Long-term augmentation therapy with Alpha-1 Antitrypsin in an MZ-AAT severe persistent asthma

https://doi.org/10.4081/monaldi.2008.380

Authors

  • I. Blanco | ignacio.blanco@sespa.princast.es Unit of Respiratory Diseases, Valle del Nalón Hospital, Langreo, Principado de Asturias, Spain.
  • H. Canto Unit of Respiratory Diseases, Valle del Nalón Hospital, Langreo, Principado de Asturias, Spain.
  • J. Flóres Unit of Respiratory Diseases, Valle del Nalón Hospital, Langreo, Principado de Asturias, Spain.
  • C. Camblor Unit of Respiratory Diseases, Valle del Nalón Hospital, Langreo, Principado de Asturias, Spain.
  • V. Cárcaba Department of Internal Medicine, Valle del Nalón Hospital, Riaño-Langreo, Principado de Asturias, Spain.
  • F.J. de Serres Center for the Evaluation of Risks to Human Reproduction, Environmental Toxicology Program. National Institute of Environmental Health Sciences. Research Triangle Park, NC, United States.
  • S. Janciauskiene Department of Medicine. Malmö University Hospital, Malmö, Sweden.
  • E.F. Bustillo Biostatistics Unit, Central University Hospital of Asturias, Oviedo, Spain.

Abstract

A young Caucasian female with severe bronchial asthma and Alpha1-antitrypsin (AAT) deficiency, MZ phenotype, experienced a quick and severe limitation of her physical capacity, which negatively affected her psychological state and social life, though she was under a strong antiasthmatic treatment. Given her declining health status and the significant chronic corticoid administration- related side-effects (including high reduction of muscle mass and bone density), a clinical trial with commercial intravenous AAT was proposed by the patient’s doctors, and accepted by the Spanish Ministry of Health, although it this therapy was not approved for MZ phenotypes yet. This new therapy quickly stopped lung function decline rate, dramatically reduced the number of hospital admissions of the patient, suppressed the oral administration of prednisone, reversed the corticosteroid-related health adverse effects, significantly improving her quality of life. Thus, although AAT replacement therapy is not approved nor indicated for the treatment of bronchial asthma in MZ patients, its favourable effects observed in this isolated case support the hypothesis that bronchial asthma could be due to pathogenic mechanisms related to a protease- antiprotease imbalance, what which could open new perspectives for future research on the field.

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Published
2016-01-26
Info
Issue
Section
Case Reports
Keywords:
Bronchial asthma, Augmentation therapy with Alpha-1 antitrypsin, SerpinA1, Alpha-1 Antitrypsin phenotypes, Alpha-1 Antitrypsin deficiency augmentation therapy
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How to Cite
Blanco, I., H. Canto, J. Flóres, C. Camblor, V. Cárcaba, F.J. de Serres, S. Janciauskiene, and E.F. Bustillo. 2016. “Long-Term Augmentation Therapy With Alpha-1 Antitrypsin in an MZ-AAT Severe Persistent Asthma”. Monaldi Archives for Chest Disease 69 (4). https://doi.org/10.4081/monaldi.2008.380.