other endpoints improved in either study. No other potential diseasemodifying treatment improved dyspnea. Non-Disease-Modifying Treatments Treatment of Pulmonary Hypertension. Three included studies directly or indirectly evaluated the impact of treating pulmonary hypertension in patients with IPF. A small randomized controlled trial found no effect of sildenafil on dyspnea in 29 subjects with a pulmonary artery systolic pressure of 25e50 mm Hg by echocardiography. 21 A second small open-label cohort study found sildenafil had no effect on dyspnea but did improve walk distance in 11 subjects with pulmonary hypertension confirmed by right heart catheterization. 20 A third study randomized 180 subjects with advanced IPF (defined by a diffusing capacity of carbon monoxide [DLCO] < 35% predicted) to 12 weeks of placebo or sildenafil (a diagnosis of pulmonary hypertension was not required for inclusion). 22 Sildenafil therapy improved resting dyspnea, some measures of QOL, DLCO, and oxygenation, with no increase in serious adverse reactions. There were no studies identified that evaluated treatment of any other comorbid disease. Pulmonary Rehabilitation. Two studies of pulmonary rehabilitation satisfied inclusion criteria. One randomized controlled study of 28 subjects found no benefit of pulmonary rehabilitation on dyspnea. 24 A second uncontrolled cohort study of 17 subjects evaluated change in dyspnea after a 12-week home-based pulmonary rehabilitation program. 25 Dyspnea improved (P ¼ 0.003) in the 15 subjects who completed the program, as did walk distance and QOL. Nine additional studies of pulmonary rehabilitation were identified in our search but were excluded for various reasons (definition of

6 776 Ryerson et al. Vol. 43 No. 4 April 2012 Fig. 2. Treatment considerations for dyspneic patients with IPF. pulmonary fibrosis not provided, n ¼ 3; 42e44 IPF not reported separately, n ¼ 3; 45e47 and not published in English, n ¼ 3 48e50 ). Dyspnea improved in eight of these nine studies, but most of these studies had significant methodological deficiencies. One randomized study did not report subjects with IPF separately, 45 but a subsequent Cochrane review retrieved the original data and reported findings for the IPF subgroup. 51 This analysis was underpowered, with a small but nonsignificant improvement in dyspnea in subjects with IPF (standardized mean difference 0.56, 95% confidence interval 1.26 to 0.14). Noninvasive Ventilatory Assistance. One study of 10 subjects was included that evaluated noninvasive ventilatory assistance during exercise, an intervention similar to bilevel positive airway pressure. 23 Exertional dyspnea was improved compared with no ventilatory assistance and treatment with continuous positive airway pressure (CPAP). Exercise tolerance and some markers of cardiovascular and

7 Vol. 43 No. 4 April 2012 Dyspnea in Idiopathic Pulmonary Fibrosis 777 ventilatory function also improved with assisted ventilation. The effects of this intervention on resting dyspnea and long-term dyspnea were not evaluated. Supplemental Oxygen. We did not identify any studies of supplemental oxygen that met inclusion criteria. One study reported the impact of oxygen on dyspnea in patients with IPF, but this study was excluded because of an inadequate definition of IPF and its small size. 52 Compared with control, oxygen improved dyspnea in 10 subjects with ILD (eight IPF, one hypersensitivity pneumonitis, one amiodarone-induced ILD) (P < 0.05). Opioids. Two studies reported the impact of opioids on dyspnea in patients with IPF, but both studies were excluded. A study of six subjects with ILD (three with IPF) showed no impact of nebulized morphine on dyspnea compared with placebo. 53 A second study of 11 subjects with IPF and severe breathlessness showed that dyspnea decreased significantly with subcutaneous diamorphone (P < ), without any respiratory depression or other significant adverse effects. 54 This study was excluded because of an inadequate definition of IPF, with only six subjects having diagnosis confirmed by computed tomography and only eight having restrictive physiology. Correlates of Dyspnea Fifteen studies evaluated correlates of dyspnea (Table 2). Seventy unique variables were tested (Appendix III at jpsmjournal.com). Most variables were evaluated in only one or two studies. Dyspnea showed statistically significant correlation with numerous variables using bivariate analysis, including functional and physiological measures (e.g., six-minute walk distance [six-mwd]) and comorbid disease (i.e., depression) (Fig. 3). Multivariate analysis was performed in six studies, showing independent association of dyspnea with several variables (Table 3). Discussion Dyspnea in IPF can potentially be treated by effective disease modification, lung transplantation, and symptom-based therapy. Diseasemodifying treatments have shown a limited impact on dyspnea. Although dyspnea improved in a small unblinded study of prednisone and colchicine, 14 there was no Table 2 Summary of Trials Evaluating Correlations of Dyspnea in IPF n Dyspnea Tools % Surgical Biopsy Mean Age a % Male % Ever Smoked Mean FVC (% Predicted) Mean DLCO (% Predicted) Studies that used the ATS/ERS diagnostic criteria for IPF Daniil et al., MRC NR Daniil et al., MRC De Vries et al., Bath breathlessness NR NR NR 45 scale Glaser et al., Borg NR NR NR Manali et al., MRC NR Mura et al., MRC Mura et al., MRC Nishiyama et al., 2005, 41 BDI/TDI, Borg ,34,b Papiris et al., MRC 85 NR Papiris et al., MRC Tzanakis et al., Borg, MRC, OCD NR Zimmermann et al., BDI/TDI NR Total/weighted mean Studies that did not use the ATS/ERS diagnostic criteria for IPF Martinez et al., BDI/TDI NR 62 NR Meliconi et al., CRP NR Total/weighted mean d IPF ¼ idiopathic pulmonary fibrosis; FVC ¼ forced vital capacity; DLCO ¼ diffusing capacity of carbon monoxide; ATS ¼ American Thoracic Society; ERS ¼ European Respiratory Society; MRC ¼ Medical Research Council dyspnea scale; NR ¼ not reported; BDI/TDI ¼ baseline/transitional dyspnea index; OCD ¼ oxygen cost diagram; CRP ¼ clinical radiologic and physiologic scoring system. a P < 0.05 for difference between studies that used and did not use the ATS/ERS diagnostic criteria for IPF. b Nishiyama et al. reported findings from the same population in two studies. These studies are combined for this table.

