To study the reliability of pulmonary vascular measurements based on computed tomography (CT) in the prediction of pulmonary hypertension (PH) in patients with advanced interstitial lung disease (ILD) compared with those without ILD. The study was approved by the Institutional Review Board. All patients gave written informed consent. A prospective study of 134 patients who underwent right-sided heart catheterization and chest CT scanning within 72 hours of admission was conducted. Patients were divided into two groups-one with ILD (group A, n = 100) and one without ILD (group B, n = 34). CT measurements of the main pulmonary artery diameter (PAD), the ratio of PAD to the ascending aorta diameter (AAD), right pulmonary artery diameter (RPAD), and left pulmonary artery diameter (LPAD) were obtained. Univariate logistic regression analysis was performed, and receiver operating characteristic curves were constructed to assess the predictive ability of vascular measurements obtained by using CT in the identification of PH. Main PAD was significantly greater in patients with PH than in those without PH in both groups (group A, P = .008; group B, P = .02). A PAD greater than 25 mm in patients with ILD was predictive of PH, with a sensitivity of 86.4% (32 of 37), a specificity of 41.2% (26 of 63), a positive predictive value of 46.3% (32 of 69), and a negative predictive value of 83.8% (26 of 31). In patients without ILD, a PAD greater than 31.6 mm and an LPAD greater than 21.4 mm were predictive of PH (sensitivity, 47.3% [nine of 19]; specificity, 93.3% [14 of 15]; positive predictive value, 90.0% [nine of 10]; and negative predictive value, 58.3% [14 of 24]). CT-derived vascular measurements were of limited utility in the prediction of PH in patients with ILD compared with those without ILD.

Introduction: Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.Methods: ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival.Results: Data from 215 patients followed for a mean±sd2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (D_LCO) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV₁)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.Conclusion: ReducedD_LCO(<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV₁/FVC ratio was identified as an independent risk factor for worsened outcomes.

Background: There are relatively few epidemiological studies on interstitial lung disease (ILD) worldwide. Objective: To report the incident cases of ILD and compare our data with reports from other populations. Methods: Newly diagnosed ILDs were prospectively collected at a single tertiary care hospital from January 2008 to December 2011. Detailed demographic and clinical data were collected at the time of diagnosis, along with the results from diagnostic procedures, including high-resolution computed tomography (HRCT), serological tests, bronchoalveolar lavage (BAL), transbronchial lung biopsy, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and surgical lung biopsy. Results: A total of 330 cases were included. The mean age was 55.4 ± 14.9 years. There was a slight predominance of females (202; 61.2%), and the male-to-female ratio was 1:1.37. The most frequent disease was connective tissue disease (CTD)-associated ILD (34.8%), followed by idiopathic pulmonary fibrosis (IPF) (23.3%), sarcoidosis (20%), and hypersensitivity pneumonitis (6.3%). Non-classifiable ILD was present in 1.8% of the total ILD cases. HRCT was performed in 97.3% of the cases, BAL in 17.5%, transbronchial lung biopsy in 21.8%, EBUS-TBNA in 4.5%, and surgical lung biopsy in 22.7% (38.6% of which were performed among the idiopathic interstitial pneumonia cases). Conclusions: CTD-ILD and IPF were the most frequently observed ILDs in this Saudi Arabian population. Similarities and differences were found with respect to the previous reports from other countries.

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH. Methods: Consecutive ILD patients who underwent right heart catheterization (n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH (p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO₂) < 88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort. Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome.

Background : Pulmonary hypertension (PH) occurs in many patients with interstitial lung disease, including sarcoidosis. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis. Methods : A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography (CT) results were collected, and compared between patients with and without PH. Results : Twenty (20.8%) patients were found to have PH. Patients with PH were more likely to be symptomatic (cough, P = 0.008; dyspnea, P = 0.04), to have an advanced radiographic stage (P = 0.001), and to be receiving systemic therapy (P = 0.001), compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Comparison of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity (61.5 vs. 28.8%, P = 0.032) and fibrosis (76.9 vs. 44.2%, P = 0.035). In total, four patients died during the study period, including three with evidence of PH. Conclusions : The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis.