[The concept of interstitial pneumonia with autoimmune features (IPAF)]. / Le concept de pneumopathie interstitielle diffuse avec manifestations auto-immunes (IPAF).

Interstitial lung diseases (ILD) are a heterogeneous group of respiratory diseases often related to connective tissue diseases. Some patients will develop an ILD with autoimmune features without reaching the recommended criteria for autoimmune diseases. Their management is difficult because they have both features for idiopathic and connective tissue disease. To better identify these patients, the concept of interstitial pneumonia with autoimmune features (IPAF) has been created. The diagnosis relies on ILD without identified cause and the presence of at least one defined criterion among 2 of the 3 following domains: clinic, serologic, and morphologic. The mean age at diagnosis is 60, a sex ratio of 1/1, and depending on the authors close to 20% of patients with IPAF will develop a connective tissue disease according to the international criterion. Their prognosis is better than for patients with idiopathic ILD and with an average 5-year survival of 70%. Older age at diagnosis, a pattern of usual interstitial pneumonia, and an impaired diffusing capacity for carbon monoxide have been identified as poor prognosis factors. The treatment relies on usual care for chronic respiratory diseases and is often associated with immunosuppressive and/or antifibrotic therapies. The objective of this classification is to better characterize these patients and improve their management.

[IgG4-related disease: A proteiform pathology with frequent chest manifestations]. / Maladie associée aux IgG4 : une maladie protéiforme aux manifestations thoraciques fréquentes.

INTRODUCTION: While IgG4-related disease (IgG4-RD) was initially described in the early 2000s, its polymorphic clinical manifestations were previously reported under different names ; they have in common the presence of IgG4+ oligoclonal plasma cells and fibrosis. STATE OF THE ART: Ruling out certain differential diagnoses, the diagnosis of IgG4-RD is based on a bundle of clinical, biological and histological features. Chest involvement is variable and can affect the mediastinum, bronchi, parenchyma, pleura and/or, more rarely, bones and (pericardium, aorta, coronary…) vascular structures. The most frequent radiological manifestations are peribronchovascular thickening, mediastinal lymphadenopathy, and nodular or interstitial patterns. Pleural involvement and posterior mediastinal fibrosis are less frequent, while thoracic paravertebral tissue thickening is highly specific. Systemic corticosteroids are the cornerstone of treatment. In case of relapse or as frontline therapy in case of risk factors for relapse and/or poor tolerance of corticosteroids), a steroid-sparing agent (most often rituximab) is added, and biannual maintenance infusions are associated with a lower risk of relapse. PERSPECTIVES: An international consensus has recently led to the development of classification criteria that should standardize the diagnostic approach and homogenize the enrolment of patients in epidemiological as well as therapeutic studies. Other treatments are also under evaluation, including biologics targeting T2 inflammation, CD-19 (inebilizumab, obexelimab), SLAMF7 (elotuzumab) surface proteins, Bruton's tyrosine kinase, and the JAK/STAT pathway. CONCLUSIONS: Substantial progress has been made over recent years in understanding IgG4-RD pathophysiology, and personalized patient care seems to be an achievable medium-term goal.

Stereotactic body radiation therapy (SBRT) as salvage treatment for early stage lung cancer with interstitial lung disease (ILD): An observational and exploratory case series of non-asian patients.

Interstitial lung disease (ILD) can coexist with early-stage lung cancer (LC) and may compromise surgery and worsen patients' outcomes. Stereotactic body radiation therapy (SBRT) is the gold standard treatment for medically inoperable early-stage lung cancer, but radiation therapy is contra-indicated for patients with ILD because of the higher risk of severe radiation-induced pneumonitis. SBRT may spare healthy lung tissue, but data are scarce in this rare population. Our exploratory case series aimed to retrospectively identify patients treated with SBRT in this setting: 19 patients were diagnosed with early-stage LC-ILD over the past 6 years and 9 received SBRT. Most of them were smokers with a median age of 71, 4 had no pathological documentation. After SBRT, 5 patients had grade I-II respiratory adverse events (AEs), but none had treatment-related grade III-IV respiratory AEs. Two patients died within 6 months of SBRT, and for both, death was related to metastatic relapse. In this case series, the radiological evolution of ILD before radiotherapy and the evolution of the radiotherapy scar on CT-Scan were also explored with different evolutionary models. This exploratory study shows available data that could be studied in a larger retrospective cohort to identify risk factors for SBRT in the LC-ILD population. The use of dosimetric data as a risk factor for SBRT should be done with cautiousness due to heterogeneous and complex dose delivery and different fractionation schedule.

