Spanish Clinical Practice Guidelines for the Diagnosis and Management of Alpha-1 Antitrypsin Deficiency…

GuidelineArchivos de bronconeumologia· Jun 2026

Spanish Clinical Practice Guidelines for the Diagnosis and Management of Alpha-1 Antitrypsin Deficiency: 2026 Update.

Myriam Calle Rubio, José Luis López-Campos, Francisco Casas Maldonado … Marc Miravitlles

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Pulmonology & Critical CareGuidelineDiagnosisQ2IF 2.0

AAT levels below 116mg/dL trigger a two-step diagnostic approach for deficiency.

Spanish Clinical Practice Guidelines for the Diagnosis and Management of Alpha-1 Antitrypsin Deficiency: 2026 Update.

Myriam Calle Rubio … Marc Miravitlles

Archivos de bronconeumologia · Jun 2026

Background

AATD remains substantially underdiagnosed and is often diagnosed late; consequently, many individuals with severe deficiency do not benefit from disease-specific counseling or tailored care modifications.

Methods

A systematic review was conducted using the GRADE and ADOLOPMENT frameworks, with an updated literature review that, together with expert opinion, served as the basis for formulating recommendations.

116mg/dL

Results

116mg/dL

lower alpha-1 antitrypsin protein levels than this trigger a two-step diagnosis

More results

Alpha-1 antitrypsin deficiency (AATD) is a genetic condition characterized by reduced blood levels of alpha-1 antitrypsin (AAT), which confers an increased risk of developing several disorders throughout life, mainly pulmonary emphysema and liver involvement.

More results

Since 2015, when the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) published an updated document on the detection and treatment of patients with chronic obstructive pulmonary disease (chronic obstructive pulmonary disease) associated with AATD, new diagnostic procedures and studies on augmentation therapy have been developed.

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Conclusion

The panel recommends augmentation therapy for patients with documented severe AATD, with a genotype associated with severe deficiency and AAT levels <57.2mg/dL (<11μmol/L), and with emphysema documented on computed tomography and impaired pulmonary function test results, who are receiving optimal pharmacologic and nonpharmacologic treatment and are not active smokers.

Spanish Clinical Practice Guidelines for the Diagnosis and Management of Alpha-1 Antitrypsin Deficiency: 2026 Update.

Spanish Clinical Practice Guidelines for the Diagnosis and Management of Alpha-1 Antitrypsin Deficiency: 2026 Update.

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