An App Platform-Facilitated Collaborative Palliative Care Intervention for Outpatients With Interstitial Lung Disease: A Pilot Randomized Trial.

Rationale: Outpatients with interstitial lung disease often experience serious symptoms, yet infrequently receive palliative care. Objective: To determine the feasibility and clinical impact of a mobile application (PCplanner) in an outpatient setting. Methods: We conducted a pilot randomized controlled trial among adults with interstitial lung disease in a single-center academic clinic. Clinical outcomes included change in Needs at the End-of-Life Screening Tool (NEST) scale between baseline and 3 months as well as frequency of advance care planning discussions and referrals to palliative care services. Results: Observed feasibility outcomes were similar to targeted benchmarks including randomization rates (82.1% vs 80%) and retention (84.8% vs 80%). Mean NEST scores between the intervention and control group were 38.9 (SD, 18.9) vs 41.5 (SD, 20.5) at baseline, 34.6 (SD, 18.9) vs 33.6 (SD, 19.4) at 1 month after clinic visit, 40.5 (SD, 21.6) vs 35.3 (SD, 25.0) at 3 months after clinic visit. Changes in NEST scores between baseline and 3 months showed no difference in the primary outcome (P = 0.481, 95% CI [-8.45, 17.62]). Conclusion: Among patients with interstitial lung disease, a mobile app designed to focus patients and clinicians on palliative care principles demonstrated evidence of feasibility. Although changes in self-reported needs were similar between intervention and control groups, more patients in the intervention group updated their advance directives and code status compared to the control group. Clinical Trial Registration: Palliative Care Planner (PCplanner) NCT05095363. https://www.clinicaltrials.gov/study/NCT05095363.

E-Cigarette Use, Small Airway Fibrosis, and Constrictive Bronchiolitis.

BACKGROUND: Vaping, including the use of electronic cigarettes (e-cigarettes), has become increasingly prevalent, yet the associated long-term health risks are largely unknown. Given the prevalence of use, particularly among adolescents early in their lifespan, it is vital to understand the potential chronic pathologic sequelae of vaping. METHODS: We present the cases of four patients with chronic lung disease associated with e-cigarette use characterized by clinical evaluation, with pulmonary function tests (PFTs), chest high-resolution computed tomography (HRCT), endobronchial optical coherence tomography (EB-OCT) imaging, and histopathologic assessment. RESULTS: Each patient presented with shortness of breath and chest pain in association with a 3- to 8-year history of e-cigarette use, with mild progressive airway obstruction on PFTs and/or chest HRCT findings demonstrating evidence of air trapping and bronchial wall thickening. EB-OCT imaging performed in two patients showed small airway-centered fibrosis with bronchiolar narrowing and lumen irregularities. The predominant histopathologic feature on surgical lung biopsy was small airway-centered fibrosis, including constrictive bronchiolitis and MUC5AC overexpression in all patients. Patients who ceased vaping had a partial, but not complete, reversal of disease over 1 to 4 years. CONCLUSIONS: After thorough evaluation for other potential etiologies, vaping was considered to be the most likely common causal etiology for all patients due to the temporal association of symptomatic chronic lung disease with e-cigarette use and partial improvement in symptoms after e-cigarette cessation. In this series, we associate the histopathologic pattern of small airway-centered fibrosis, including constrictive bronchiolitis, with vaping, potentially defining a clinical and pathologic entity associated with e-cigarette use. (Funded in part by the National Institutes of Health.).

Antifibrotic Therapy: Is There a Role in Myositis-Interstitial Lung Disease?

Interstitial lung disease (ILD) is a cause of substantial morbidity and mortality amongst autoimmune diseases, including myositis. Despite first-line therapy with immunosuppression, many inflammatory ILDs advance to a fibrotic stage. In such patients, progressive fibrosis may be amenable to treatment with antifibrotic medications, which were initially studied and approved for the treatment of idiopathic pulmonary fibrosis. We here review the available data that support the use of antifibrotics in connective tissue diseases and progressive fibrosing ILDs. There is now a growing body of evidence in both large randomized clinical trials and on the evolving pathophysiologic pathways to support the use of antifibrotics in select patients with autoimmune ILD and a fibrotic phenotype. Further study of antifibrotics in combination with immunosuppressive medications, and in the myositis-ILD population, is needed.