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Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON.
Lung
January 2025
National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, UMR 754, ERN-LUNG, Lyon, France.
Purpose: In the INBUILD trial in patients with progressive pulmonary fibrosis (PPF), nintedanib slowed the decline in forced vital capacity (FVC) versus placebo, with a safety profile characterised mainly by gastrointestinal events. INBUILD-ON, the open-label extension of INBUILD, assessed the safety of nintedanib during longer-term treatment. Data on FVC were collected.
View Article and Find Full Text PDFExercise-Induced Oxygen Desaturation and Outcomes After Nintedanib Therapy for Fibrosing Interstitial Lung Disease in Patients Without Dyspnea.
J Clin Med
December 2024
Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Asahi-Machi 67, Kurume 830-0011, Japan.
The degree of exercise-induced oxygen desaturation and outcomes following antifibrotic drug therapy in asymptomatic patients with fibrosing interstitial lung disease (FILD) remain unclear. We compared clinical data, incidence of annual FILD progression, overall survival, and tolerability after initiating nintedanib between 58 patients with dyspnea and 18 patients without. Annual FILD progression was defined as >10% decrease in forced vital capacity (FVC), >15% decrease in diffusing capacity of the lungs for carbon monoxide (D), developing acute exacerbations, or FILD-related death within 1 year of starting nintedanib.
View Article and Find Full Text PDFTwo Novel Biallelic Variants in the Gene: The First Italian Case and a Literature Review.
Genes (Basel)
December 2024
Translational Cytogenomics Research Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
: The gene encodes for the catalytic α subunit of Cytoplasmic phenylalanine-tRNA synthetase (FARS1), an essential enzyme for protein biosynthesis in transferring its amino acid component to tRNAs. Biallelic pathogenic variants have been associated with a multisystemic condition, characterized by variable expressivity and incomplete penetrance. Here, we report the case of an 11 year-old girl presenting interstitial lung disease, supratentorial leukoencephalopathy with brain cysts, hepatic dysfunction, hypoalbuminemia, skin and joint hyperlaxity, growth retardation, and dysmorphic features.
View Article and Find Full Text PDFA Complex Case of Acute Liver Failure Following Idiosyncratic Drug-Induced Liver Injury, Potentiated by Herpes Simplex Virus Infection.
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de São José, Lisbon, PRT.
Acute liver failure (ALF) is a rare, life-threatening condition that may be secondary to drug-induced liver injury (DILI) and certain viral infections. We present the case of a 73-year-old male with a history of fibrotic hypersensitivity pneumonitis with a progressive phenotype, type 2 diabetes mellitus, hypertension, and hyperlipidemia, who was admitted with ALF potentially secondary to DILI. Prior to admission, he was receiving therapy that may be related to idiosyncratic DILI (I-DILI) and ALF, namely nintedanib, which appears to have a most probable relation to I-DILI in this case, considering it was the most recently started drug.
View Article and Find Full Text PDFPersonalized Antifibrotic Therapy in CKD Progression.
J Pers Med
December 2024
Department of Nephrology, Ghent University Hospital, 9000 Ghent, Belgium.
Chronic kidney disease (CKD) is a chronic disorder characterized by kidney fibrosis and extracellular matrix accumulation that can lead to end-stage kidney disease. Epithelial-to-mesenchymal transition, inflammatory cytokines, the TGF-β pathway, Wnt/β-catenin signaling, the Notch pathway, and the NF-κB pathway all play crucial roles in the progression of fibrosis. Current medications, such as renin-angiotensin-aldosterone system inhibitors, try to delay disease development but do not stop or reverse fibrosis.
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