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Blair G Fulton,1 Christopher J Ryerson1,2 1Department of Medicine, 2Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada Abstract: Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. Keywords: interstitial lung disease, management, idiopathic pulmonary fibrosis, comorbidities

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. Design: A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Setting: Interstitial lung disease referral centers in California, Minnesota, and Italy. Patients: 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). Measurements: The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Results: Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and Dlco). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index >=71.9). Limitation: Patients were drawn from academic centers and analyzed retrospectively. Conclusion: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF. Primary Funding Source: University of California, San Francisco Clinical and Translational Science Institute. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]