Interstitial Lung Disease Specialist Research Articles

The Interstitial Lung Disease Patient Pathway: From Referral To Diagnosis

BackgroundSuspected Interstitial Lung Disease (ILD) patients may be referred to an ILD-specialist centre or non-ILD-specialist centre for diagnosis and treatment. Early referral and management of patients at ILD-specialist centres has been shown to improve survival and reduce hospitalisations. The COVID-19 pandemic has affected the ILD patient diagnostic pathway and prompted centres to adapt.AimsThis study investigates and contrasts ILD patient pathways in ILD-specialist and non-ILD-specialist centres, focusing on referrals, caseloads, diagnostic tools, multi-disciplinary team (MDT) meeting practices, and resource accessibility.MethodsConducted as a cross-sectional study, a global self-selecting survey ran from September 2022 to January 2023. Participants included ILD specialists and healthcare professionals (HCPs) from ILD-specialist centres and non-ILD-specialist centres.ResultsOf 363 unique respondents from 64 countries, 259 were from ILD-specialist centres and 104 from non-ILD-specialist centres. ILD centres had better resource availability, exhibiting higher utilisation of diagnostic tests (median: 12 tests), than non-ILD centres (9 tests); and better access to specialist professions attending MDTs (median: 6 professions at meeting) in specialist centres than non-ILD centres (3 professions at meeting). Transitioning to virtual MDTs allowed HCPs from outside of centres to join meetings in nearly 90% of all centres, increasing regular participation in 60% of specialist centres and 72% of non-ILD centres. For treatment of patients, specialist centres had better access to antifibrotic drugs (91%) compared to non-ILD centres (60%).ConclusionsDiagnostic pathways for ILD patients diverged between specialist centres and non-ILD centres. Disparities in resource and specialist availability existed between centres.

PULMONARY NOCARDIOSIS AS A CONSEQUENCE OF DOMESTICALLY GROWN MARIJUANA

PULMONARY NOCARDIOSIS AS A CONSEQUENCE OF DOMESTICALLY GROWN MARIJUANA

Barriers to antigen detection and avoidance in chronic hypersensitivity pneumonitis in the United States

BackgroundChronic hypersensitivity pneumonitis (CHP) is an interstitial lung disease (ILD) caused by long term exposure to an offending antigen. Antigen avoidance is associated with improved outcomes. We are unable to identify the antigen source in approximately half of patients. When an antigen is successfully identified, patients have difficulty with avoidance.MethodsWe conducted three structured group discussions with US based ILD specialists utilizing the nominal group technique (NGT). Participants listed barriers to antigen detection and avoidance in CHP. Each participant ranked what they perceived to be the top three barriers in the list in terms of importance. The master list of barriers was consolidated across the three groups into themes that were prioritized based on receiving the highest rankings by participants.ResultsTwenty-five physicians participated; 56% had experience caring for CHP patients for ≥ 16 years. Sixty barriers to antigen detection were categorized into seven themes of which the top three were: 1. unclear significance of identified exposures; 2. gaps in clinical knowledge and testing capabilities; 3. there are many unknown and undiscovered antigens. Twenty-eight barriers to antigen avoidance were categorized into five themes of which the top three were: 1. patient limitations, financial barriers and lack of resources; 2. individual patient beliefs, emotions and attachments to the antigen source; and 3. gaps in clinical knowledge and testing capabilities.ConclusionsThis study uncovered challenges at the individual patient, organizational, and societal levels and ranked them in terms of level of importance. These findings provide information to guide development and validation of multidisciplinary support and interventions geared towards antigen identification and avoidance in CHP.

Programmed cell death 1 (PD-1)/PD-ligand 1(PD-L1) inhibitors-related pneumonitis in patients with advanced non-small cell lung cancer.

Immune‐related pneumonitis is an uncommon but potentially fatal immune‐related adverse event in advanced non–small cell lung cancer (NSCLC) patients during treatment with anti‐programmed cell death 1 (PD‐1) and programmed cell death‐ligand 1 (PD‐L1). Underlying emphysema, interstitial lung disease (ILD), and previous radiation therapy for lung cancer might be factors precipitating immune‐related pneumonitis. The incidence of immune‐related pneumonitis is reported to be higher in those treated with PD‐1 inhibitors than in those treated with anti‐PD‐L1 inhibitors. Early detection and diagnosis and appropriate management according to the severity are critical to improving the prognosis. The first‐line physicians, including the primary responsible oncologists, family doctors, emergency physicians and NSCLC patients should be trained to identify and report symptoms of immune‐related pneumonitis as early as possible. Multidisciplinary treatment teams involving clinicians (including ILD specialists and lung cancer specialists), radiologists and pathologists are recommended for the treatment of immune‐related pneumonitis.

How consistently do physicians diagnose and manage drug-induced interstitial lung disease? Two surveys of European ILD specialist physicians.

