Publications by authors named "Mark G J P Platenburg"
Background And Objective: Real-world data on lung function course of patients with progressive pulmonary fibrosis (PPF) treated with anti-fibrotic medication are limited. We evaluated forced vital capacity (FVC) decline in patients with PPF and idiopathic pulmonary fibrosis (IPF) who started anti-fibrotic treatment.
Methods: This was a nationwide multi-centre registry study in 16 hospitals throughout the Netherlands.
Article Synopsis
- Pirfenidone and nintedanib are antifibrotic drugs that help slow the decline of lung function in patients with idiopathic pulmonary fibrosis (IPF), and real-world data suggests they may improve survival, though the evidence is mixed in clinical trials.
- The study investigated whether IPF patients receiving these medications have better transplant-free survival compared to those not receiving treatment, while also considering different GAP stages of the disease, which classify the severity of the condition.
- Results from 457 patients indicated that those treated with antifibrotics had significantly longer median survival rates and lower cumulative mortality rates, particularly in GAP stages II and III, highlighting the advantages of these medications in managing IPF.
Decreased Survival and Lung Function in Progressive Pulmonary Fibrosis.
Medicina (Kaunas)
February 2023
Progressive pulmonary fibrosis (PPF) is a recently described term reserved for patients with fibrotic ILD other than idiopathic pulmonary fibrosis (IPF) with fast clinical deterioration. Here, survival and prognostic biomarkers at the time of diagnosis for PPF are investigated in a fibrotic ILD other than IPF cohort (non-IPF). : Patients diagnosed during the period of 2012-2018 at the ILD Center of Excellence (St.
View Article and Find Full Text PDFBackground: In some patients with progressive fibrosing interstitial lung disease (ILD), disease is caused by carriage of a mutation in a surfactant-related gene (SRG) such as SFTPC, SFTPA2, or ABCA3. However, no aggregated data on disease evolution and treatment outcome have been presented for these patients.
Research Question: In adult patients with ILD with an SRG mutation, what is the course of lung function after diagnosis and during treatment and the survival in comparison with patients with sporadic idiopathic pulmonary fibrosis (sIPF) and familial pulmonary fibrosis (FPF)?
Study Design And Methods: We retrospectively examined the clinical course of a cohort of adults with an SRG mutation by screening 48 patients from 20 families with an SRG mutation for availability of clinical follow-up data.
Background: Serologic testing for autoantibodies is recommended in interstitial lung diseases (ILDs), as connective tissue diseases (CTDs) are an important secondary cause. Myositis antibodies are associated with CTD-ILD, but clinical associations with other ILDs are unclear. In this study, associations of myositis antibodies in various ILDs were evaluated.
View Article and Find Full Text PDFConnective tissue diseases (CTDs) are an important secondary cause of interstitial lung disease (ILD). If a CTD is suspected, clinicians are recommended to perform autoantibody testing, including for myositis autoantibodies. In this study, the prevalence and clinical associations of novel myositis autoantibodies in ILD are presented.
View Article and Find Full Text PDFPurpose Of Review: The current review aims to recognize the variability in clinical presentation of adult patients with bi-allelic ABCA3 mutations, create more depth in ABCA3 mutations reported and highlight the influence of environmental factors on disease course.
Recent Findings: Mutations in ABCA3 are predominantly linked to neonatal and pediatric interstitial lung disease (ILD) with a minority surviving beyond puberty. Here, we present three patients with ABCA3 mutations who present with disease at the age of 19, 61 and 77.