[Computed tomography lung density and small vessel quantification in pulmonary hypertension associated with progressive fibrosing interstitial lung disease (PF-ILD)]. / Densité pulmonaire et quantification vasculaire tomodensitométrique dans l'hypertension pulmonaire associée aux pneumopathies interstitielles diffuses fibrosantes.

INTRODUCTION: The main objective of this work was to investigate a possible link between lung density, small pulmonary vessels, and pulmonary hypertension (PH) in patients with progressive fibrosing interstitial lung disease (PF-ILD). METHODS: The study focused on patients with PF-ILD, all of whom underwent right cardiac catheterization and chest computed tomography prior to lung transplantation. Computed tomography scans were analyzed quantitatively for density and pulmonary vascularity. The relationship between computed tomography features and PH was investigated. RESULTS: Fifty-one patients with usual interstitial pneumonia (UIP) damage on lung explant were included. mPAP was positively correlated with lung mass (r=0.36, P=0.03) and lung volume (r=0.43, P=0.007). Patients with severe PH had more voxels lower than -856 Hounsfield Units (HU) (+16%, P=0.02), fewer voxels greater than -700 HU (-20%, P=0.03), and a higher lung volume (+1.57L, P=0.007) compared to patients without PH. No correlation was found between vascularization and HTP. CONCLUSIONS: Patients with PF-ILD and severe PH have lower lung density than patients with moderate or without PH.

18F-FDG brain PET hypometabolism in patients with long COVID.

PURPOSE: In the context of the worldwide outbreak of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), some patients report functional complaints after apparent recovery from COVID-19. This clinical presentation has been referred as "long COVID." We here present a retrospective analysis of 18F-FDG brain PET of long COVID patients from the same center with a biologically confirmed diagnosis of SARS-CoV-2 infection and persistent functional complaints at least 3 weeks after the initial infection. METHODS: PET scans of 35 patients with long COVID were compared using whole-brain voxel-based analysis to a local database of 44 healthy subjects controlled for age and sex to characterize cerebral hypometabolism. The individual relevance of this metabolic profile was evaluated to classify patients and healthy subjects. Finally, the PET abnormalities were exploratory compared with the patients' characteristics and functional complaints. RESULTS: In comparison to healthy subjects, patients with long COVID exhibited bilateral hypometabolism in the bilateral rectal/orbital gyrus, including the olfactory gyrus; the right temporal lobe, including the amygdala and the hippocampus, extending to the right thalamus; the bilateral pons/medulla brainstem; the bilateral cerebellum (p-voxel < 0.001 uncorrected, p-cluster < 0.05 FWE-corrected). These metabolic clusters were highly discriminant to distinguish patients and healthy subjects (100% correct classification). These clusters of hypometabolism were significantly associated with more numerous functional complaints (brainstem and cerebellar clusters), and all associated with the occurrence of certain symptoms (hyposmia/anosmia, memory/cognitive impairment, pain and insomnia) (p < 0.05). In a more preliminary analysis, the metabolism of the frontal cluster which included the olfactory gyrus was worse in the 7 patients treated by ACE drugs for high blood pressure (p = 0.032), and better in the 3 patients that had used nasal decongestant spray at the infectious stage (p < 0.001). CONCLUSION: This study demonstrates a profile of brain PET hypometabolism in long COVID patients with biologically confirmed SARS-CoV-2 and persistent functional complaints more than 3 weeks after the initial infection symptoms, involving the olfactory gyrus and connected limbic/paralimbic regions, extended to the brainstem and the cerebellum. These hypometabolisms are associated with patients' symptoms, with a biomarker value to identify and potentially follow these patients. The hypometabolism of the frontal cluster, which included the olfactory gyrus, seems to be linked to ACE drugs in patients with high blood pressure, with also a better metabolism of this olfactory region in patients using nasal decongestant spray, suggesting a possible role of ACE receptors as an olfactory gateway for this neurotropism.

