PT - JOURNAL ARTICLE AU - David McAllister AU - Pauline Macfarlane AU - Gareth Stewart AU - Andrew Robson AU - William Wallace AU - John Murchison AU - John Simpson AU - Nik Hirani TI - Disease progression according to IPF phenotype DP - 2012 Sep 01 TA - European Respiratory Journal PG - P3634 VI - 40 IP - Suppl 56 4099 - http://erj.ersjournals.com/content/40/Suppl_56/P3634.short 4100 - http://erj.ersjournals.com/content/40/Suppl_56/P3634.full SO - Eur Respir J2012 Sep 01; 40 AB - IntroductionOur understanding of disease progression in IPF is based on cohorts of patients with 'definite' IPF. Such studies have reported a prognostic index for IPF (Du Bois 2011 AJRCCM 184; 459). In practice a diagnosis of definite IPF is not always attained; in some cases where the HRCT pattern is not incontrovertibly UIP, corroboratory invasive investigations are not performed. We define this condition 'probable IPF'.AimsTo determine 1) disease progression and 2) the prognostic value of an IPF index in definite or probable IPF.MethodsConsecutively presenting patients with IPF were prospectively recruited to a database. All IPF diagnoses required an HRCT appearance of UIP with ≥ 70% probability. Patients with HRCT scans with ≥ 95% probability of UIP, or a UIP biopsy were defined as definite IPF. BAL was not included in the diagnostic criteria.ResultsOf 193 patients, 89 had definite IPF. Median survival for definite v probable IPF was 3.2 v 6.0 years respectively (HR 1.45; 95%CI 0.94 to 2.25, p=0.09 adjusting for age, sex, height, VC and smoking). A relative decline in VC of 5-10% in the first 6 months was associated with increased risk of death (HR 3.12; 95% CI 1.55 to 6.30). Similar results were obtained in patients with definite and probable IPF. An IPF index was calculated in our cohort. Scores of 0-8 (n=97), 10-29 (n=76) and 30-61 (n=20) predicted median survival of 74, 48 (HR 2.02; 95%CI 1.26 to 3.23) and 12 months (HR 15.3; 95%CI 8.22 to 28.4) respectively (p < 0.001). The index had similar predictive value in definite and probable IPF.ConclusionsPatients with both definite and probable IPF have poor prognoses but survival was worse in definite IPF. The IPF index may be of clinical value in definite and probable IPF.