Usability of a smartphone application for patients with interstitial lung disease: Results from the Registry for Better Understanding of ILD (RE-BUILD) pilot study.

Background And Objective: Digital technologies offer opportunities for remote monitoring, increased patient engagement and incorporation of patient-reported outcome measures (PROMs) into interstitial lung disease (ILD) care and research. This study evaluated the usability and patient experience of the RE-BUILD (Registry for Better Understanding of ILD) application, an ILD-specific smartphone app.

Methods: Patients with ILD aged ≥18 years were recruited from three tertiary ILD centres to use the RE-BUILD app for 6 months.

Hermansky-Pudlak Syndrome: An unusual pattern of pulmonary fibrosis.

Hermansky-Pudlak Syndrome is a rare genetic cause of pulmonary fibrosis, associated with albinism, nystagmus, and a bleeding diathesis. Histologically, Hermansky-Pudlak Syndrome Pulmonary Fibrosis (HPS-PF) typically resembles usual interstitial pneumonia (UIP), however radiologically this is not always the case with a range of features described in the current literature. HPS-PF typically occurs earlier in life than idiopathic pulmonary fibrosis (IPF) and there is limited evidence to support the use of antifibrotic therapy.

Implications of the 2022 lung function update and GLI global reference equations among patients with interstitial lung disease.

Background: Lung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and the updated interpretation strategies remain uncertain.

Methods: Adult patients with ILD with baseline forced vital capacity (FVC) were included from the Australasian ILD registry and the National Healthcare Group ILD registry, Singapore.

A self-management package for pulmonary fibrosis: A feasibility study.

Background And Objective: There is currently no self-management package designed to meet the needs of people with pulmonary fibrosis (PF). This study evaluated the feasibility and acceptability of a PF-specific self-management package.

Methods: Adults with PF were randomly allocated (1:1) to either receive the self-management package with healthcare professional (HCP) support or standardised PF information.

Trimethoprim-sulfamethoxazole acute respiratory distress syndrome requiring lung transplantation.

Trimethoprim-sulfamethoxazole (TMP-SMX) acute respiratory distress syndrome (ARDS) is a rare, but severe complication of a commonly prescribed antibiotic. TMP-SMX typically affects young, otherwise well patients with a specific human leukocyte antigen type (HLA-B*07:02 and HLA-C*07:02). The condition is poorly understood with a unique pathological appearance and mechanism that remains unclear.