EARCO takes advantage of existing AATD registries that have been developed at the national
level. Several countries have established registries in which AATD patients are included and
followed-up with clinical and biological data collected. However, these registries differ in
terms of inclusion criteria, data collected and frequency and extent of follow-up. Within
EARCO, we will harmonize the data collection process and assess the quality of the data
within a short time frame after the data are generated and entered into the database.
We expect to collect detailed information from around 1,000 patients from at least 10
countries during the first year, expanding to 3,000 from more than 25 countries over the 5
years of the CRC and continue a long term follow-up. .
The study objectives are:
To generate long-term, high-quality clinical data covering a pan-European population of
AATD individuals in all age groups and all stages of disease severity.
To understand the natural history and prognosis of AATD better with the goal to create
and validate prognostic tools to support medical decision making.
To investigate the effect of augmentation therapy on the progression of emphysema and to
examine its impact on clinical and functional outcomes, such as FEV1, quality of life
and mortality in a "real-life" population
To learn more about the course of the disease in patients suffering from severe AATD
with genotypes different from Pi*ZZ
Study design:
The EARCO registry is a non-interventional, multi-centre, pan-European, prospective,
repetitive, observational cohort study enrolling patients with AATD (as confirmed by serum
level and genotyping). Patients will be managed according to their local procedures and
policies with no interference from the study team. Patients will give informed consent to
participate, and participating investigators will collect data prospectively. The following
domains are covered: demographics, proteinase inhibitor genotype and other laboratory
analyses, comorbidities, lung function, transient elastography of the liver, exacerbations,
quality of life (QoL), chest CT (if applicable), and treatment.
The study protocol was reviewed by the Research Ethics Committee of the Vall d'Hebron
University Hospital of Barcelona, Spain, and the study is sponsored by Vall d'Hebron Research
Institute, Barcelona, Spain. The study website will be located at www.AATD.eu. The registry
was developed according to recommendations on the design, implementation, governance and
long-term sustainability of disease registries in the European Union (EU). It is funded as a
clinical research collaboration (CRC) under the umbrella of the European Respiratory Society
(ERS). Clinical Research Collaborations (CRCs) are pan-European, multi-centre networks of
principal investigators aiming to advance science and clinical research within a specific
disease area (www.ersnet.org/ers-funding/clinical-research-collaboration.html). The European
Alpha-1 Clinical Research Collaboration (EARCO) was established as an ERS CRC with the
objective of developing a European AATD Registry and bringing researchers together
internationally to promote clinical research, clinical care and education.