Publications by authors named "Nathan Hambly"

Background: 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.

Research Question: In patients with fibrotic ILD, are BAL findings associated with radiologic features, patterns, and clinical diagnoses?

Study Design And Methods: Patients with fibrotic ILD who underwent BAL for diagnostic evaluation and who were enrolled in the prospective Canadian Registry for Pulmonary Fibrosis were re-reviewed in a standardized multidisciplinary discussion (MDD).

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Background And Objective: Establishing an accurate and timely diagnosis of idiopathic pulmonary fibrosis (IPF) is essential for appropriate management and prognostication. In some cases, surgical lung biopsy (SLB) is performed but carries non-negligible risk. The objective of this retrospective study was to determine if SLB is associated with accelerated lung function decline in patients with IPF using the Canadian Registry for Pulmonary Fibrosis.

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

Research Question: Is the Clinical Frailty Scale (CFS) a valid tool to improve risk stratification in patients with fibrotic ILD?

Study Design And Methods: Patients with fibrotic ILD were included from the prospective multicenter Canadian Registry for Pulmonary Fibrosis. The CFS was assessed using available information from initial ILD clinic visits.

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Objectives: Interstitial lung disease (ILD) in CTDs has highly variable morphology. We aimed to identify imaging features and their impact on ILD progression, mortality, and immunosuppression response.

Methods: Patients with CTD-ILD had high-resolution chest CT (HRCT) reviewed by expert radiologists blinded to clinical data for overall imaging pattern [usual interstitial pneumonia (UIP); non-specific interstitial pneumonia (NSIP); organizing pneumonia (OP); fibrotic hypersensitivity pneumonitis (fHP); and other].

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Article Synopsis
  • Interstitial pneumonia with autoimmune features (IPAF) shares similarities with connective tissue disease-associated interstitial lung disease (CTD-ILD) but does not fully meet the criteria for a specific connective tissue disease.
  • A study compared the characteristics and survival of IPAF with CTD-ILD and unclassifiable ILD, revealing that IPAF patients are older and have a greater smoking history compared to CTD-ILD, while having worse lung function than unclassifiable ILD.
  • The findings suggest that while patients with IPAF experience worse survival rates compared to CTD-ILD, they have similar mortality to those with unclassifiable ILD, raising questions about whether IPAF should be viewed as a separate category of interstitial lung disease
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  • Hypoxemia in fibrotic interstitial lung disease (ILD) signals disease progression and can predict patient mortality.
  • A risk prediction tool was created to identify new-onset exertional and resting hypoxemia using data from ILD registries in Canada, Australia, and the U.S.
  • The model's effectiveness was assessed, showing good performance based on factors like age, body mass index, and specific lung function measures, although the validation cohort showed some issues with calibration for resting hypoxemia.
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  • Clinical practice guidelines currently distinguish between different radiologic patterns for usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP), but do not clarify how to use these approaches together for diagnosis in individual patients.
  • A study involving 1,593 patients aimed to integrate these radiologic patterns for diagnosing interstitial lung disease (ILD), revealing that typical UIP patterns strongly correlated with idiopathic pulmonary fibrosis (IPF) diagnoses, while the typical fHP pattern was linked to fHP diagnoses in a significant portion of cases.
  • The findings suggest that an integrated approach is feasible, highlighting that over 5% gas trapping on expiratory imaging is a key diagnostic feature that helps differentiate compatible fHP from other patterns, thus calling
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Background: Risk stratification is fundamental in the management of pulmonary arterial hypertension (PAH). Pulmonary artery pulsatility index (PAPi), defined as pulmonary arterial pulse pressure divided by right atrial pressure (RAP), is a hemodynamic index shown to predict acute right ventricular (RV) dysfunction in several settings. Our objective was to test the prognostic utility of PAPi in a diverse multicentre cohort of patients with PAH.

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Background: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is challenging to manage, with a paucity of robust data to guide treatment. Our aim was to characterize the pharmacologic treatment of RA-ILD utilizing a retrospective design in a national multi-center prospective cohort, and to identify associations between treatment and change in lung function and survival.

Methods: Patients with RA-ILD and a radiological pattern of non-specific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) were included.

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Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease of unknown etiology. The accumulation of macrophages is associated with disease pathogenesis. The unfolded protein response (UPR) has been linked to macrophage activation in pulmonary fibrosis.

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Background: Some studies suggest that patients with pulmonary hypertension (PH) may be at higher risk of complications and death after noncardiac surgery. However, the magnitude of these associations is unclear.

