Jeff Swigris

National Jewish Health · Autoimmune Lung Center and Interstitial Lung Disease Program

Rationale: In interstitial lung disease (ILD), symptoms drive impairments in health-related quality of life (HRQL). Patient-reported outcome measures (PROs) can assess whether interventions change symptom severity. The meaningfulness of change in a PRO's score is estimated by anchoring it to a related variable for which meaningful change has been...

Purpose of review Pulmonary fibrosis (PF) negatively influences health-related quality of life (HRQOL). Patients living with PF have voiced the desire for a focus on symptoms and HRQOL in both disease monitoring and treatment decisions. Recent findings Currently available disease modifying treatments do little to impact HRQOL. Newer studies evalua...

Rationale and Objective Disease-specific health-related quality of life (HRQOL) instruments enable us to capture domains that are most relevant to specific patient populations and are useful when a more individualized approach to patient assessment is desired. In this study, we assessed the validity and reliability of the first instrument specifica...

Background Dyspnea impairs quality of life (QOL) in patients with fibrotic hypersensitivity pneumonitis (FHP). The Living with Pulmonary Fibrosis questionnaire (L-PF) assesses symptoms, their impacts and PF-related QOL in patients with any form of PF. Its scores have not undergone validation analyses in an FHP cohort. Methods We used data from the...

Background: Idiopathic pulmonary fibrosis (IPF) carries significant mortality and unpredictable progression, with limited therapeutic options. Designing trials with patient-meaningful endpoints, enhancing the reliability and interpretability of results, and streamlining the regulatory approval process are of critical importance to advancing clinic...

Dyspnoea and cough can have a profound impact on the lives of patients with pulmonary fibrosis. We investigated the effects of nintedanib on the symptoms and impact of pulmonary fibrosis in patients with progressive pulmonary fibrosis (PPF) in the INBUILD trial using the Living with Pulmonary Fibrosis (L-PF) questionnaire. Patients had a fibrosing...

Background Interstitial lung disease due to rheumatoid arthritis (RA-ILD) affects a substantial minority of patients with RA, inducing life-altering symptoms, impairing quality of life (QOL) and forcing patients to confront the potential for shortened survival. Dyspnoea is the predominant respiratory symptom of RA-ILD and a strong driver of QOL imp...

Rationale: Radiologic pattern has been shown to predict survival in patients with fibrosing interstitial lung disease. The additional prognostic value of fibrosis extent by quantitative CT is unknown. Objectives: We hypothesized that fibrosis extent provides information beyond visually assessed CT pattern, that is useful for outcome prediction....

Many patients with fibrosing interstitial lung disease (fILD) will need to use supplemental oxygen (O 2 ) to maintain normoxia at some point in their illness. If it is not needed at the time of diagnosis, then if fILD progresses—or if a comorbid condition like pulmonary hypertension develops—O 2 will become necessary, often, initially, during exert...

Background Fibrotic hypersensitivity pneumonitis (FHP) is an irreversible lung disease with high morbidity and mortality. We sought to evaluate the safety and effect of pirfenidone on disease progression in such patients. Methods We conducted a single-centre, randomised, double-blinded, placebo-controlled trial in adults with FHP and disease progr...

Objective: Interstitial lung disease (ILD) is a leading cause of mortality in rheumatoid arthritis (RA), particularly in those with the usual interstitial pneumonia subtype (RA-UIP). Serum antibodies to peptidylarginine deiminase type 4 (anti-PAD4), particularly a subset that cross-react with PAD3 (PAD3/4XR), have been associated with imaging evid...

Rationale: Idiopathic pulmonary fibrosis is a rare, irreversible, and progressive disease of the lungs. Common genetic variants, in addition to non-genetic factors, have been consistently associated with IPF. Rare variants identified by candidate gene, family-based, and exome studies have also been reported to associate with IPF. However, the exte...

Background: Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has varied clinical and radiographic manifestations. Severe initial viral infection, cytokine release, opportunistic infection and post-viral inflammation may all contribute to progressive symptoms and severe lung injury. Acute f...

