Background: Currently, two disease-modifying antifibrotic drugs are indicated for the treatment of idiopathic pulmonary fibrosis. The objective of this study was to analyse antifibrotic and overall prescription medication use of IPF patients in the real world.
Methods: Data was collected from the FinnishIPF registry and the Registry of the Social Insurance Institution of Finland (SII). Purchases of all prescription medicines were assessed. The frequency, the initiation interval, the duration, and the breaks of the antifibrotic treatments were defined. The association between the prescription of antifibrotic therapy and different patient-related clinical parameters was studied. Accordingly, the relationships between the delay in starting therapy and patient-related variables were analysed.
Results: Of the 263 IPF patients, 132 (50.2%) had started antifibrotic treatment during the study period 2011-2018. The mean interval from the diagnosis to the first purchase was 367 (SD 429) days. The antifibrotic drug was switched in 14% of patients. Discontinuation of therapy occurred most commonly during the first year of the treatment. The one-year persistence was 77.1% for pirfenidone and 78.9% for nintedanib. A tendency of treating patients under 75 years was noticed. Low forced vital capacity predicted earlier initiation of medication.
Conclusions: The initiation of antifibrotics after diagnosis was slow, probably due to reimbursement limitations. Younger age at diagnosis affected treatment initiation although it is unknown which patients benefit most from these medications. The reasons for discontinuation of the antifibrotic therapy during the first year should be a focus in clinical work and further studies.
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http://dx.doi.org/10.1186/s12890-023-02630-1 | DOI Listing |
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.
Clin Rheumatol
January 2025
Department of Pediatric Rheumatology, Zeynep Kamil Women and Children's Diseases Training and Research Hospital, Istanbul, Turkey.
Introduction/objectives: The study aimed to determine whether in children with newly diagnosed juvenile idiopathic arthritis (JIA) hepatitis B surface antibody (anti-HBs) differs from healthy children and to see whether the revaccination is safe and effective under JIA treatment.
Methods: Patients who were followed up with a diagnosis of JIA between January 2020 and February 2024 were included. The control group consisted of healthy children matched for age and gender.
Clin Rheumatol
January 2025
Division of Pediatric Rheumatology and Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.
Turk J Pediatr
December 2024
Division of Pediatric Nephrology, İstanbul Faculty of Medicine, İstanbul University, İstanbul, Türkiye.
Background: Idiopathic nephrotic syndrome (NS) is the most prevalent glomerular disease in children. Heat shock protein 70 (HSP70) is synthesized in response to diverse stress factors like infections and oxidative stress. We aimed to evaluate serum and urine levels of HSP70 in children with steroid-sensitive nephrotic syndrome (SSNS) and to assess changes in HSP70 levels with prednisolone treatment.
View Article and Find Full Text PDFTurk J Pediatr
December 2024
Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Türkiye.
Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.
Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels.
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