Survival after inpatient or outpatient pulmonary rehabilitation in patients with fibrotic interstitial lung disease: a multicentre retrospective cohort study.

BACKGROUND: The impact of pulmonary rehabilitation (PR) on survival in patients with fibrotic interstitial lung disease (ILD) is unknown. Given the challenges conducting a large randomised controlled trial, we aimed to determine whether improvement in 6-minute walk distance (6MWD) was associated with better survival. METHODS: This retrospective, international cohort study included patients with fibrotic ILD participating in either inpatient or outpatient PR at 12 sites in 5 countries. Multivariable models were used to estimate the association between change in 6MWD and time to death or lung transplantation accounting for clustering by centre and other confounders. RESULTS: 701 participants (445 men and 256 women) with fibrotic ILD were included. The mean±SD ages of the 196 inpatients and 505 outpatients were 70±11 and 69±12 years, respectively. Baseline/changes in 6MWD were 262±128/55±83 m for inpatients and 358±125/34±65 m for outpatients. Improvement in 6MWD during PR was associated with lower hazard rates for death or lung transplant on adjusted analysis for both inpatient (HR per 10 m 0.94, 95% CI 0.91 to 0.97, p<0.001) and outpatient PR (HR 0.97, 95% CI 0.95 to 1.00, p=0.042). Participation in ≥80% of planned outpatient PR sessions was associated with a 33% lower risk of death (95% CI 0.49% to 0.92%). CONCLUSIONS: Patients with fibrotic ILD who improved physical performance during PR had better survival compared with those who did not improve performance. Confirmation of these hypothesis-generating findings in a randomised controlled trial would be required to definitely change clinical practice, and would further support efforts to improve availability of PR for patients with fibrotic ILD.

The Impact of Multidisciplinary Discussion (MDD) in the Diagnosis and Management of Fibrotic Interstitial Lung Diseases.

Accurate diagnosis of interstitial lung disease (ILD) is crucial for management and prognosis but can be challenging even for experienced clinicians. Expert multidisciplinary discussion (MDD) is considered the reference standard for ILD diagnosis; however, there remain concerns regarding lack of validation studies and relative limited information on the impact of MDD in real-life clinical practice. The goal of this study was to assess the effect of MDD in providing a specific ILD diagnosis, changing the diagnosis provided upon referral, and to determine how often and in which way MDD altered management. Material and Methods. Retrospective observational study in an ILD referral tertiary academic center. MDD diagnoses were categorized as specific, provisional, and unclassifiable ILD. Pre-MDD and MDD diagnoses were compared for change in diagnosis and concordance rates for specific diagnoses. Relevant change in management including initiation or change in pharmacological treatment, referral to surgical biopsy, and nonpharmacological management were recorded. Results. 126 cases were included (79M, 47F, 36-93 years, mean 70 y). Specific MDD diagnosis was provided in 62% (78/126); 12% (15/126) had provisional diagnosis, and 21% (27/126) was unclassifiable. Overall agreement for specific pre-MDD and MDD diagnosis was 41% (52/126) and 80% for idiopathic pulmonary fibrosis (IPF) diagnosis. MDD altered diagnosis in 37% (47/126) and changed management in 39% (50/126). Amongst concordant diagnoses, management was altered in 46% (24/52). In summary, MDD provided a specific diagnosis discordant with pre-MDD diagnosis in a significant proportion of cases and was particularly valuable in the diagnosis of non-IPF ILD. MDD often altered management and had relevant impact on management even in cases with concordant pre-MDD diagnosis.

Systematic review of content and quality of idiopathic pulmonary fibrosis review articles.

Narrative reviews are frequently accessed; however, the content and quality of review articles on idiopathic pulmonary fibrosis (IPF) have not been assessed. A systematic review assessed content and quality of narrative review articles that addressed the diagnosis or management of IPF and were published from 2001 to 2015. Article recommendations were assessed relative to contemporary IPF guidelines. Quality was assessed using the DISCERN instrument. Articles were predominantly written by physicians and published in respiratory journals. Conflicts of interest and sources of funding were reported in 52% and 24% of reviews, respectively. European authors were more likely to recommend bronchoscopy (adjusted p=0.02) and were more likely to recommend pirfenidone or nintedanib prior to publication of definitive clinical trials (adjusted p=0.04). A total of 39% of management-focused articles suggested therapies that were never recommended in guidelines. Predictors of higher article quality were citation of the contemporary IPF guideline (p=0.01) and more recent publication (p=0.001). Quality of reviews increased over time; however, review articles frequently made discordant recommendations compared to IPF guidelines. These findings indicate the need for authors, peer reviewers, editors and readers to critically appraise the content and quality of narrative reviews on IPF, and the need for frequent guideline updates to reflect new evidence.

