AATD diagnosis and treatment

Diagnóstico y tratamiento del déficit del DAAT

On Monday, February 14, GSK organized the Konéctate event.

Pulmonology professionals from all over Spain were able to spread their knowledge in a clinical session that took place in the San Cecilio University Hospital, HUSC.

In this event, Phd. Francisco Casas, a specialist pulmonologist, gave a presentation on AATD.

Furthermore, Pneumology specialists from another 38 hospitals throughout Spain participated.

What is Alpha 1 Antitripsine?

Alpha-1 antitrypsin (AAT) is a glycoprotein of the serpin family secreted mainly by the liver.

Therefore, it is the most abundant protease inhibitor in human serum and its specific target is neutrophil elastase, a voracious protease capable of digesting elastin, basement membranes and other components of the extracellular matrix.

In addition, it has antioxidant, anti-inflammatory, antimicrobial and immunomodulatory properties that have currently gained great relevance due to its possible application in the treatment of SARS-CoV-2 coronavirus infection (COVID-19).

The rare disease Alpha 1 Antitrypsin

Alpha-1 antitrypsin deficiency (DAAT) is a congenital disease that causes low serum levels of AAT and predisposes to the development of chronic obstructive pulmonary disease (COPD), pulmonary emphysema, and in some cases also liver disease.

So, it is a very underdiagnosed disease since less than 10% of patients with severe alpha-1 antitrypsin deficiency (DAAT) are diagnosed.

Besides, until the patient is diagnosed with AATD, an average of 7-10 years usually elapse when they arrive at a consultation with an average of 5 doctors without ever determining the concentration of AAT in their blood.

For this reason, the World Health Organization (WHO) and the main Scientific Societies of Pulmonology recommend requesting its determination to all patients with COPD once in their lives.

Diagnosis and treatment of DAAT deficiency

We currently have diagnostic tools that have been incorporated into a novel diagnostic algorithm that we present.

In conclusion, patients with severe AATD and COPD must be treated according to the Clinical Practice Guidelines.

In addition, we must assess the criteria for starting treatment with intravenous AAT, recommending its control and follow-up in Units or Centers of Excellence in Alpha-1.

Centro Andaluz Alfa 1

The Andalusian Center Alfa-1 of Granada is structured as a Multidisciplinary Unit in which Digestive (Hepatology Unit), Paediatrics, Rehabilitation and Nursing (Medical Day Hospital) participate, it is accredited by the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR ) with the level of “Excellence” and cares for about 50 patients with AATD.

To resume, In this session, a brief historical review of DAAT has been carried out with the most representative milestones of the last 20 years.

The new diagnostic algorithm has been presented, the treatment has been updated and the therapeutic perspectives under development have been reviewed.

MODERATOR: Dr. Manuel Gallardo Medina. Head of the Pneumology Clinical Management Unit of the San Cecilio Clinical University Hospital in Granada.

SPEAKER: Dr. Francisco Casas Maldonado. Coordinator of the Andalusian Center Alfa-1 of Granada.

Centro Andaluz Alfa-1