IntroductionCurrently there are no general guidelines for diagnosis or management of suspected drug-induced (DI) interstitial lung disease (ILD). The objective was to survey a sample of current European practice in the diagnosis and management of DI-ILD, in the context of the prescribing information approved by regulatory authorities for 28 licenced drugs with a recognised risk of DI-ILD.MethodsConsultant physicians working in specialist ILD centres across Europe were emailed two surveys via a website link. Initially, opinion was sought regarding various diagnostic and management options based on seven clinical ILD case vignettes and five general questions regarding DI-ILD. The second survey involved 29 statements regarding the diagnosis and management of DI-ILD, derived from the results of the first survey. Consensus agreement was defined as 75% or greater.ResultsWhen making a diagnosis of DI-ILD, the favoured investigations used (other than computed tomography) included pulmonary function tests, bronchoscopy and blood tests. The preferred method used to decide when to stop treatment was a pulmonary function test. In the second survey, the majority of the statements were accepted by the 33 respondents, with only four of 29 statements not achieving consensus when the responses “agree” and “strongly agree” were combined as one answer.ConclusionThe two surveys provide guidance for clinicians regarding an approach to the diagnosis and management of DI-ILD in which the current evidence base is severely lacking, as demonstrated by the limited information provided by the manufacturers of the drugs associated with a high risk of DI-ILD that we reviewed.

Abandoning developmental silos: what can paediatricians and adult interstitial lung disease physicians learn from each other?

Interstitial lung disease (ILD) consists of a large and heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic or pathologic manifestations. Overall, although there is overlap between adult and childhood ILD (chILD), there are many differences in disease causes and prevalences. In the last few years, our understanding of adult ILD pathobiology has improved substantially. This is particularly true for idiopathic pulmonary fibrosis, the most common of the idiopathic interstitial pneumonias, wherein recently developed guideline documents provide recommendations for the diagnosis and clinical management of patients. For chILD, similar guidelines are yet to be developed. However, complications and long-term pulmonary outcomes of paediatric disease are better appreciated, which make the implementation of a successful transition program from paediatric to adult care an urgent need. Similarly important is the development of guidelines on performance and interpretation of genetic testing in affected and unaffected relatives of familial cases and in children of adult-onset ILD patients. Lung transplantation appears to be as successful as in adult patients for end-stage disease. Paediatric pulmonologists should engage with the adult multidisciplinary teams and benefit from their much more extensive experience. Childhood and adult ILD share a number of aspects, which give children and adult ILD specialists exciting opportunities to collaborate and learn from each other. Such collaborative effort between child and adult ILD experts is crucial for successful future development in the field.

Visual analogue scales for interstitial lung disease: a prospective validation study.

Visual analogue scales (VAS) are simple symptom assessment tools which have not been validated in interstitial lung disease (ILD). Simple measures of ILD disease burden would be valuable for non-specialist clinicians monitoring disease away from ILD specialist centres. To validate VAS to assess change in dyspnoea, cough and fatigue in ILD, and to define the minimal clinically important difference (MCID) for change in these. Patients of 64 with ILD completed VAS for dyspnoea, cough and fatigue. Baseline King's Brief ILD questionnaire (K-BILD) scores, lung function and 6-min walk test results were collected. Tests were repeated 3-6 months later, in addition to a seven-point Likert scale. The MCID was estimated using median change in VAS in patients who reported 'small but just worthwhile change' in symptoms at follow-up. Methods were repeated in a validation cohort of 31 ILD patients to confirm findings. VAS scores were significantly higher for patients who reported a 'small but just worthwhile change' in symptoms vs. 'no change' or 'not worthwhile change' (P < 0.01). The MCID for VAS Dyspnoea was estimated as 22.0 mm and 14.5 mm for VAS Fatigue. These results were reproducible in the validation cohort. Results were not significant for VAS Cough. Change in VAS Dyspnoea correlated with change in K-BILD (r = -0.51, P < 0.01), forced vital capacity (r = -0.32, P = 0.01) and 6-min walking distance (r = -0.37, P = 0.01). The VAS is valid for assessing change in dyspnoea and fatigue in ILD. The MCID is estimated as 22.0 mm for dyspnoea and 14.5 mm for fatigue. This could be used to monitor disease in settings away from ILD specialist review. Lung Diseases, Interstitial, Dyspnoea, Fatigue, Cough.

Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age.

Idiopathic pulmonary fibrosis (IPF) is the most common cause of interstitial lung disease (ILD) and carries a worse prognosis than many cancers. Until recently, there were no active treatment options available for patients with IPF, meaning palliation or lung transplantation in selected patients were the only options. The management of IPF has changed dramatically over the last decade with the advent of two antifibrotic agents; pirfenidone and nintedanib. These new agents have been shown to reduce decline in lung function and pirfenidone has been shown to reduce mortality. The changing landscape of IPF diagnosis and management present a number of issues that may be encountered including management of side effects related to antifibrotic therapy. This article aims to give an overview of the holistic approach to the management of patients with IPF, including antifibrotic management, symptom management and the invaluable role of the ILD specialist nurse.