TY - JOUR T1 - Immune and inflammatory responses in induced sputum (IS) in Anderson Fabry disease (AFD), COPD and healthy volunteers JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - p3885 AU - Nadia Shafi AU - Derralynn Hughes AU - Atul Mehta AU - Colette Smith AU - Marc Lipman Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/p3885.abstract N2 - AFD is an X-linked lysosomal storage disorder caused by mutations of the GLA gene. Reduced enzyme results in accumulation of storage material, leading to multi-organ pathology including airflow obstruction. Given the association between intra-cellular lipid and disease, we hypothesised subjects with pulmonary AFD would have detectable changes in airway immune/inflammatory cell profiles.Methods: AFD and COPD subjects matched for GOLD stage plus healthy age/sex matched volunteers underwent lung function testing and sputum induction.IS cells were stained with combinations of CD3, CD4, CD8, CD16 & CD56 antibodies and examined using flow cytometry for T cell populations including NKT cells, thought to be relevant to AFD immuno-pathology.Results: 46% of AFD patients had evidence of airflow obstruction (AO+).The% of total T cells in IS in AFD AO+ was greater than AFD AO- (p<0.01), and similar to COPD patients. AFD AO- had lower T cell% than seen in COPD (p<0.01). Other T cell populations including NKT cells were similar between groups.View this table:T cell populations from ISConclusion: Sputum T cell profiles in AFD and airways obstruction are similar to those of COPD whilst AFD AO- are more like healthy volunteers. This suggests a role for T cells in AFD lung disease. ER -