Background The Living with Pulmonary Fibrosis (L-PF) questionnaire assesses symptoms and quality of life in patients with fibrosing interstitial lung diseases (ILDs). Its Dyspnoea and Cough domains, whose items’ responses are based on a 24-hour recall, have scores ranging from 0 to 100, with higher scores indicating greater symptom severity. We eva...

Introduction Idiopathic pulmonary fibrosis (IPF) and non-IPF, progressive fibrotic interstitial lung diseases (PF-ILD), are associated with a progressive loss of lung function and a poor prognosis. Treatment with antifibrotic agents can slow, but not halt, disease progression, and treatment discontinuation because of adverse events is common. Fibro...

Rationale: Patients with fibrotic interstitial lung disease often progress to the point of requiring supplemental oxygen. This is invariably accompanied by an impaired quality of life and limitations on activities of daily living. Objectives: This study aimed to assess the improvement in physical activity in patients with interstitial lung disease...

Chronic lung diseases can cause great suffering and impair quality of life (QOL) in patients forced to live with them. In this chapter, we discuss the need to identify and alleviate symptoms (dyspnea, cough, fatigue, impaired sleep, anxiety and depression) and other factors that drive QOL impairment. Practitioners can partner with patients to imple...

Objectives This study sought to determine the level of understanding and opinion amongst rheumatologist and pulmonologists regarding risk factors, diagnostic approach and treatment of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Methods We conducted an international electronic survey of rheumatologists and pulmonologists uti...

Hypersensitivity pneumonitis (HP) is an immunologically mediated form of lung disease resulting from inhalational exposure to any of a large variety of antigens. A subgroup of patients with HP develops pulmonary fibrosis (fibrotic HP; FHP), a significant cause of morbidity and mortality. This study will evaluate the safety and efficacy of the antif...

The Living with Idiopathic Pulmonary Fibrosis (L-IPF) questionnaire was developed with substantial input from patients with IPF to assess symptoms and health-related quality of life (HRQoL). Because IPF is the prototypical chronic fibrosing interstitial lung disease (ILD) with a progressive phenotype, we expanded applicability of the L-IPF by delet...

Background: Evidence-based guidelines are needed for effective delivery of home oxygen therapy to appropriate patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD). Methods: The multidisciplinary panel created six research questions using a modified Delphi approach. A systematic review of the literature was...

This phase 2, randomised, double-blind, placebo-controlled trial evaluated the efficacy and safety of lebrikizumab, an interleukin-13 monoclonal antibody, alone or with background pirfenidone therapy, in patients with idiopathic pulmonary fibrosis (IPF). Patients with IPF aged ≥40 years with % predicted forced vital capacity (%FVC) 40%–100% and dif...

Rationale: Several new drugs for idiopathic pulmonary fibrosis (IPF) are in development. Tools are needed to assess whether these drugs benefit patients on outcomes that matter most to them. Health-related quality of life (HRQL) is one such outcome. It is influenced by many factors, but symptoms and their impacts are two strong drivers. Objective...

Background: Air pollution contributes to an estimated six million deaths per year. Epidemiological and experimental studies show an association between air pollutant exposure and respiratory allergy. Objective: We aimed to write a narrative review of the epidemiology of air pollution-related respiratory-related allergic disorders (including asth...

Background On high-resolution computed tomography (HRCT), pulmonary artery (PA) dimensions may hint at the presence of pulmonary hypertension. We aimed to determine how accurately various measures of the PA, as viewed on HRCT, predict right heart catheterisation (RHC)-confirmed pulmonary hypertension. Methods We retrospectively reviewed patients w...

Purpose: The interstitial lung diseases include a variety of disorders, many of which are characterized by fibrotic changes (fILD). Of the fILDs, Idiopathic Pulmonary Fibrosis (IPF) is the most common. Pulmonary hypertension (PH) frequently complicates fILD and is associated with impaired functional capability, lower physical activity and signific...

Background: In the Phase III INPULSIS® trials, treatment of patients with idiopathic pulmonary fibrosis (IPF) with nintedanib significantly reduced the annual rate of decline in forced vital capacity (FVC) versus placebo, consistent with slowing disease progression. However, nintedanib was not associated with a benefit in health-related quality of...