8 778 Ryerson et al. Vol. 43 No. 4 April 2012 Fig. 3. Schema of statistically significant correlates of dyspnea severity in IPF. improvement in a larger study of similar design. 19 There are several possible explanations for the absence of benefit with diseasemodifying therapies. First, the treatments studied may be ineffective treatments for IPF, as suggested by the absence of convincing Study Table 3 Correlates of Dyspnea in IPF Using Multivariate Analysis Variables Included in Final Model n (P-value for Variable) R 2 of Model (P-value for Model) Manali et al., Six-MWD (P < 0.05) NR Mura et al., DLCO % predicted (P < 0.05) 0.28 (P ¼ 0.008) HRCT score (P < 0.05) Mura et al., Extent of fibrosis/fibrosis score (P < 0.05) 0.17 (P ¼ 0.063) Extent of emphysema (P < 0.05) Nishiyama et al., SpO 2 at end of six-mwt (P ¼ ) 0.27 (P ¼ ) Papiris et al., PaCO 2 (P ¼ 0.02) NR FVC% predicted (P ¼ 0.004) Papiris et al., CD8 þ T lymphocyte count (BAL) (P ¼ 0.042) NR IPF ¼ idiopathic pulmonary fibrosis; MWD ¼ minute walk distance; NR ¼ no response; DLCO ¼ diffusing capacity of carbon monoxide; HRCT ¼ high-resolution computed tomography; SpO2 ¼ oxygen saturation by pulse oximetry; MWT ¼ minute walk test; PaCO2 ¼ arterial partial pressure of carbon dioxide; FVC ¼ forced vital capacity; BAL ¼ bronchoalveolar lavage.

