Estimated Worldwide Prevalence of the PI*ZZ Alpha-1 Antitrypsin Genotype in Subjects With Chronic Obstructive Pulmonary Disease

• Ignacio Blanco ^[2] ; Isidro Diego ^[1] ; César Castañón ^[1] ; Patricia Bueno ^[3] ; Marc Miravitlles ^[4]
  1. [1] Universidad de Oviedo

     Universidad de Oviedo

     Oviedo, España

     
  2. [2] Alpha1-Antitrypsin Deficiency Spanish Registry, Spanish Society of Pneumology and Thoracic Surgery (SEPAR), Barcelona, Spain
  3. [3] Internal Medicine Dept, County Hospital of Jarrio, Jarrio, Principality of Asturias, Spain
  4. [4] Pneumology Dept, Hospital Universitari Vall d’Hebron/Vall d’Hebron Research Institute (VHIR), Barcelona, Catalonia, Spain

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• Localización: Archivos de bronconeumología: Organo oficial de la Sociedad Española de Neumología y Cirugía Torácica SEPAR y la Asociación Latinoamericana de Tórax ( ALAT ), ISSN 0300-2896, Vol. 59, Nº. 7, 2023, págs. 427-434
• Idioma: inglés
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  • Introduction The prevalence of α1-antitrypsin PI*ZZ genotypes in patients with COPD is only partially known. We aimed to estimate this prevalence worldwide.

    Method A systematic review of the literature was conducted for studies investigating the prevalence of COPD and the prevalence of severe alpha-1-antitrypsin deficiency (AATD) PI*ZZ genotype. Results are shown in tables and on a whole world interpolation map.

    Results Studies from 48 countries with available data (21 from Europe, 9 from the Americas, 5 from Africa, 11 from Asia and 2 from Australasia) were selected. About 235,000 individuals with PI*ZZ genotypes were accounted: 50% in Europe, 37% in America, 9% in Asia, 3% in Australasia and 1% in Africa. The estimated crude prevalence of COPD in adults older than 40 years was 12.45% in Europe, 13.51% in America, 13.22% in Africa, 11.70% in Asia and 11.86% in Australasia. The highest PI*ZZ weighted average prevalence among COPD subjects (expressed as 1/x [95% confidence intervals]) were found in Northern Europe (395 [252–576]) followed by Western (797 [538–1165]), Southern (944 [600–1475]) and Central Europe (1096 [687–1738]). Outside Europe, high values were found in Australia–New Zealand (1007 [684–1509]), Saudi Arabia (1276 [563–2961]), United States (1298 [1094–1540]), Canada (1482 [1057–2083]) and Thailand (1807 [717–4692]). In the rest of the world, prevalence was significantly lower, especially in vast regions of Asia and Africa where the PI*Z gene is practically non-existent.

    Conclusions Severe AATD is associated with a significant number of cases of COPD, especially in Europe, USA, Canada, New Zealand and Australia.