TY - JOUR T1 - Significance of abnormal autoantibodies in patients presenting with IPF JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - 2824 AU - Sam Hayward AU - David McAllister AU - Pauline Macfarlane AU - Phoebe Wright AU - Gareth Stewart AU - William Wallace AU - John Murchison AU - John Simpson AU - Nikhil Hirani Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/2824.abstract N2 - 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. ER -