A diagnostic model for chronic hypersensitivity pneumonitis.

The objective of this study was to develop a diagnostic model that allows for a highly specific diagnosis of chronic hypersensitivity pneumonitis using clinical and radiological variables alone. Chronic hypersensitivity pneumonitis and other interstitial lung disease cases were retrospectively identified from a longitudinal database. High-resolution CT scans were blindly scored for radiographic features (eg, ground-glass opacity, mosaic perfusion) as well as the radiologist's diagnostic impression. Candidate models were developed then evaluated using clinical and radiographic variables and assessed by the cross-validated C-statistic. Forty-four chronic hypersensitivity pneumonitis and eighty other interstitial lung disease cases were identified. Two models were selected based on their statistical performance, clinical applicability and face validity. Key model variables included age, down feather and/or bird exposure, radiographic presence of ground-glass opacity and mosaic perfusion and moderate or high confidence in the radiographic impression of chronic hypersensitivity pneumonitis. Models were internally validated with good performance, and cut-off values were established that resulted in high specificity for a diagnosis of chronic hypersensitivity pneumonitis.

Under-recognised co-morbidities in idiopathic pulmonary fibrosis: A review.

Co-morbidities in idiopathic pulmonary fibrosis are common. These co-morbidities include obstructive sleep apnoea, gastro-oesophageal reflux disease, pulmonary hypertension and depression. The presence of co-morbidities among patients with idiopathic pulmonary fibrosis contributes to worse quality of life, morbidity and mortality. Despite the high prevalence of certain co-morbidities in idiopathic pulmonary fibrosis, the optimal screening and management of many of these conditions remains unclear. The impact of co-morbidities on this patient population is becoming more apparent. Their relevance will only increase as significant effort is being made to develop novel therapeutics that will alter the disease trajectory of patients with idiopathic pulmonary fibrosis. The purpose of this review is to focus on the epidemiology, pathophysiology, diagnosis and management of select co-morbidities, including obstructive sleep apnoea, gastro-oesophageal reflux disease, pulmonary hypertension and depression, in idiopathic pulmonary fibrosis.

Exacerbation frequency and clinical outcomes in adult patients with cystic fibrosis.

BACKGROUND: Despite advances in treatment of cystic fibrosis (CF), pulmonary exacerbations remain common. The aim of this study was to determine if frequent pulmonary exacerbations are associated with greater declines in lung function, or an accelerated time to death or lung transplantation in adults with CF. METHODS: A 3-year prospective cohort study was conducted on 446 adult patients with CF from Ontario, Canada who could spontaneously produce sputum. Patients enrolled from 2005 to 2008 and were stratified into groups based upon their exacerbation rates over the 3 year study: <1 exacerbation/year (n=140), 1-2 exacerbations/year (n=160) and >2 exacerbations/year (n=146). Exacerbations were defined as acute/subacute worsening of respiratory symptoms severe enough to warrant oral or intravenous antibiotics. Patient-related factors associated with frequent exacerbations were determined, and clinical outcomes were compared among the three exacerbation groups. RESULTS: Patients with frequent exacerbations were more likely to be female, diabetic and have poorer baseline lung function. Patients with >2 exacerbations/year had an increased risk of experiencing a 5% decline from baseline forced expiratory volume in 1 s (FEV(1)); unadjusted HR 1.47 (95% CI 1.07 to 2.01, p=0.02), adjusted HR 1.55 (95% CI 1.10 to 2.18, p=0.01) compared with patients with <1 exacerbation/year. Patients with >2 exacerbations/year also had an increased risk of lung transplant or death over the 3 year study; unadjusted HR 12.74 (95% CI 3.92 to 41.36, p<0.0001), adjusted HR 4.05 (95% CI 1.15 to 14.28, p=0.03). CONCLUSIONS: Patients with CF with frequent exacerbations appear to experience an accelerated decline in lung function, and they have an increased 3 year risk of death or lung transplant.