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Following the WHO recommendations in 1996, a joint American Thoracic Society (ATS) and European Respiratory Society (ERS) Task Force was established in 1998 to develop guidelines for the identification and management of patients with severe alpha1-antitrypsin (AAT) deficiency.1 The Task Force published its recommendations in 2003.
Clinical Recognition of AAT Deficiency
Physicians should consider the possibility of severe AAT deficiency in patients with:
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Early-onset emphysema (age 45 or less) |
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Emphysema in the absence of a recognized risk factor (smoking, occupational dust exposure, etc.) |
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Emphysema with prominent basilar hyperlucency |
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Otherwise unexplained liver disease |
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Necrotizing panniculitis |
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Anti-proteinase 3-positive vasculitis (C-ANCA [anti-neutrophil cytoplasmic antibody]—positive vasculitis) |
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Family history of any of the following: emphysema, bronchiectasis, liver disease, or panniculitis |
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Bronchiectasis without evident etiology |
The Task Force went on to recommend genetic testing for AAT deficiency with the following categorizations2:
Type A (Genetic testing is recommended)
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Symptomatic adults with emphysema, COPD, or asthma with airflow obstruction that is incompletely reversible after aggressive treatment with bronchodilators |
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Individuals with unexplained liver disease, including neonates, children and adults, particularly the elderly |
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Asymptomatic individuals with persistent obstruction on pulmonary function tests with identifiable risk factors (ie, cigarette smoking, occupational exposure) |
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Siblings of an individual with AAT deficiency |
Type B (Genetic testing should be discussed and could be reasonably accepted or declined)
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Adults with bronchiectasis without evident etiology |
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Adolescents with persistent airflow obstruction |
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Asymptomatic individuals with persistent airflow obstruction and no risk factors |
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Adults with C-ANCA-positive (anti-proteinase 3-positive) vasculitis |
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Individuals with a family history of COPD or liver disease not known to be attributed to AAT deficiency |
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Distant relatives of an individual who is homozygous for AAT deficiency |
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Offspring or parents of an individual with homozygous AAT deficiency |
References:
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ATS/ERS Task Force. Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency. AJRCCM. 2003; 168;820-900. |
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Gadek JE, Fulmer JD, Gelfand JA, et al. Danazol-induced augmentation of serum alpha1-antitrypsin levels in individuals with marked deficiency of this antiprotease. J Clin Invest. 1980;66:82-87. |
Important Safety Information
Prolastin®, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 PI (alpha1-antitrypsin deficiency) with clinically demonstrable panacinar emphysema. Weekly Prolastin® therapy has demonstrated a low occurrence of side effects. In clinical studies with Prolastin®, reactions were observed in 1.16% of infusions, the most common events being fever (0.77%), light-headedness (0.19%), and dizziness (0.19%). As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated. Individuals with selective IgA deficiencies who have known antibody against IgA (anti-IgA antibody) should not receive Prolastin®, since these patients may experience severe reactions, including anaphylaxis, to IgA which may be present.
You are encouraged to report negative side effects of prescription drugs
to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Please click here for Prolastin® full Prescribing Information.
Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor or healthcare provider if you have any questions about your health or any of your medications.
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