RT Journal Article SR Electronic T1 Significance of abnormal autoantibodies in patients presenting with IPF JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 2824 VO 40 IS Suppl 56 A1 Sam Hayward A1 David McAllister A1 Pauline Macfarlane A1 Phoebe Wright A1 Gareth Stewart A1 William Wallace A1 John Murchison A1 John Simpson A1 Nikhil Hirani YR 2012 UL http://erj.ersjournals.com/content/40/Suppl_56/2824.abstract AB IntroductionA subset of patients with IPF present with abnormal auto-antibodies (AAs) without clinical features sufficient to diagnose connective tissue disease (CTD). CTD-associated ILD is generally associated with a better prognosis than IPF.AimsTo study the prevalence and significance of abnormal AAs in IPF.MethodsConsecutively presenting patients with suspected IPF between 1/1/02 and 31/12/10 were prospectively recruited to a database. All IPF diagnoses required exclusion of overt CTD and an HRCT appearance of UIP with ≥ 70% probability. Patients with HRCT scans with ≥ 95% probability of UIP, or a confirmatory surgical lung biopsy were defined as definite IPF. The remainder were defined as probable IPF. Abnormal AA profile was defined as the presence of at least one of: RhF ≥40, ANA ≥1/640 and/or positive specific ENA screen. Patients were followed-up until Dec 2011 (median [IQR] among survivors 45 [25-63] months). Of 233 patients recruited, 25 did not have AAs performed within 12 months of presentation and were excluded. Of the 208 patients reported, 95 had definite IPF.ResultsAAs were abnormal in 18% of patients. Definite v probable IPF, gender, age, smoking and baseline lung function were similar for normal and abnormal AA groups. Only 3 patients developed overt CTD and all had abnormal AAs. Median survival was lower in those with abnormal AAs (39 v 69 months; unadjusted HR 1.57 [0.97 to 2.53] p=0.07; adjusted for age, sex, baseline VC, smoking and definite/probable IPF, HR 1.69 [1.03 to 2.78] p=0.04).ConclusionsOnly 1% of all IPF patients developed overt CTD. Abnormal AA serology was associated with a poorer survival.