Management of Patients With Alpha-1 Antitrypsin Deficiency Associated Emphysema

Management of Patients With Alpha-1 Antitrypsin Deficiency Associated Emphysema. A National Registry.

The aim of this study is to describe the natural history of patients with alpha-1 antitrypsin associated emphysema and to figure out associated prognostic factors.

All patients who suffer from this disease are followed every 6 months during a 5 years period. FEV1 decline is the primary endpoint. Quality of life assessed using the Saint George's Respiratory Questionary is recorded too.

Inclusion Criteria:

• Serum alpha one antitrypsin < 0.8 g/l
• Emphysema diagnosed by CT scan
• Adult
• Obstructive disorder

Exclusion Criteria:

• Liver or lung transplantation