This is an exciting time for those interested in ɑ1-antitrypsin deficiency (AATD): a greater understanding of the science underpinning pathophysiological mechanisms is translating into trials of innovative therapies, and knowledge about the role of augmentation therapy is changing with the recent publication of well-conducted trials using novel CT imaging end-points. The latest ERS Monograph on ɑ1-Antitrypsin Deficiency is therefore timely.
Guest Editors Pavel Strnad, Mark L. Brantly and Robert Bals have put together a Monograph that offers a comprehensive and up-to-date overview of AATD. It features a patient perspective chapter that sets the tone, then goes on to cover basic biology, genetics, laboratory diagnostics and the major organ manifestations. The Guest Editors acknowledge that liver infection in AATD has been studied even less than lung involvement; indeed, the burden of liver disease in AATD is only recently being appreciated. With this in mind, they have included chapters that focus on the mechanisms of liver disease, diagnosis and assessment of AATD, and the clinical approach to liver disease in both adults and children.
The book has been tailored to meet the needs of the physician, who takes care of lung and liver patients in daily practice, but also includes useful information for the general practitioner, who is responsible for the medical guidance of these patients. It should be essential reading for those interested in AATD and the “go to” reference work on the topic for many years to come.
For online access to the book, visit books.ersjournals.com (access is free for ERS members) or order a print copy from ersbookshop.com
You can view related figures:
- ERM-0326-2018: Chest computed tomography showing panacinar emphysema in both lung bases with a few instances of cylindrical bronchiectasis.
- EDU-0330-2018: Histological appearance of AAT aggregates in a 58-year-old female patient carrying a homozygous Glu342Lys substitution (Pi*ZZ genotype). Arrows highlight location of aggregates.