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BAL cellular analysis is often recommended during the initial diagnostic evaluation of fibrotic interstitial lung disease (ILD). Despite recommendation for its use, between-center heterogeneity exists and supportive data concerning the clinical utility and correlation of BAL findings with radiologic features or patterns remain sparse.

Previous studies have shown the importance of frailty in patients with fibrotic interstitial lung disease (ILD).

RationaleThe European Quality of Life 5-Dimensions 5-Levels questionnaire (EQ-5D-5L) is a multidimensional patient-reported questionnaire that supports calculation of quality-adjusted life-years. Our objectives were to demonstrate feasibility of use and to calculate the minimum important difference (MID) of the EQ-5D-5L and its associated visual analogue scale (EQ-VAS) in patients with fibrotic interstitial lung disease (ILD).MethodsPatients who completed the EQ-5D-5L were identified from the prospective multicentre CAnadian REgistry for Pulmonary Fibrosis. Validity, internal consistency and responsiveness of the EQ-5D-5L were assessed, followed by calculation of the MID for the EQ-5D-5L and EQ-VAS. Anchor-based methods used an unadjusted linear regression against pulmonary function tests (PFTs) and dyspnoea and other quality of life questionnaires. Distribution-based method used one-half SD and SE measurement (SEM) calculations.Results1816 patients were analysed, including 472 (26%) with idiopathic pulmonary fibrosis. EQ-5D-5L scores were strongly correlated with the dyspnoea and other quality of life questionnaires and weakly associated with PFTs. The estimated MID for EQ-5D-5L ranged from 0.0050 to 0.054 and from 0.078 to 0.095 for the anchor-based and distribution-based methods, respectively. The MID for EQ-VAS ranged from 0.5 to 5.0 and from 8.0 to 9.7 for the anchor-based and distribution-based methods. Findings were similar across ILD subtypes, sex and age.ConclusionWe used a large and diverse cohort of patients with a variety of fibrotic ILD subtypes to suggest validity and MID of both the EQ-5D-5L and EQ-VAS. These findings will assist in designing future clinical trials and supporting cost-effectiveness analyses of potential treatments for patients with fibrotic ILD.

AbstractRATIONALE: INSPIRATION PLUS (NCT02552849) was a prospective, observational registry of Canadian patients with idiopathic pulmonary fibrosis (IPF) treated with the oral antifibrotic agent pirfenidone. As an exploratory analysis of the registry, pirfenidone usage and safety were examined in patients with incident pirfenidone use.OBJECTIVE: To describe pirfenidone usage and tolerability in Canadian patients with IPF.METHODS: Patients with IPF who received pirfenidone were monitored prospectively beginning in April 2013. Planned outcomes included change in percent predicted forced vital capacity and the proportion of patients receiving a full pirfenidone dose (2403 mg/day). Data were collected during routine clinical visits. Pirfenidone usage and safety were analyzed in patients who initiated pirfenidone within 3 months of enrollment.RESULTS: A total of 297 patients enrolled; 125 patients with incident pirfenidone use; 98 patients with both baseline data and ≥ 1 follow-up visit comprised the analysis population. Median (range) duration of follow-up and number of follow-up visits were 10 (0.9–34.4) months and 3 (1–9) visits, respectively. Dose modifications occurred more frequently during the first 6 months of treatment than in the subsequent 6 months (23% vs 8% of patients). The incidence of adverse drug reactions was consistent with the known safety profile for pirfenidone; gastrointestinal-related events were most frequent. Increased age or a history of smoking was associated with an increased risk of discontinuing pirfenidone in the first 12 months of treatment.CONCLUSIONS: Canadian patients with IPF receiving pirfenidone had a similar safety profile compared with clinical trial data.

Fibrotic interstitial lung diseases (ILDs) are highly morbid chronic disorders that frequently occur in working age individuals. The goal of this study was to determine workplace productivity loss, its determinants, and its estimated costs in patients with fibrotic ILD.

AbstractRATIONALE: There is considerable variability in patient outcomes following bronchial thermoplasty (BT), an adjunct therapy for patients with severe asthma. It is unclear if the presence of airway inflammation is important to consider prior to BT.OBJECTIVE: The objective of the article was to ascertain if optimizing airway inflammation guided by sputum cell counts prior to BT, as compared to guideline-based-care, results in better patient outcomes and improved airway hyperresponsiveness.METHODS: We retrospectively evaluated asthmatics who underwent BT at our institution (Firestone Institute for Respiratory Health, St. Joseph’s Healthcare Hamilton, Canada) and compared clinical outcomes (asthma-quality-of-life-questionnaire [AQLQ], asthma-control-questionnaire [ACQ] and provocative concentration of methacholine required to decrease the forced expiratory volume in one second by 20% [PC20]) in those patients managed using guideline-based-care (n = 7) versus a protocol that involved optimizing airway inflammation using quantitative sputum cell counts (n = 7).MEASUREMENTS AND MAIN RESULTS: Following BT, there were greater improvements in the inflammation-optimized group than in the guideline-based-care group for the asthma-quality-of-life-questionnaire (ΔAQLQ: 2.2 ± 1.2 vs 0.8 ± 0.9, p = 0.04), asthma-control-questionnaire (ΔACQ: −2.3 ± 1.3 vs −0.5 ± 1.1, p = 0.02) and airway hyperresponsiveness (ΔPC20: 3.9 ± 1.2 vs. 0.6 ± 0.8 doubling-doses, p = 0.008).CONCLUSION: This is the first study to provide preliminary evidence that BT, when performed after normalizing sputum cell counts, significantly improves patient reported outcomes and airway hyperresponsiveness, compared to current guidelines that presume airway inflammation is controlled.

AbstractStridor is an uncommon presentation for Parkinson’s disease dystonia. We report a case of a 68-year-old female with poorly controlled Parkinson’s disease who presented to the hospital with recurrent episodes of respiratory distress and stridor. Aggressive management of her Parkinson’s disease, including the insertion of a deep brain stimulator, led to resolution of these episodes.

Clinical practice guidelines separately describe radiologic patterns of usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), without direction on whether or how to apply these approaches concurrently within a single patient.

Mortality risk assessment in interstitial lung disease (ILD) is challenging. Our objective was to determine the prognostic significance of BMI and change in weight in the most common fibrotic ILD subtypes.

Background. St. Joseph’s Health System has implemented an integrated comprehensive care bundle care (ICC) program with the hopes that it would improve patients’ care while reducing overall costs. The aim of this analysis was to evaluate the performance of the ICC program within patients admitted with chronic pulmonary obstructive disease (COPD). Methods. We conducted a retrospective observational cohort study comparing ICC patients to non-ICC patients admitted to St. Joseph’s Healthcare Hamilton for COPD being discharged with support services between June 2012 and March 2015, using administrative data. Confounding adjustment was achieved through the use of propensity score matching. Medical resource utilizations during the initial hospitalization and within the 60 days following discharge were compared using regression models. Results. All 76 patients who entered the ICC program (100.0%) were matched 1 : 1 to 76 eligible non-ICC patients (28.4%). Length of stay (6.47 [7.29] versus 9.55 [10.21] days) and resource intensity weights (1.16 [0.80] versus 1.64 [1.69]) were lower in the ICC group within the initial hospitalization but, while favoring the ICC program, healthcare resource use tended not to differ statistically following discharge. Interpretation. The ICC program was able to reduce initial medical resource utilization without increasing subsequent medical resource use.