[Evaluation with chest CT scan of CPAP as a treatment for tracheobronchomalacia in the frame of a Mounier-Kuhn syndrome]. / Évaluation par tomodensitométrie thoracique d'un traitement de la trachéobronchomalacie par pression positive continue dans le cadre d'un syndrome de Mounier­Kuhn.

INTRODUCTION: Tracheobronchomegaly disease is often associated with a tracheobronchomalacia which is responsible for recurrent lower respiratory tract infections. Currently there is no evidence to support any specific treatment for the condition. CASE REPORT: We report the case of a 79 years old patient presenting with tracheobronchomegaly in the context of Mounier-Kuhn syndrome complicated by a tracheobronchomalacia responsible for her symptomatology. The diagnosis of tracheobronchomalacia had been confirmed by high-resolution chest computed tomography (CT) with expiratory slices and virtual bronchoscopy. Treatment with continuous positive airway pressure (CPAP) was proposed, and we confirmed its efficacy using high-resolution chest CT, which showed a decrease in tracheobronchial collapse and a reduction in air trapping. CONCLUSIONS: Three-dimensional virtual bronchoscopy is an interesting tool and a noninvasive method to diagnose tracheobronchomegaly for patients who are at a high anesthetic risk. It is also possible to use it demonstrate the effect of CPAP in tracheobronchomalacia.

Prostatic artery embolization using three-dimensional cone-beam computed tomography.

PURPOSE: The purpose of this study was to report the use of three-dimensional (3D) cone-beam computed tomography (CBCT) for prostatic artery embolization (PAE) in patients with benign prostatic hypertrophy (BPH). MATERIALS AND METHODS: Twenty-three consecutive men who underwent PAE using 3D CBCT from June 2016 to September 2018, were retrospectively included in this observational single-center study. There were 23 men with a mean age of 73±12 (SD) years (range: 52-94years) with moderate to severe lower urinary tract symptoms (mean international prostate symptom score, 21±5.7 [SD]; range: 9-30) due to BPH (mean prostate weight, 100g±63 [SD]; range: 30-250g). PAEs were analyzed with respect to procedure time, fluoroscopy time, technical success, complications and dosimetric indices. RESULTS: The mean catheterization time of the prostatic artery from the internal iliac artery was 17.3±12.5 (SD) min (range: 8-57min) on the right side and 23.6±14.9 (SD) min (range: 6-54min) on the left side. A technical success was achieved in 21 patients (21/23; 91%). PAE was bilateral in 14 patients (14/21; 66%) and unilateral in 7 patients (7/21; 33%). No occurrence of non-target embolization was reported. The mean dose area product was 146.7±47.9 (SD) Gy.cm2 (range: 54-254Gy.cm2) and mean cumulative air kerma was 771.4±333.3 (SD) mGy; range: 280-1560 mGy. The mean fluoroscopy time was 42.3±23.1 (SD) min (range: 19.4-118.2min). CONCLUSION: 3D CBCT is a useful tool to identify the prostatic arteries and facilitates catheterization of prostatic arteries with an acceptable level of radiation exposure.

Quantification of right ventricular extracellular volume in pulmonary hypertension using cardiac magnetic resonance imaging.

PURPOSE: The purpose of this prospective study was to assess the value of biventricular extracellular volume (ECV) in pre-capillary pulmonary hypertension (PH) obtained using cardiac magnetic resonance imaging (CMR) and to correlate ECV with markers of prognosis such as strain echocardiography and blood biomarkers of fibrosis. MATERIALS AND METHODS: Twelve patients with PH (6 men, 6 women; mean age=50±16 [SD] years; age range: 22-73 years) underwent the same day: (i), transthoracic echocardiography including measurement of right ventricular (RV) fractional shortening (RVfs), tricuspid annular plane systolic excursion (TAPSE), maximal tricuspid annular velocity, RV global and segmental deformation; (ii), right heart catheterization measuring pulmonary arterial pressures (inmmHg) and cardiac output (inL/min); (iii), CMR at 1.5-T measuring RV volumes and ejection fraction; (iv), native and 15min post-contrast T1 mapping using modified look-locker inversion-recovery sequence; and (v), serum quantification of two biomarkers of collagen turnover and hematocrit. Non-parametric Mann-Whitney tests were used to search for differences between categorical variables. Spearman correlation test was used for search for correlation between quantitative values. RESULTS: Global RV ECV was 34%±4.2 (SD) for our entire population. A significant correlation was found between RV ECV and RVfs (r=0.6; P=0.026), S wave velocity (r=0.7; P=0.009), TAPSE (r=0.6; P=0.040) and RV systolic ejection fraction on CMR (r=0.6; P=0.04). There were no correlations between the ECV values in the lateral wall of the RV and in the septum (r=0.4; P=0.206). A significant correlation was found between septal ECV and 2D septal strain (r=0.7; P=0.013). CONCLUSION: ECV in PH as obtained using CMR appears to correlate with known echocardiographic prognostic markers and more specifically with the markers, which assess RV systolic function.

