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Augmentation Therapy in Alpha-1 Antitrypsin Deficiency

A deficiency of the circulating serum glycoprotein alpha-1 antitrypsin (α 1-AT) is the most common identified genetic cause of chronic obstructive pulmonary disease (COPD) and is also a risk factor for liver disease in children and adults. Prevalence estimates suggest there are about 100,000 severely deficient adults in the US, and a similar number in Europe, yet only about 6% of these individuals have been identified. Diagnosis requires only a simple blood test. One reason to identify individuals at risk of lung disease due to α 1-AT deficiency is that specific therapy is available. This therapy, referred to as augmentation therapy, is a human plasma fractionation product highly enriched for α 1-AT protein and is available from a number of companies in the US and Europe. Generically, these products are all labeled: alpha1-proteinase inhibitor, human. In the latter half of the 1980s, the US Food and Drug Administration approved the first of these augmentation therapies based on its biochemical efficacy, that is, its ability to raise blood and bronchoalveolar epithelial lining fluid levels of α 1-AT. Traditional efficacy trials were not feasible prior to marketing approval in large part due of the extremely small number of patients with α 1-AT deficiency identified at that time. Since the introduction of the first augmentation therapy product, several studies have tried to assess the effectiveness of augmentation therapy at slowing the progression of lung destruction in α 1-AT deficiency. While the results of each of these studies has been encouraging, none have provided definitive proof of effectiveness and each clinician and payor seems to have a dif-