Objectives: To determine the associations between PH and adverse outcomes after noncardiac surgery.

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Background: Functional capacity, as measured by the 6-min walk test (6MWT), is often reduced in fibrotic interstitial lung disease (ILD). This study evaluated longitudinal changes and the prognostic significance of 6MWT parameters, and explored change in oxygenation status as a physiological criterion to define disease progression in patients with fibrotic ILD.

Research Questions: What are the trajectories and prognostic value of 6MWT parameters in patients with fibrotic ILD?

Study Design And Methods: Using prospective registries in Australia and Canada, patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD were stratified by the presence of criteria for progressive pulmonary fibrosis (PPF).

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Background And Objective: Rheumatoid arthritis (RA) is a frequent cause of interstitial lung disease (ILD); however, the impact of rheumatoid factor and anti-citrullinated peptide antibody seropositivity in ILD without connective tissue disease (CTD) is unclear. We examined the association of seropositivity with ILD progression, mortality and response to immunosuppression in non-CTD ILD.

Methods: A total of 1570 non-CTD patients (with idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, interstitial pneumonia with autoimmune features or unclassifiable ILD) and 181 RA-ILD patients were included from a prospective registry.

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Background And Objective: Inhalational exposures are a known cause of interstitial lung disease (ILD), but little is understood about their prevalence across ILD subtypes and their relationship with pulmonary function and survival.

Methods: Patients with fibrotic ILD were identified from the multicentre Canadian Registry for Pulmonary Fibrosis. Patients completed questionnaires regarding ILD-related occupational and environmental exposures.

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Background: Progressive fibrosing interstitial lung disease (PF-ILD) is characterised by progressive physiological, symptomatic and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain.

Methods: Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015 and 2020.

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Background: Infliximab is a monoclonal antibody that binds and neutralizes circulating tumor necrosis factor-alpha, a key inflammatory cytokine in the pathophysiology of sarcoidosis. Despite the paucity of randomized clinical trials, infliximab is often considered a therapeutic option for refractory disease. Our study aimed to investigate the effectiveness of infliximab in patients with refractory sarcoidosis.

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Article Synopsis
  • A study highlighted the need for data on how different antifibrotic therapies affect patients with idiopathic pulmonary fibrosis (IPF), particularly for those whose lung function is declining.
  • Researchers examined patients who experienced a significant decline in lung function while on antifibrotic treatment and found that continuing therapy was linked to less further decline and a lower risk of severe outcomes, compared to stopping the medication.
  • The results emphasized that ongoing antifibrotic therapy helps reduce future lung function decline and lowers the probability of death or needing a lung transplant, even in real-world settings.
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Survivors of severe SARS-CoV-2 infections frequently suffer from a range of post-infection sequelae. Whether survivors of mild or asymptomatic infections can expect any long-term health consequences is not yet known. Herein we investigated lasting changes to soluble inflammatory factors and cellular immune phenotype and function in individuals who had recovered from mild SARS-CoV-2 infections ( = 22), compared to those that had recovered from other mild respiratory infections ( = 11).

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  • The study investigates the significance of Body Mass Index (BMI) and weight changes as indicators of mortality risk in patients with fibrotic interstitial lung disease (ILD).
  • It involved a retrospective analysis of data from two large cohorts (from Canada and UCSF), categorizing patients by their BMI and tracking weight changes over time.
  • Findings revealed that underweight patients had significantly higher mortality rates compared to those with normal or higher BMI, and weight loss of 2 kg or more within a year raised the risk of death, reinforcing BMI and weight loss as valuable prognostic factors in ILD.
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  • Researchers studied how pulmonary rehabilitation (PR) affects survival in patients with a lung disease called fibrotic interstitial lung disease (ILD).
  • They looked at the 6-minute walk distance (6MWD) of patients before and after PR to see if walking farther made a difference in surviving longer.
  • Results showed that patients who improved their 6MWD during PR had a better chance of living longer, and those who attended most of their PR sessions were even less likely to die.
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  • The UCSDSOBQ is a specialized questionnaire that measures shortness of breath but lacks established benchmarks in fibrotic interstitial lung disease (ILD) populations.
  • The study evaluated the questionnaire's performance in 1,933 patients, examining its validity and reliability through various methods, including comparisons with other established questionnaires.
  • Findings showed the UCSDSOBQ has high internal consistency and is strongly correlated with other measures, establishing a minimum important difference (MID) of 5-8 points for this patient group.
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