Systemic sclerosis associated interstitial lung disease: a survey of current practices in France.

Background: Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Objective: We performed an overview of the diagnostic approaches, follow-up and treatment strategies used in France for the management of SSc-associated ILD (SSc-ILD). Design Structured nationwide online surveyMethods: A structured nationwide online survey was submitted to participants via the French Medical Societies for Internal Medicine and Pneumology, and research groups on SSc-ILD from May 2018 to June 2020. The 79 multiple-choice and 9 open-ended questions covered the screening of ILD at baseline, monitoring of patients with established SSc-ILD and its management. Fourteen optional vignettes exploring different clinical phenotypes of SSc-ILD were submitted to evaluate therapeutic decisions. Results: All of the 93 participants screened SSc patients for ILD at baseline with 83 (89%) participants relying on a systematic chest computed tomography (CT) scan. Pulmonary function tests (PFT) were prescribed by 87 (94%) participants at baseline and during follow-up. Treatment was started based on abnormal PFT (95%), chest CT scan characteristics (89%), worsening dyspnoea (72%) and drop in SpO2 during 6-min walk tests (66%). First-line therapy was cyclophosphamide (CYC) (89%), mycophenolate mofetil (MMF) (83%) and prednisone (73%). Rituximab as second-line immunosuppressive therapy (41%) was preferred to antifibrotic agents (18%), and a median daily prednisone dose of 10 mg (interquartile range, 10-15) was prescribed by 73% participants. Extensive SSc-ILD with worsening PFT (95%), regardless of diffusing capacity for carbon monoxide values and skin extension, were more likely to be treated, and CYC was favoured over MMF (p < 0.01). Extensive SSc-ILD with disease duration of less than 5 years was also a criterium for treatment initiation. Conclusion: This overview of practices in diagnosis, follow-up and treatment of SSc-ILD in France describes real-life management of patients. It highlights heterogeneity in this management and gaps in current strategies that should be addressed to improve and harmonize clinical practices in SSc-ILD.

Cryoballoon ablation of atrial fibrillation in patients with atypical right pulmonary vein anatomy.

BACKGROUND: Cryoballoon ablation is widely used for pulmonary vein isolation in patients with atrial fibrillation. There are no data regarding the clinical efficacy of cryoballoon ablation in patients with atypical right pulmonary vein anatomy. AIM: We aimed to evaluate the impact of right pulmonary vein anatomy on the safety and efficacy of cryoballoon ablation. METHODS: Patients referred for cryoballoon ablation of paroxysmal atrial fibrillation were enrolled prospectively. Left atrial computed tomography was performed before cryoballoon ablation to determine whether the right pulmonary vein anatomy was "normal" or "atypical". For patients with atypical anatomy, cryoballoon ablation was only performed for right superior and right inferior pulmonary veins, neglecting accessory pulmonary veins. RESULTS: Overall, 303 patients were included: 254 (83.8%) with normal and 49 (16.2%) with atypical right pulmonary vein anatomy. First-freeze isolation for right superior and right inferior pulmonary veins occurred in 44 (89.8%) and 37 (75.5%) patients with atypical pulmonary vein anatomy, and in 218 (85.8%) and 217 (85.4%) patients with typical pulmonary vein anatomy, respectively (P not significant). Phrenic nerve palsies were only observed in patients with normal anatomy (0 vs. 26 [8.6%]; P=0.039). Mid-term survival free from atrial arrhythmia was similar, regardless of right pulmonary vein anatomy. CONCLUSIONS: A significant proportion of patients have atypical right pulmonary vein anatomy. Procedural characteristics, acute pulmonary vein isolation success and mid-term procedural efficacy were similar, regardless of right pulmonary vein anatomy. In addition to left-side pulmonary vein isolation, cryoballoon ablation of right superior and right inferior pulmonary veins only, neglecting accessory pulmonary veins, is sufficient to obtain acute right-side pulmonary vein isolation and mid-term sinus rhythm maintenance in patients with atypical anatomy.

Association of silica exposure with chest HRCT and clinical characteristics in systemic sclerosis.

CONTEXT: Thoracic lymphadenopathy (LA) has been identified as a key prognostic factor in interstitial lung disease (ILD) of all-cause. Crystalline silica is a risk factor of systemic sclerosis (SSc). The association of a history of crystalline silica exposure with chest high-resolution computed tomography (HRCT) features and thoracic LA are still to be determined in SSc patients. OBJECTIVES: We performed an observational study to assess the association of lifetime exposure to silica, with chest HRCT characteristics in a population of SSc patients fulfilling the 2013 ACR/EULAR classification criteria for SSc. METHODS: A specific questionnaire based on a multidisciplinary approach was used to assess occupational and non-occupational exposure to silica in 100 consecutive SSc patients. Clinical characteristics and chest HRCT at diagnosis and at the latest visit were evaluated to assess the association of silica exposure with disease characteristics. RESULTS: 16% of the overall population and 58% of men had an occupation with specific high silica exposure. A higher silica exposure score was associated with the combination of mediastinal and hilar LA on HRCT (OR=8.09, 95%CI=2.01-32.52, P = 0.002). More than 12% of the patients had a combination of mediastinal and hilar LA on HRCT. This marker of silica exposure was predictive of worsening of pulmonary involvement in univariate analysis (OR=5.86, 95%CI=1.64-20.89, P = 0.007) and multivariate analysis (OR=4.57, 95%CI =1.12-18.60, P = 0.034). CONCLUSIONS: In patients with SSc, the combination of mediastinal and hilar LA on HRCT was associated with exposure to silica and was also significantly associated with a more severe evolution of ILD.

