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http://dx.doi.org/10.1016/j.jaad.2025.01.002 | DOI Listing |
Exp Biol Med (Maywood)
January 2025
Department of Pediatric Surgery, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China.
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with a poor prognosis. Its non-specific clinical symptoms make accurate prediction of disease progression challenging. This study aimed to develop molecular-level prognostic models to personalize treatment strategies for IPF patients.
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December 2024
Paediatrics and Paediatric Respirology, National Heart and Lung Institute, Faculty of Medicine, Imperial College London, London, United Kingdom.
Ataxia-telangiectasia (A-T) is an ultrarare autosomal recessive disorder and occurs in all racial and ethnic backgrounds. Clinically, children and young people with A-T are affected by sinopulmonary infections, neurological deterioration with concomitant bulbar dysfunction, increased sensitivity to ionizing radiation, immunodeficiency, a decline in lung function, chronic liver disease, endocrine abnormalities, cutaneous and deep-organ granulomatosis, and early death. Pulmonary complications become more frequent in the second decade of life and are a leading cause of death in individuals with A-T.
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January 2025
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Introduction: The critical role played by vascular dysfunction and ineffective angiogenesis in the pathophysiology of systemic sclerosis (SSc) suggests that circulating biomarkers reflecting these alterations may be useful in the clinical evaluation of this patient group. We sought to address this issue by conducting a systematic review and meta-analysis of studies investigating a such candidate biomarker, endostatin, an endogenous glycoprotein exerting anti-angiogenic effects, in SSc patients and healthy controls.
Methods: A literature search was conducted in the electronic databases Web of Science, PubMed, and Scopus from inception to 27 May 2024.
RMD Open
January 2025
Service de Rhumatologie, Hôpital Cochin, APHP-Centre Université Paris Cité, Paris, France
Objective: To examine the course of interstitial lung disease associated with rheumatoid arthritis (RA-ILD) in France on treatment with Janus kinase inhibitors (JAKis) using the MAJIK-SFR registry.
Methods: Prospective national multicentre observational study identifying patients with RA-ILD from the MAJIK-SFR registry. Pulmonary assessment data were collected at JAKi initiation and follow-up visits (6 months, 12 months and a median of 21 months postinclusion), including chest high-resolution CT (HRCT), pulmonary function tests (forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO)), acute exacerbations of ILD, respiratory infections and lung cancers.
J Comput Assist Tomogr
November 2024
From the Department of Medical Imaging, Jiangyin Hospital Affiliated to Nanjing University of Chinese Medicine, Jiangyin City, Jiangsu Province, China.
Objectives: The aims of the study are to predict lung function impairment in patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) through computed tomography (CT) quantitative analysis parameters based on CT deep learning model and density threshold method and to assess the severity of the disease in patients with CTD-ILD.
Methods: We retrospectively collected chest high-resolution CT images and pulmonary function test results from 105 patients with CTD-ILD between January 2021 and December 2023 (patients staged according to the gender-age-physiology [GAP] system), including 46 males and 59 females, with a median age of 64 years. Additionally, we selected 80 healthy controls (HCs) with matched sex and age, who showed no abnormalities in their chest high-resolution CT.
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