Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry

Rationale Progressive fibrosing interstitial lung disease (PF-ILD) is characterized by progressive physiologic, 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 2015-2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation, or any 2 of: relative FVC decline ≥5 and <10%, worsening respiratory symptoms, or worsening fibrosis on computed tomography of the chest, all within 24 months of diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups. Characteristics associated with progression were determined by multivariable regression. Results Of 2,746 patients with fibrotic ILD (mean age 65±12 years, 51% female), 1,376 (50%) met PFILD criteria in the first 24 months of follow-up. PF-ILD occurred in 427 (59%) patients with idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP), 281 (51%) with unclassifiable ILD (U-ILD), and 402 (45%) with connective tissue diseaseassociated ILD (CTD-ILD). Compared to IPF, time to progression was similar in patients with HP (hazard ratio [HR] 0.96, 95% confidence interval, CI 0.79-1.17), but was delayed in patients with U-ILD (HR 0.82, 95% CI 0.71-0.96) and CTD-ILD (HR 0.65, 95% CI 0.56-0.74). Background treatment varied across diagnostic subtypes with 66% of IPF patients receiving antifibrotic therapy, while immunomodulatory therapy was utilized in 49%, 61%, and 37% of patients with CHP, CTD-ILD, and U-ILD respectively. Increasing age, male sex, gastroesophageal reflux disease, and lower baseline pulmonary function were independently associated with progression. Interpretation Progression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF. Routinely collected variables help identify patients at risk for progression and may guide therapeutic strategie

What to Do with All of These Lung Nodules?

Caplan syndrome is a rare entity that is specific to rheumatoid arthritis and presents with multiple, well-defined necrotic nodules in patients with occupational dust exposure. The present report describes a case of Caplan syndrome involving a 71-year-old man with a known diagnosis of seropositive rheumatoid arthritis who presented to the authors’ centre with a five-year history of multiple, bilateral cavitary lung nodules with mild dyspnea on exertion. He was an ex-smoker (30 pack-years) and had previously worked with silica. The case highlights the clinical, radiological and pathological features of this syndrome and outlines the importance of considering a broad differential in the management of pulmonary nodules, especially in patients with rheumatoid arthritis

What to Do with All of These Lung Nodules?

Caplan syndrome is a rare entity that is specific to rheumatoid arthritis and presents with multiple, well-defined necrotic nodules in patients with occupational dust exposure. The present report describes a case of Caplan syndrome involving a 71-year-old man with a known diagnosis of seropositive rheumatoid arthritis who presented to the authors’ centre with a five-year history of multiple, bilateral cavitary lung nodules with mild dyspnea on exertion. He was an ex-smoker (30 pack-years) and had previously worked with silica. The case highlights the clinical, radiological and pathological features of this syndrome and outlines the importance of considering a broad differential in the management of pulmonary nodules, especially in patients with rheumatoid arthritis.Peer Reviewe

Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Peer Reviewe

Diagnostic Disparity of Previous and Revised American Thoracic Society Guidelines for Idiopathic Pulmonary Fibrosis

BACKGROUND: A revised guideline for the diagnosis of idiopathic pulmonary fibrosis (IPF) was formulated by the American Thoracic Society (ATS) in 2011 to improve disease diagnosis and provide a simplified algorithm for clinicians. The impact of these revisions on patient classification, however, remain unclear.OBJECTIVE: To examine the concordance between diagnostic guidelines to understand how revisions impact patient classification.METHODS: A cohort of 54 patients with either suspected IPF or a working diagnosis of IPF was evaluated in a retrospective chart review, in which patient data were examined according to previous and revised ATS guidelines. Patient characteristics influencing the fulfillment of diagnostic criteria were compared using one-way ANOVA and χ2 tests.RESULTS: Revised and previous guideline criteria for IPF were met in 78% and 83% of patients, respectively. Revised guidelines modified a classification based on previous guidelines in 28% of cases. Fifteen percent of patients meeting previous ATS guidelines failed to meet revised criteria due to a lack of honeycombing on high-resolution computed tomography and the absence of a surgical lung biopsy. Patients failing to meet previous and revised diagnostic criteria for IPF were younger.CONCLUSION: The revised guidelines for the diagnosis of IPF classify a substantial proportion of patients differently than the previous guidelines.Peer Reviewe

An unusual case of interstitial lung disease: Revisiting peribronchiolar metaplasia interstitial lung disease (PBM‐ILD)

Abstract Peribronchiolar metaplasia (PBM) is a histological finding of uncertain significance commonly seen in interstitial lung disease (ILD). PBM is thought to be secondary to small airway injury from insults such as tobacco smoke and other environmental exposures. The term PBM‐ILD has been proposed for patients with ILD where PBM is the major histologic finding, however a lack of radiographic changes supportive of ILD in previously reported cases has limited recognition of the diagnosis. We present a rare case of welding‐associated ILD with clinical, radiographic, and histologic evidence consistent with the proposed definition of PBM‐ILD. We outline an approach to its consideration as a diagnosis based on our experience through multidisciplinary discussion

Managing the Risk of Lung Toxicity with Trastuzumab Deruxtecan (T-DXd): A Canadian Perspective

Ongoing advances in precision cancer therapy have increased the number of molecularly targeted and immuno-oncology agents for a variety of cancers, many of which have been associated with a risk of pulmonary complications, among the most concerning being drug-induced interstitial lung disease/pneumonitis (DI-ILD). As the number of patients undergoing treatment with novel anticancer agents continues to grow, DI-ILD is expected to become an increasingly significant clinical challenge. Trastuzumab deruxtecan (T-DXd) is an antibody–drug conjugate targeting human epidermal growth factor receptor 2 that is gaining widespread use in the metastatic breast cancer setting and is undergoing exploration for other oncologic indications. ILD/pneumonitis is an adverse event of special interest associated with T-DXd, which has potentially fatal consequences if left untreated and allowed to progress. When identified in the asymptomatic stage (grade 1), T-DXd-related ILD can be monitored and treated effectively with the possibility of treatment continuation. Delayed diagnosis and/or treatment, however, results in progression to grade 2 or higher toxicity and necessitates immediate and permanent discontinuation of this active agent. Strategies are, therefore, needed to optimize careful monitoring during treatment to ensure patient safety and optimize outcomes. Several guidance documents have been developed regarding strategies for the early identification and management of T-DXd-related ILD, although none have been within the context of the Canadian health care environment. A Canadian multidisciplinary steering committee was, therefore, convened to evaluate existing recommendations and adapt them for application in Canada. A multidisciplinary approach involving collaboration among medical oncologists, radiologists, respirologists, and allied health care professionals is needed to ensure the proactive identification and management of T-DXd-related ILD and DI-ILD associated with other agents with a similar toxicity profile