Fabrizio Mezzasalma

Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study

The natural history of idiopathic pulmonary fibrosis (IPF) is not well defined and its clinical course is variable. We sought to investigate the survival and incidence of acute exacerbations (AEs) and their significant predictors in newly diagnosed patients. 70 patients newly diagnosed with IPF were prospectively followed for at least 3 yrs. Baseline evaluation included Medical Research Council dyspnoea score (MRCDS), 6-min walk test, pulmonary function tests, all of which were repeated at 6 months, and high-resolution computed tomography. A retrospective cohort of 68 patients was used for confirmation. Mean survival from the time of diagnosis was 30 months, with a 3-yr mortality of 46%. A Risk stratificatiOn ScorE (ROSE) based on MRCDS >3, 6-min walking distance ≤72% predicted and composite physiologic index >41 predicted 3-yr mortality with high specificity. 6-month progression of ROSE predicted rapid progression. 3-yr incidence of AE was 18.6%, mostly occurring in the first 18 months; risk factors for AE were concomitant emphysema and low diffusing coefficient of the lung for carbon monoxide. Results were confirmed in an independent cohort of patients. In newly diagnosed IPF, advanced disease at presentation, rapid progression and AEs are the determinants of 3-yr survival. The purpose of the multifactorial ROSE is to risk-stratify patients in order to predict survival and detect rapid disease progression.

Pulmonary hypertension in idiopathic pulmonary fibrosis: prevalence and clinical progress.

The aims of the present study are to define the prevalence of Pulmonary Hypertension (PH) in a cohort of Idiopathic Pulmonary Fibrosis (IPF) patients, to investigate any correlations between systolic pulmonary artery pressure (PAPs) and functional data, to evaluate clinical progress and to compare long-term survival in IPF patients with and without PH. A population of 126 IPF patients was recruited. A high prevalence of PH (39.7%, 50/126), evaluated by echocardiography on the basis of PAPs > 36 mmHg, was mainly observed in smokers and female patients. Regression analysis revealed a significant correlation between PAPs > 50 mmHg and DLCO/VA (p=0.0294). Mean PAPs was significantly greater one year after onset of PH (p=0.01). 11/21 patients with FVC <50% had a significant increase in PAPs one year after onset of PH (p=0.02). There was a highly significant difference between survival of IPF patients with and without PH (p=0.0001; hazard ratio = 3.56). This study revealed that PH has a high prevalence in patients with IPF and is associated with increased risk of mortality. Early diagnosis of IPF patients with pulmonary hypertension is important, so that they can be enrolled in waiting lists for lung transplant as soon as possible.

Diagnostic yield of fluoroscopy-guided transbronchial biopsy in peripheral lung lesion: Adequacy of transbronchial sample for tumor genotyping

INTRODUCTION The fluoroscopy-guided transbronchial lung biopsy (TBLB) is an useful technique for diagnosis of peripheral lung lesion (PLL). The diagnostic yield of TBLB is limited and variable, ranging in literature from 16-80%. Nowadays the specimens collected must be suitable for tumor genotyping and the published data for this issue are not conclusive. Aim of the study was to evaluate the diagnostic yield of fluoroscopy guided TBLB and the adequacy of sample for molecular testing in adenocarcinoma subtype. METHODS The pts. referred to our Unit in the period 2013-2015 due to peripheral lung opacities with diameter ≥ 2 cm were included in the study. We excluded pts with uncontrolled coagulopathy, pregnancy, emodinamic instability. All the pts underwent bronchoscopy and fluoroscopy-guided TBLB and TB needle aspiration (TBNA). We performed 4-6 TBLB and 4-6 TBNA for each patient. In case of histopathologic diagnosis of adenocarcinoma, genotyping for EGFR, ALK, K-RAS was done. RESULTS 263 patients have been included, 164 males, age 68.8±11, range 33-88 yrs. TBNA and TBLB allow a diagnosis in 167 patients (diagnostic yield 68%)(table 1); the false negative were 48 (18.2%). The adenocarcinoma represented the 40% of malignant lesions: in this group the sample was adequate for genotyping examination in the majority of cases (> 90%). CONCLUSIONS The fluoroscopy-guided TBLB allowed a correct diagnosis in about 70% of cases with very few adverse events. The specimen resulted adequate for genotyping of adenocarcinoma in the majority of patients.