9 Vol. 43 No. 4 April 2012 Dyspnea in Idiopathic Pulmonary Fibrosis 779 benefit in the primary endpoints in most studies. Second, dyspnea was not a primary endpoint in most of the reviewed studies, and some studies were underpowered to evaluate change in dyspnea. Finally, most dyspnea metrics used in these studies were developed in non-ild populations and may not be as accurate or sensitive to change in patients with ILD. We did not identify any studies that evaluated the impact of lung transplant on dyspnea. Lung transplantation is likely an appropriate treatment for dyspnea in patients with endstage IPF, but it is a costly and limited resource that is not suitable for many patients. Given the absence of effective disease-modifying therapies and the limited availability and appropriateness of lung transplantation, symptom-based therapy for dyspnea remains an important potential alternative. Treatment of Comorbid Disease Pulmonary hypertension is present in up to 46% of patients with severe IPF 55 and is an important predictor of mortality. 56,57 Although the largest study of sildenafil in patients with severe IPF did not require subjects to have pulmonary hypertension, the hypothesized mechanism of action was via the effects of sildenafil on the pulmonary vasculature, rather than the underlying fibrotic process. 22 Although sildenafil did not improve the primary endpoint of six-mwd, there was statistically significant and clinically meaningful improvement in resting dyspnea. The results of this large, randomized, placebo-controlled trial (180 subjects) must be balanced against the negative results from two much smaller studies of sildenafil (total of 40 subjects). Overall, the balance of evidence suggests that sildenafil may have a role in the treatment of dyspnea in selected patients with severe IPF. We did not identify any studies that evaluated the impact of treating other comorbidities, such as emphysema or cardiovascular disease, in patients with IPF. Some patients with IPF have more emphysema than fibrosis, suggesting that symptoms may improve by treating obstructive lung disease. Similarly, cardiovascular disease is common in IPF, 58 but the effect of treating cardiovascular disease in IPF has not been examined. The impact of treating comorbidities, such as emphysema and cardiovascular disease in IPF warrants additional study. Other Symptomatic Therapies Studies of pulmonary rehabilitation in general populations of ILD patients (which contained large numbers of IPF patients) have generally shown that pulmonary rehabilitation improves dyspnea and other important endpoints including walk distance. 42e47 The balance of these studies in patients with ILD suggests that pulmonary rehabilitation may be beneficial in managing dyspnea in IPF despite the discordant IPF specific studies. No studies of supplemental oxygen and opioid therapy in IPF satisfied inclusion criteria for this systematic review. Limited evidence from excluded studies and extrapolation from another chronic respiratory disease (i.e., chronic obstructive pulmonary disease) suggests that these therapies may be useful. Clearly, additional research is needed. Although proportional assisted ventilation during exercise did improve exercise capacity and exertional dyspnea in one small study, this treatment is cumbersome and impractical for most patients. Additional therapies directed at other mechanisms of dyspnea also warrant further investigation. Correlates of Dyspnea The pathogenesis of dyspnea in IPF likely results from complex interactions of pulmonary, cardiovascular, neuromechanical, and psychological inputs. Identification of a variety of correlates of dyspnea supports this multidimensional model of dyspnea in IPF. Future studies should investigate the treatment of correlates of dyspnea, as some of these correlations also may represent causation. Deconditioning and depression correlate with dyspnea in IPF and are promising candidates for this approach. The impact of pulmonary rehabilitation in ILD may well be a result of extrapulmonary effects, such as cardiovascular and peripheral muscle function, rather than improvements in ventilatory restriction and gas exchange. 24,44,45 Similarly, dyspnea is a common somatic symptom of depression, suggesting that treatment of depression, when present, may have a secondary effect of improving dyspnea. This systematic review highlights the limitations of the current literature regarding correlates and potential contributors to dyspnea in IPF. Only six studies used multivariate analysis,

10 780 Ryerson et al. Vol. 43 No. 4 April 2012 and these analyses were performed on small cohorts, limiting the ability to adjust for potential confounders. An important area of future research is to examine the correlates of dyspnea using a multivariate approach that incorporates a wider variety of potentially modifiable factors. Limitations There are limitations to this review. First, we were unable to assess for publication bias. However, the objective of this review was to identify effective treatments for dyspnea in patients with IPF, and studies showing such effects are more likely to be published. Second, we were unable to provide summary estimates because of the temporal and methodological heterogeneity of the included studies. Several dyspnea tools were used, and these metrics have unique characteristics that may contribute to the inconsistent results observed for some treatments. Third, we only reviewed articles published in English. Notwithstanding these limitations, this review is the first to systematically examine the treatment and correlates of dyspnea in IPF, providing an important summary of the existing literature and gaps in knowledge. Conclusion This systematic review summarizes what is currently known about the treatment of dyspnea in IPF, provides recommendations for the treatment of dyspnea in IPF, and suggests areas for further research in this field. Sildenafil and pulmonary rehabilitation are potential therapies for dyspnea in selected patients with IPF. Supplemental oxygen and opioids may be additional potential therapies; however, the evidence supporting their use is weak. Additional research should focus on the management of functional status and comorbidities as potential treatments for dyspnea. Disclosures and Acknowledgments No funding was received for this study. C. J. R., S. Z. P., and H. R. C. conceived the research question and study design. C. J. R. developed the search strategy. C. J. R. and D. D. performed the search and data extraction. The initial draft of the manuscript was prepared by C. J. R. and H. R. C. All authors read and approved the final manuscript, and all authors take responsibility for the integrity of the work as a whole. Appendix Supplementary Material Supplementary data associated with this article can be found, in the online version, at /j.jpainsymman References 1. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810e Bjoraker JA, Ryu JH, Edwin MK, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998;157:199e Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a population-based cohort study. Chest 1998;113:396e Swigris JJ, Kuschner WG, Jacobs SS, Wilson SR, Gould MK. Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review. Thorax 2005;60:588e King TE Jr, Schwarz MI, Brown K, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med 2001;164:1025e Demedts M, Behr J, Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005;353:2229e Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010;35:821e Ryerson CJ, Collard HR, Pantilat SZ. Management of dyspnea in interstitial lung disease. Curr Opin Support Palliat Care 2010;4:69e American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000;161(2 Pt 1):646e American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of

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