Objective To describe the phenotypic characteristics and natural history of patients with autoimmune forms of interstitial lung disease (ILD). Methods Retrospective, descriptive, single-center study of patients with autoimmune forms of ILD evaluated between February 2008 and August 2014. All data were extracted from the electronic medical record....

Objectives: The etiology of idiopathic pulmonary fibrosis (IPF) is unknown. Because it shares genetic, histopathologic and radiographic features with the fibrosing interstitial lung disease seen in rheumatoid arthritis (RA), we sought to investigate RA-related autoantibodies in IPF. Methods: We included IPF subjects from two separate cohorts at...

Rheumatoid arthritis (RA) is a common systemic autoimmune disease whose fibro-inflammatory manifestations may affect a number of tissues and organs, including the lungs. In fact, interstitial lung disease (ILD) is a leading cause of mortality among patients with RA. RA-related interstitial lung disease (RA-ILD) most often presents in an injury patt...

Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with poor quality of life. Debilitating symptoms and the reality of shortened survival impact patients’ physical and emotional well-being and constrain the lives of patients’ caregivers. This study assessed the informational needs of medical providers who care for patients with...

Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods...

RATIONALE: 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 descri...

Background Quality of life (QoL) is profoundly impaired in patients with idiopathic pulmonary fibrosis (IPF). However, data is limited regarding the course of QoL. We therefore analysed longitudinal data from the German INSIGHTS-IPF registry. Methods Clinical status and QoL were assessed at enrollment and subsequently at 6- to 12-months intervals....

Background: Patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) often experience impaired health status. In daily clinical practice, a short and easy instrument for assessing health status would be useful to help better understand the patient's condition. The COPD Assessment Test (CAT) is a simple questionnaire a...

Idiopathic pulmonary fibrosis (IPF) was once thought to be a rare disease and has been classically described as a disease that progresses in a “relentless and often insidious manner.” However, recent epidemiologic studies have revealed the true burden of this disease on society and identified risk factors for the development of disease that may ult...

Pulmonary rehabilitation and supplemental oxygen therapy are two interventions commonly employed as adjuncts to pharmacotherapy in the treatment of patients with idiopathic pulmonary fibrosis (IPF). Results from a number of randomized, controlled trials suggest that pulmonary rehabilitation is associated with several beneficial effects in patients...

The effects of interstitial lung disease (ILD) create a significant burden on patients, unsettling almost every domain of their lives, disrupting their physical and emotional well-being and impairing their quality of life (QoL). Because many ILDs are incurable, and there are limited reliably-effective, life-prolonging treatment options available, t...

More than 1.5 million adults in the United States use supplemental oxygen for a variety of respiratory disorders to improve their quality of life and prolong survival. This document describes recommendations from a multidisciplinary workshop convened at the ATS International Conference in 2017 with the goal of optimizing home oxygen therapy for adu...

Objective: Interstitial lung disease (ILD) is commonly associated with rheumatoid arthritis (RA) and can have significant morbidity and mortality. The objective of this study was to calculate the prevalence, incidence, healthcare costs, and mortality of RA-related ILD (RA-ILD) in the United States. Methods: This retrospective cohort analysis use...

Objective: This paper aims to determine the reliability and validity of the Chinese version of a tool that assesses the quality of life in idiopathic pulmonary fibrosis (cATAQ-IPF) in patients with interstitial lung disease (ILD). Methods: We used the process of scale introduction to establish cATAQ-IPF. The content validity of the scale was eva...

Rationale: The optimal method of physical activity measurement has not been determined in patients with fibrotic interstitial lung disease (ILD). Objectives: To assess the validity, internal consistency, and responsiveness of the International Physical Activity Questionnaire long form (IPAQ-LF) and to estimate the minimal important difference (M...

We evaluated performance characteristics and estimated the minimal clinically important difference (MCID) of data-driven texture analysis (DTA), a high-resolution computed tomography (HRCT)-derived measurement of lung fibrosis, in subjects with idiopathic pulmonary fibrosis (IPF). The study population included 141 subjects with IPF from two interve...

