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The impact of highly effective modulator therapy on sinusitis and dysosmia in young children with cystic fibrosis: a prospective study protocol.
ERJ Open Res
January 2025
Department of Otolaryngology-Head and Neck Surgery, University of California, Los Angeles, Los Angeles, CA, USA.
Background: Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are prevalent disease complications in people with cystic fibrosis. These understudied comorbidities significantly impact quality of life. The impact of highly effective modulator therapy (HEMT) in young children with cystic fibrosis (YCwCF) on these disease complications is unknown.
View Article and Find Full Text PDFSurvival and early outcomes following lung transplantation for interstitial lung disease associated with non-scleroderma connective tissue disease: a national cohort study.
Clin Exp Rheumatol
January 2025
Department of Organ Transplantation, and Department of Thoracic Surgery and Oncology, the First Affiliated Hospital of Guangzhou Medical University, State Key Laboratory of Respiratory Disease and National Clinical Research Center for Respiratory Disease, Guangzhou, China.
Objectives: The progressive decline in interstitial lung disease associated with non-scleroderma connective tissue disease (ILD-NSCTD) is linked to poor prognosis and frequently results in respiratory failure. Lung transplantation (LTx) offers a viable treatment option, yet its outcomes in ILD-NSCTD remain contentious, particularly across different subtypes.
Methods: This retrospective cohort study included patients with idiopathic pulmonary fibrosis (IPF) (n=11,610) and ILD-NSCTD (n=610) listed in the United Network for Organ Sharing (UNOS) database who underwent lung transplantation between May 5, 2005, and December 31, 2022.
Background: Systemic sclerosis (SSc) is a rare connective tissue disease, frequently affecting the skin, lungs, and pulmonary vasculature. Approximately 30-50% of SSc patients develop interstitial lung disease (SSc-ILD), with 30-35% of related deaths attributed to it. Even though men are less likely to develop systemic sclerosis, they have a higher incidence of SSc-ILD than women, and they tend to develop it at a younger age with a higher mortality rate.
View Article and Find Full Text PDFErlotinib-induced Perioral Lesions Resembling Scleroderma.
Acta Dermatovenerol Croat
November 2024
Constantin A. Dasanu MD, PhD, Lucy Curci Cancer Center, Eisenhower Health, 39000 Bob Hope Dr, Rancho Mirage, CA 92270 , USA;
Erlotinib, an epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI), is currently used in the therapy of several solid malignancies. This agent has been associated with several dermatological side-effects, the most common being papulo-pustular acneiform rash. Herein we describe a unique skin effect in a patient treated with erlotinib for non-small cell lung cancer.
View Article and Find Full Text PDFLongitudinal association between nailfold capillaroscopy and incident interstitial lung disease: A EUSTAR database analysis.
J Scleroderma Relat Disord
January 2025
Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands.
Objectives: To evaluate (1) the association between nailfold capillaroscopy pattern and 5-year risk for incident interstitial lung disease and (2) the association between transition in nailfold capillaroscopy pattern and risk of incident interstitial lung disease.
Methods: Data of adult patients from the EUSTAR database fulfilling the ACR-EULAR criteria with a disease duration ⩽5 years, having a scleroderma pattern at nailfold capillaroscopy with high-resolution computed tomography confirmed absence of interstitial lung disease (i.e.
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