Predicting clinical outcome with phenotypic clusters using quantitative CT fibrosis and emphysema features in patients with idiopathic pulmonary fibrosis

BACKGROUND: The clinical course of IPF varies. This study sought to identify phenotyping with quantitative computed tomography (CT) fibrosis and emphysema features using a cluster analysis and to assess prognostic impact among identified clusters in patient with idiopathic pulmonary fibrosis (IPF). Furthermore, we evaluated the impact of fibrosis and emphysema on lung function with development of a descriptive formula. METHODS: This retrospective study included 205 patients with IPF. A texture-based automated system was used to quantify areas of normal, emphysema, ground-glass opacity, reticulation, consolidation, and honeycombing. Emphysema index was obtained by calculating the percentage of low attenuation area lower than -950HU. We used quantitative CT features and clinical features for clusters and assessed the association with prognosis. A formula was derived using fibrotic score and emphysema index on quantitative CT. RESULTS: Three clusters were identified in IPF patients using a quantitative CT score and clinical values. Prognosis was better in cluster1, with a low extent of fibrosis and emphysema with high forced vital capacity (FVC) than cluster2 and cluster3 with higher fibrotic score and emphysema (p = 0.046, and p = 0.026). In the developed formula [1.5670—fibrotic score(%)*0.04737—emphysema index*0.00304], a score greater ≥ 0 indicates coexisting of pulmonary fibrosis and emphysema at a significant extent despite of normal spirometric result. CONCLUSIONS: Cluster analysis identified distinct phenotypes, which predicted prognosis of clinical outcome. Formula using quantitative CT values is useful to assess extent of pulmonary fibrosis and emphysema with normal lung function in patients with IPF.