Background: Antifibrotics are recommended for the treatment of individuals with idiopathic pulmonary fibrosis (IPF), but treatment use remains at ∼60%. Objective: To investigate the views of individuals with IPF and pulmonologists on the diagnosis and management of IPF to understand treatment patterns. Methods: Interviews and/or online surveys...

Introduction Temporary dose modifications, such as reductions or interruptions, may allow patients to better manage adverse events (AEs) associated with pirfenidone use and continue treatment for idiopathic pulmonary fibrosis (IPF). However, the impact of such dosing adjustments on efficacy and safety is uncertain. Methods Patients randomised to r...

Patients with idiopathic pulmonary fibrosis (IPF) face a poor prognosis and endure intrusive symptoms that impair quality of life. Many patients with IPF will require supplemental oxygen (O2) at some point in the course of their illness, and although it can improve blood oxygen and symptoms, O2 creates physical and emotional challenges for patients...

Introduction We evaluated the psychometric properties of the St George’s Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis (IPF) using data from the two INPULSIS trials. Methods Data from 1061 patients treated with nintedanib or placebo were pooled. Internal consistency, test–retest reliability, construct validity, kn...

Purpose: The purpose of this article was to determine whether a novel finding on coronal computed tomography (CT) can help differentiate usual interstitial pneumonia (UIP) from nonspecific interstitial pneumonia (NSIP) in order to obviate lung biopsy. Materials and methods: Two chest radiologists, blinded to clinical data, reviewed 3 preselected...

Background and objective The St George’s Respiratory Questionnaire (SGRQ) is a self‐administered questionnaire used to assess health‐related quality of life (HRQoL) in various chronic respiratory diseases. Few studies have assessed the performance of the SGRQ in patients with connective tissue disease‐associated interstitial lung disease (CTD‐ILD)....

In lymphangioleiomyomatosis patients receiving sirolimus treatment, transient leukopenia in the morning may be due to circadian rhythm, with leukocyte counts recovering later in the day, indicating that a decrease in drug dose may not be warranted http://ow.ly/jPFz30iysgV.

Background May patients with interstitial lung disease (ILD) require supplementary oxygen (O2) therapy to maintain normoxia. However, ambulatory O2 delivery devices are constraining and cumbersome. The physiologic and symptomatic impact of these devices on ILD patients is unknown. Methods We conducted a prospective study of 30 clinically stable IL...

Background Idiopathic pulmonary fibrosis (IPF) is a progressive, incurable lung disease whose intrusive symptoms rob patients of their quality of life. Patients with IPF rely on their caregivers for support and assistance in amounts that vary according to patients’ individual circumstances and disease severity. Knowledgeable and well-informed patie...

In 2000, the American Thoracic Society and European Respiratory Society published the first consensus statement providing guidelines on the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF). This statement presented, for the first time, diagnostic criteria for IPF and recommendations for treatment. Results from several studies have res...

Introduction A majority of patients with IPF do not receive antifibrotic therapy with pirfenidone or nintedanib. We investigated viewpoints about IPF care and treatment amongst patients, and amongst physicians with a ‘watch and wait’ approach (WWP) or a proactive approach (PP). Methods Participants from Europe and Canada took part in an online sur...

Background Patients with pulmonary fibrosis (PF) have impaired quality of life, and research suggests that dyspnea and physical activity are primary drivers. As PF progresses, some patients notice the disease “shrinks their worlds”. The objective of this study is to describe movement (both physical activity and activity space) in a cohort of patien...

Abstract Background Dyspnea is the hallmark symptom of pulmonary fibrosis. Supplemental oxygen (O2) is prescribed to many patients with pulmonary fibrosis in hopes of alleviating dyspnea and improving physical functioning. We used response data from the University of California San Diego Shortness of Breath Questionnaire (UCSD) which was administer...

Objective: Patients with systemic sclerosis (SSc) often develop interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH). The effect of ILD and PAH on healthcare costs among patients with SSc is not well described. The objective of this analysis was to describe healthcare costs in patients with newly diagnosed SSc and SSc patie...

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It is associated with increasing cough and dyspnoea and impaired quality of life. IPF affects ∼3 million people worldwide, with incidence increasing dramatically wi...