A multi-disciplinary task force of respiratory experts has published a new statement document to guide best practice in the diagnosis and treatment of pulmonary disease in patients with alpha-1 antitrypsin deficiency (AATD).
The Task Force statement is published today (30 November, 2017) in the European Respiratory Journal.
The statement provides a broad update on “state-of-the-art” knowledge in the study and management of pulmonary disease associated with AATD.
Task force members compiled a list of issues that they considered important and relevant to the diagnosis and management of pulmonary disease in AATD, including:
- Laboratory diagnosis and hierarchy of testing
- Monitoring the progression of lung disease in AATD; including physiology/lung function, CT densitometry
- The risk of lung disease in heterozygotes
- Role and benefits of screening
- Augmentation therapy for AATD
- Lung volume reduction surgery in AATD
- New lines of research in AATD
- Access to optimal care and augmentation therapy for AATD in Europe
The updated statement provides guidance on new novel, more accurate and less expensive genetic diagnostic methods, new outcome parameters for use in clinical trials, and more evidence concerning the efficacy and safety of augmentation therapy.