TY - JOUR T1 - Anti periplakin antibodies in connective tissue-associated lung fibrosis JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P5118 AU - Camille Taillé AU - Mathilde Neuville AU - Sabine Grootenboer-Mignot AU - Philippe Dieudé AU - Yannick Allanore AU - Sylvie Chollet-Martin AU - Bruno Crestani Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P5118.abstract N2 - Introduction: Auto antibodies directed against periplakin (PPL), a desmosomal component, have been detected in 40 % of patients with idiopathic pulmonary fibrosis, and are associated with a more pronounced impairment of pulmonary function tests. We assessed whether anti PPL Abs could be detected in lung fibrosis associated with connective tissue diseases and whether their detection is associated with severity of the respiratory disease.Material and methods: Anti PPL Abs were detected by immunoblot in the serum from 101 patients with rheumatoid arthritis (RA) and in 45 patients with systemic sclerosis (SSc).Results: Anti PPL Abs were detected in 17/101 RA patients (16.8%) and in 13/45 SSc patients (28.9%) (P=0.07). ILD was detected in 25 RA (24%) and 19 SSc patients (45%). In RA patients, anti PPL Abs rate was similar in patients with (20%) and without ILD (15%, p=0.5). In SSc patients, detection of anti PPL Abs tends to be more frequent in patients with ILD (42% versus 19%, p=0.11). RA patients with anti PPL Abs did not differ from those without Abs for age, sex, smoking habits, age at onset, anti CCP status, bone erosions. Anti PPL Abs were more frequent in non Caucasian patients (33% versus 60%, p=0.006). SSc patients with anti PPL Abs were not different for age, sex, smoking, anti SCL70, anti RNP or anti centromere antibodies, pulmonary hypertension or other visceral manifestations. Among patients with ILD, with RA or SSc, lung function tests results were similar in anti PPL (+) and PPL (-) patients,.Conclusion: Anti PPL Abs are frequently detected in patients with RA or SSc. Anti PPL Abs are not associated with the presence of ILD. ER -