The challenging right inferior pulmonary vein: A systematic approach for successful cryoballoon ablation.

BACKGROUND: Pulmonary vein isolation (PVI) using cryoballoon ablation is widely used for rhythm control in patients with paroxysmal atrial fibrillation. This technique has a steep learning curve, and PVI can be achieved quickly in most patients. However, the right inferior pulmonary vein (RIPV) is often challenging to occlude and isolate. AIM: We aimed to analyse the efficacy of RIPV ablation using a systematic approach. METHODS: Consecutive patients referred for cryoballoon ablation of paroxysmal atrial fibrillation were enrolled prospectively. A systematic approach was used for RIPV cryoablation. The primary endpoint was acute RIPV isolation during initial freeze. RESULTS: A total of 214 patients were included. RIPV isolation during initial freeze occurred in 179 patients (82.2%). Real-time PVI could be observed in 72 patients (33.6%), whereas cryoballoon stability required pushing the Achieve™ catheter inside the RIPVs in the remaining patients. The rate of unsuccessful or aborted first freeze as a result of insufficient minimal temperature was significantly higher in patients with real-time pulmonary vein potential recording (16.7% vs. 6.3%; P=0.031). To overcome this issue and obtain both stability and real-time PVI, a dedicated "whip technique" was developed. Twelve patients (5.6%) required a redo ablation; only two of these had a reconnected RIPV. CONCLUSIONS: A systematic approach to RIPV cryoablation can lead to a high rate of first freeze application. Operators should not struggle to visualize pulmonary vein potentials before ablation, as this may decrease cryoapplication efficacy. Thus, stability should be preferred over real-time PVI for RIPV ablation. Both stability and real-time PVI can be obtained using a "whip technique".

Changes in brain perfusion in successive arterial spin labeling MRI scans in neonates with hypoxic-ischemic encephalopathy.

The primary objective of this study was to evaluate changes in cerebral blood flow (CBF) using arterial spin labeling MRI between day 4 of life (DOL4) and day 11 of life (DOL11) in neonates with hypoxic-ischemic encephalopathy (HIE) treated with hypothermia. The secondary objectives were to compare CBF values between the different regions of interest (ROIs) and between infants with ischemic lesions on MRI and infants with normal MRI findings. We prospectively included all consecutive neonates with HIE admitted to the neonatal intensive care unit of our institution who were eligible for therapeutic hypothermia. Each neonate systematically underwent two MRI examinations as close as possible to day 4 (early MRI) and day 11 (late MRI) of life. A custom processing pipeline of morphological and perfusion imaging data adapted to neonates was developed to perform automated ROI analysis. Twenty-eight neonates were included in the study between April 2015 and December 2017. There were 16 boys and 12 girls. Statistical analysis was finally performed on 37 MRIs, 17 early MRIs and 20 late MRIs. Eleven neonates had both early and late MRIs of good quality available. Eight out of 17 neonates (47%) had an abnormal on late MRI as performed and 7/20 neonates (35%) had an abnormal late MRI. CBF values in the basal ganglia and thalami (BGT) and temporal lobes were significantly higher on DOL4 than on DOL11. There were no significant differences between DOL4 and DOL11 for the other ROIs. CBF values were significantly higher in the BGT vs. the cortical GM, on both DOL4 and DOL11. On DOL4, the CBF was significantly higher in the cortical GM, the BGT, and the frontal and parietal lobes in subjects with an abnormal MRI compared to those with a normal MRI. On DOL11, CBF values in each ROI were not significantly different between the normal MRI group and the abnormal MRI group, except for the temporal lobes. This article proposes an innovative processing pipeline for morphological and ASL data suited to neonates that enable automated segmentation to obtain CBF values over ROIs. We evaluate CBF on two successive scans within the first 15 days of life in the same subjects. ASL imaging in asphyxiated neonates seems more relevant when used relatively early, in the first days of life. The correlation of intra-subject changes in cerebral perfusion between early and late MRI with neurodevelopmental outcome warrants investigation in a larger cohort, to determine whether the CBF pattern change can provide prognostic information beyond that provided by visible structural abnormalities on conventional MRI.