RT Journal Article SR Electronic T1 Pulmonary rehabilitation (PR) in patients with COPD due to alpha-1 antitrypsin deficiency (AATD): 15 years experience at the Maugeri Institute JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP P3567 VO 42 IS Suppl 57 A1 Bruno Balbi A1 Michele Vitacca A1 Piero Ceriana A1 Mauro Carone A1 Gianni Balzano A1 Andrea Zanini A1 Claudio Fracchia A1 Pierachille Santus A1 Armando Capelli A1 Giancarlo Piaggi A1 Giovanna Callegari A1 Ilaria Ferrarotti A1 Maurizio Luisetti A1 Antonio Spanevello YR 2013 UL http://erj.ersjournals.com/content/42/Suppl_57/P3567.abstract AB Background. In Italy < 250 patients with respiratory disease, mainly COPD, due to AATD are in the National Registry. PR is considered the gold standard non pharmacological treatment for COPD. No data are available on COPD due to AATD.Methods. We examined electronic databases and clinical records of the last 15 years from the 8 PR centers of the Maugeri Foundation referring to patients with COPD due to AATD. Data were grouped for the first access to our Institution, the second and the last access (if present). Patients underwent PR programs as COPD patients without AATD.Results. From 1997 to 2012, 30 patients with severe AATD (i.e. homozygous or double heterozygous for AATD alleles) underwent one, 18 patients two and 14 patients multiple (up to 12) PR programs. At baseline there were 19 males and 11 females, 54±10 yrs, 82% current or former smokers, BMI 25±4. COPD was graded as very severe (GOLD 2007) in 50% of patients, and 47% had 2 or more comorbidities. Augmentation therapy with exogenous AAT was present in 59% of the subjects, liver disease in 23% of patients. The genotypes were ZZ in 40% of patients, SZ in 10%, rare in 26%, undetermined in 24%. After PR there was a tendency to amelioration of lung function test: +3±9 %FVC, +5±12%FEV1, +0.2±0.3 L FEV1 (p=0.04), -13±22%RV. 6mWD after PR improved > 33 m (+33 to +250m) in 38% of cases. Among the 14 patients who repeated PR programs over years, 80% were GOLD stage 4.Conclusion. PR may be applied to COPD due to AATD as shown by this cohort of middle aged but severely diseased patients. To maximize its effects specific PR protocol should be tailored to the characteristics of these patients.