Precision Diagnosis and Care for Families With Pulmonary Fibrosis in Ireland

Launched by ROYAL COLLEGE OF SURGEONS, IRELAND · Nov 20, 2024

Keywords

ClinConnect Summary

This clinical trial, titled "Precision Diagnosis and Care for Families With Pulmonary Fibrosis in Ireland," is studying how genes and environmental factors contribute to pulmonary fibrosis, a serious lung disease that causes scarring of the lungs. The goal is to better understand why some people develop this condition, especially those with a family history of pulmonary fibrosis, and to identify ways to detect the disease early. The researchers will follow 300 participants over one year, including 100 people with idiopathic pulmonary fibrosis (IPF), 100 with familial pulmonary fibrosis (FPF), and 100 with interstitial lung disease related to connective tissue diseases (CTD ILD).

To join the study, participants must be diagnosed with a specific type of fibrotic lung disease and be willing to provide informed consent. They will undergo tests to help identify genetic factors that might influence the disease and may use at-home devices to monitor their lung function. This research aims to improve early detection and treatment options, ultimately helping patients and their families manage this challenging condition more effectively.

Gender

ALL

Eligibility criteria

• Inclusion Criteria:
• • Able and willing to give written informed consent.
• • An MDT diagnosis of fibrotic ILD which fall into one of the following three catagories;
• • 1. Have a multidisciplinary team (MDT) diagnosis of a fibrotic interstitial lung disease, reporting one or more relatives with a fibrotic form of ILD
• • 2. Have a MDT diagnosis of IPF in accordance with consensus criteria, ATS, ERS, JRS, ALAT guidelines without a family history of pulmonary fibrosis.
• • 3. Meet the American College of Rheumatology/European League Against Rheumatism criteria for rheumatoid arthritis, scleroderma, Sjogren's syndrome, idiopathic inflammatory myopathy and systemic lupus erythematosus.
• Exclusion Criteria:
• • Currently participating in an interventional clinic trial.
• • Change in clinical phenotype from initial radiological diagnosis to screening.
• • Acute or chronic hypersensitivity pneumonitis with consensus criteria (appropriate exposure history, radiological features ± avian and fungal precipitins).
• • Asbestosis (appropriate occupational history and radiological evidence of asbestos exposure)
• • Life expectancy for any disease, including ILD \<12 months (investigator assessment)
• • Major extrapulmonary physiological restriction (e.g. chest wall abnormality, large pleural effusion)