Clinical Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension.

BACKGROUND AND OBJECTIVE: Pulmonary hypertension (PH) is a known complication of pulmonary sarcoidosis and its aetiology is unclear. Different pathophysiological mechanisms in sarcoidosis-associated pulmonary hypertension (SAPH) are known. Clinical phenotyping can aid clinicians in choosing the optimal treatment strategy. This study aimed to describe clinical phenotypes of SAPH and their characteristics. METHODS: A retrospective cohort study was performed on all SAPH patients at a tertiary referral centre. All patients were extensively analysed and discussed case by case in a multidisciplinary expert team to determine the most likely pathophysiological mechanism of PH. Patients were then classified into conceptual clinical phenotypes. RESULTS: Forty (40) patients with SAPH were identified between 2010 and 2019. Three (3) patients were classified as the postcapillary phenotype. Of the remaining 37 patients with precapillary PH, six were classified as 'compression of pulmonary vasculature', 29 as 'parenchymal', one as 'suspected vasculopathy', and one as 'chronic pulmonary emboli' phenotypes. Of the patients with compression of pulmonary vasculature, four showed compression by fibrotic disease and two by active sarcoidosis-based disease. Within the parenchymal phenotype, 20 patients (69%) showed pulmonary vascular resistance >3.0 Wood Units (WU) and had significantly lower diffusing capacity of the lung for carbon monoxide compared with the nine patients (31%) with pulmonary vascular resistance ≤3.0 WU. CONCLUSION: SAPH had multiple pathophysiological mechanisms and clinical phenotypes in this retrospective study. Further studies are necessary to examine how these phenotypes can affect appropriate treatment and prognosis.

Video capsule endoscopy in patients with nonresponsive celiac disease.

GOALS AND BACKGROUND: Discriminating between patients with nonresponsive but otherwise uncomplicated celiac disease (CD) and patients with refractory celiac disease (RCD) and/or lymphoma is difficult, especially as many abnormalities encountered in complicated CD are not within reach of conventional gastroduodenoscopy. We aimed to describe video capsule endoscopy (VCE) findings in patients with CD and persisting or relapsing symptoms despite a gluten-free diet and to identify VCE findings associated with poor prognosis. METHODS: We retrospectively analyzed 48 VCE studies performed in adult patients with CD because of persisting or relapsing symptoms despite adherence to a gluten-free diet. Patients with either uncomplicated CD or RCD type I were considered to have a good prognosis, whereas patients with either RCD type II or enteropathy-associated T-cell lymphoma were considered to have a poor prognosis. Multivariate analysis was performed to identify VCE findings independently associated with either good or poor prognosis. RESULTS: Proximal focal erythema (odds ratio, 6.7; 95% confidence interval, 1.2-38.7; P=0.033) and absence of progression of the capsule to the distal intestine (odds ratio, 16.5; 95% confidence interval, 1.2-224.9; P=0.035) were independently associated with poor prognosis. Of the 28 patients with none of these 2 features, none died during follow-up, compared with 2 (13.3%) of the 15 patients with one of both features, and 4 (80.0%) of the 5 patients with both the features. CONCLUSIONS: VCE is a minimally invasive endoscopic modality that could be of use in identifying patients with nonresponsive CD who are at risk of poor prognosis.

Optimization of Preoperative Lymph Node Staging in Patients with Muscle-Invasive Bladder Cancer Using Radiomics on Computed Tomography.

Approximately 25% of the patients with muscle-invasive bladder cancer (MIBC) who are clinically node negative have occult lymph node metastases at radical cystectomy (RC) and pelvic lymph node dissection. The aim of this study was to evaluate preoperative CT-based radiomics to differentiate between pN+ and pN0 disease in patients with clinical stage cT2-T4aN0-N1M0 MIBC. Patients with cT2-T4aN0-N1M0 MIBC, of whom preoperative CT scans and pathology reports were available, were included from the prospective, multicenter CirGuidance trial. After manual segmentation of the lymph nodes, 564 radiomics features were extracted. A combination of different machine-learning methods was used to develop various decision models to differentiate between patients with pN+ and pN0 disease. A total of 209 patients (159 pN0; 50 pN+) were included, with a total of 3153 segmented lymph nodes. None of the individual radiomics features showed significant differences between pN+ and pN0 disease, and none of the radiomics models performed substantially better than random guessing. Hence, CT-based radiomics does not contribute to differentiation between pN+ and pN0 disease in patients with cT2-T4aN0-N1M0 MIBC.