Distribution of left ventricular trabeculation across age and gender in 140 healthy Caucasian subjects on MR imaging.

PURPOSE: The purpose of this study was to quantify the distribution of trabeculated (T) and compact (C) left ventricular (LV) myocardium masses in a healthy Caucasian population against age, gender and LV parameters, and to provide normal values for T, C and T/C. MATERIALS AND METHODS: One hundred and forty healthy subjects were prospectively recruited and underwent cardiac MRI at 1.5T with a stack of short-axis cine sequences covering the entire LV. End-diastolic volume (EDV), C and T masses were quantified using a semi-automatic method. Ejection fraction (EF) and T/C ratio were computed. RESULTS: We included 70 men and 70 women with a mean age of 44±14 (SD) years (range: 20-69 years). The mean EF was 63.7±6.3 (SD) % (range: 50.7-82.0%), the mean EDV was 75.9±16.2 (SD) mL/m2 (range: 36.4-112.2mL/m2), the mean C mass was 53.9±11.2 (SD) g/m2 (range: 26.5-93.4g/m2) and the mean T mass was 4.9±2.4 (SD) g/m2 (range: 1.1-11.4g/m2). The T/C ratio was 9.2±4.5% (range: 2.0-29.4%). Multivariate ANOVA test showed that the compact mass was influenced by EDV (P<0.0001), EF (P=0.001) and gender (P<0.0001), and the trabeculated mass depended on EDV (P<0.0001), gender (P=0.002) and age (P<0.0001), while the T/C ratio was only influenced by age (P=0.0003). Spearman test showed a correlation between EDV and C (r=0.60; P<0.0001),T (r=0.46; P<0.0001) and T/C ratio (r=0.26; P=0.0023).T and T/C ratio correlated with EF (r=-0.18, P=0.0373; r=-0.18, P=0.0321, respectively). CONCLUSION: While the compact and trabeculated myocardium masses appear to relate separately to the cardiac function, age and gender, their ratio T/C appears to only decrease with age. Furthermore, we propose here normal values for T, C and T/C in a cohort of healthy Caucasians subjects.

[Pleuro-pneumonia revealing septic thrombophlebitis of the jugular vein: Think about the Lemierre's syndrome]. / Pleuro-pneumopathie révélant une thrombophlébite septique de la veine jugulaire : pensez au syndrome de Lemierre.

INTRODUCTION: Infection by Fusobacterium necrophorum, a Gram negative anaerobic bacteria, can lead to the development of Lemierre's syndrome, an uncommon but potentially fatal infection of the internal jugular vein. Since the introduction of antibiotics, the morbidity and mortality associated with this syndrome have been dramatically reduced. This syndrome is characterized by a pharyngeal infection, which leads to the development of septic thrombophlebitis of the internal jugular vein with septic emboli, which usually spread into the lung. Sometimes the syndrome can be revealed by other unusual clinical symptoms. CASE REPORT: We present the case of a young patient with an atypical pleural infection associated with pharyngeal infection and thrombosis from the internal jugular vein. CONCLUSIONS: The diagnosis of Lemierre's syndrome is mainly clinical, based on a range of suggesting symptoms. Confirmation and monitoring of the condition can be done by ultra-sound and/or contrast enhanced computed tomography. Treatment is based on long-term, high-dose antibiotic therapy and eventually surgical drainage of the infected collection with surgical excision of the internal jugular vein where there is extensive thrombosis. The important message is that in the context of a young patient without comorbidity, who presents with a significant infectious pleurisy, it is important to consider this clinical complication.