RT Journal Article SR Electronic T1 Primary pulmonary interstitial glycogenosis. Long term outcome JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP P2343 VO 42 IS Suppl 57 A1 Olaia Sardon A1 Sandra Rovira A1 Paula Corcuera A1 Javier Korta A1 Joan Carles Ferreres A1 Elena Perez A1 Eduardo G. Perez-Yarza A1 Antonio Moreno YR 2013 UL http://erj.ersjournals.com/content/42/Suppl_57/P2343.abstract AB Background:Pulmonary interstitial glycogenosis (PIG) or infantile cellular interstitial pneumonia (ICIP) is a rare and poorly understood entity of the infant lung.Objectives:Describe clinical presentation and long term clinical outcome of infants diagnosed with primary PGI or ICIP.Methods:Multicentre prospective study of patients diagnosed by lung biopsy between October 2003 and December 2012.Results: Eight patients were included; 6 term born infants and 2 preterm infants (GA 36-32+2 weeks).Three infants were male. Four had respiratory distress and two required mechanical ventilation.Two infants had prolonged hypoxemia and the others were studied because of persistent tachypnea (median respiratory rate 57.5; range 45-70).Hospital admission because of acute bronchiolitis or wheezing bronchitis was required in 5/8 cases.CT-scan showed ground glass attenuation (n=8), parenchymal bands (n= 7) and septal thickening (n=8). Median age at diagnosis was 5 months (range 2-9).Patients were treated with intravenous methylprednisolone (7/8), hydroxychloroquine (4/8), and home oxygen (3/8; range 11-36 months). Median follow-up was 6.5 years (1-9 years). All patients enhanced clinically with resolution of tachypnea and hypoxemia.All infants had wheezing bronchitis until the median age of 3 years (range 1.5-8). Spirometry results (6/8) showed a median FVC of -1.76 z-score (range 0.07 to -2.85) and a median FEV1 of -2.27 z-score (range -0.54 to -4.37) suggesting a restrictive pattern (5/6) or a mixed pattern (1/6).Plethysmographic study confirmed a restrictive pattern in 4/5 patients.Conclusions:Although patients with ICIP/PIG showed a good long term clinical outcome they have significant functional, mainly restrictive, defects.