Using chest computed tomography and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis.

Objectives: Lumacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). The aim of this study was to assess lung structural changes after 1 year of lumacaftor-ivacaftor treatment and to use unsupervised machine learning to identify morphological phenotypes of lung disease that are associated with response to lumacaftor-ivacaftor.

Methods: Adolescents and adults with CF from a French multicentre real-world prospective observational study evaluating the first year of treatment with lumacaftor-ivacaftor were included if they had pre-therapeutic and follow-up chest computed tomography (CT) scans available.

Systemic Pulmonary Events Associated with Myelodysplastic Syndromes: A Retrospective Multicentre Study.

Although pulmonary events are considered to be frequently associated with malignant haemopathies, they have been sparsely studied in the specific context of myelodysplastic syndromes (MDS). We aimed to describe their different types, their relative proportions and their relative effects on overall survival (OS). We conducted a multicentre retrospective cohort study.

Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients.

Objective: The aim of this study was to determine the frequency and characteristics of antisynthetase syndrome (ASS) revealed by polyarthritis.

Methods: First we conducted a retrospective single-centre study to assess the frequency of ASS patients who presented with polyarthritis without pulmonary and/or muscle symptoms. Secondly, we conducted a larger, multicentre study in order to describe the clinical characteristics of these patients.

Six-minute stepper test to assess effort intolerance in interstitial lung diseases.

The six-minute stepper test (6MST) is a new test for evaluating exercise tolerance. Unlike the six-minute walk test (6MWT) it can be carried out in a limited space. The aim of this study was to compare the 6MST and the 6MWT in patients with various diffuse interstitial lung disease (ILD).

[Effects of thromboendarterectomy for post-embolic pulmonary hypertension on physiological dead space on exercise].

The effect of therapy on the modification of a functional parameter is an elegant method for assessing the causal relationship between functional abnormalities and symptoms. We report an analysis of the effects of thromboendarterectomy for post-embolic pulmonary arterial hypertension on exercise functional parameters. A patient (62 years) had post-embolic pulmonary hypertension documented by pulmonary angiography and right heart catheterization (PAP mean: 48 mmHg).