[Genetic diffuse cystic lung disease in adults]. / Maladies kystiques pulmonaires de l'adulte d'origine génétique.

Multiple cystic lung diseases comprise a wide range of various diseases, some of them of genetic origin. Lymphangioleiomyomatosis (LAM) is a disease occurring almost exclusively in women, sporadically or in association with tuberous sclerosis complex (TSC). Patients with LAM present with lymphatic complications, renal angiomyolipomas and cystic lung disease responsible for spontaneous pneumothoraces and progressive respiratory insufficiency. TSC and LAM have been ascribed to mutations in TSC1 or TSC2 genes. Patients with TSC are variably affected by cutaneous, cognitive and neuropsychiatric manifestations, epilepsy, cerebral and renal tumors, usually of benign nature. Birt-Hogg-Dubé syndrome is caused by mutations in FLCN encoding folliculin. This syndrome includes lung cysts of basal predominance, cutaneous fibrofolliculomas and various renal tumors. The main complications are spontaneous pneumothoraces and renal tumors requiring systematic screening. The mammalian target of rapamycin (mTOR) pathway is involved in the pathophysiology of TSC, sporadic LAM and Birt-Hogg-Dubé syndrome. MTOR inhibitors are used in LAM and in TSC while Birt-Hogg-Dubé syndrome does not progress towards chronic respiratory failure. Future challenges in these often under-recognized diseases include the need to reduce the delay to diagnosis, and to develop potentially curative treatments. In France, physicians can seek help from the network of reference centers for the diagnosis and management of rare pulmonary diseases.

[French practical guidelines for the diagnosis and management of IPF - 2021 update, full version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version intégrale.

BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

[French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version courte.

BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

Improved coronary calcium detection and quantification with low-dose full field-of-view photon-counting CT: a phantom study.

OBJECTIVE: The aim of the current study was to systematically assess coronary artery calcium (CAC) detection and quantification for spectral photon-counting CT (SPCCT) in comparison to conventional CT and, in addition, to evaluate the possibility of radiation dose reduction. METHODS: Routine clinical CAC CT protocols were used for data acquisition and reconstruction of two CAC containing cylindrical inserts which were positioned within an anthropomorphic thorax phantom. In addition, data was acquired at 50% lower radiation dose by reducing tube current, and slice thickness was decreased. Calcifications were considered detectable when three adjacent voxels exceeded the CAC scoring threshold of 130 Hounsfield units (HU). Quantification of CAC (as volume and mass score) was assessed by comparison with known physical quantities. RESULTS: In comparison with CT, SPCCT detected 33% and 7% more calcifications for the small and large phantoms, respectively. At reduced radiation dose and reduced slice thickness, small phantom CAC detection increased by 108% and 150% for CT and SPCCT, respectively. For the large phantom size, noise levels interfered with CAC detection. Although comparable between CT and SPCCT, routine protocols CAC quantification showed large deviations (up to 134%) from physical CAC volume. At reduced radiation dose and slice thickness, physical volume overestimations decreased to 96% and 72% for CT and SPCCT, respectively. In comparison with volume scores, mass score deviations from physical quantities were smaller. CONCLUSION: CAC detection on SPCCT is superior to CT, and was even preserved at a reduced radiation dose. Furthermore, SPCCT allows for improved physical volume estimation. KEY POINTS: • In comparison with conventional CT, increased coronary artery calcium detection (up to 156%) for spectral photon-counting CT was found, even at 50% radiation dose reduction. • Spectral photon-counting CT can more accurately measure physical volumes than conventional CT, especially at reduced slice thickness and for high-density coronary artery calcium. • For both conventional and spectral photon-counting CT, reduced slice thickness reconstructions result in more accurate physical mass approximation.

Head-to-head comparison of lung perfusion with dual-energy CT and SPECT-CT.

PURPOSE: To compare the quantitative and qualitative lung perfusion data acquired with dual energy CT (DECT) to that acquired with a large field-of-view cadmium-zinc-telluride camera single-photon emission CT coupled to a CT system (SPECT-CT). MATERIALS AND METHODS: A total of 53 patients who underwent both dual-layer DECT angiography and perfusion SPECT-CT for pulmonary hypertension or pre-operative lobar resection surgery were retrospectively included. There were 30 men and 23 women with a mean age of 65.4±17.5 (SD)years (range: 18-88years). Relative lobar perfusion was calculated by dividing the amount (of radiotracer or iodinated contrast agent) per lobe by the total amount in both lungs. Linear regression, Bland-Altman analysis, and Pearson's correlation coefficient were also calculated. Kappa test was used to test agreements in morphology and severity of perfusion defects assessed on SPECT-CT and on DECT iodine maps with a one-month interval. Wilcoxon rank sum test was used to compare the sharpness of perfusion defects and radiation dose among modalities. RESULTS: Strong correlations for relative lobar perfusion using linear regression analysis and Pearson's correlation coefficient (r=0.93) were found. Bland-Altman analysis revealed a -0.10 bias, with limits of agreement between [-6.01; 5.81]. With respect to SPECT- CT as standard of reference, the sensitivity, specificity, PPV, NPV, accuracy for lobar perfusion defects were 89.4% (95% CI: 82.6-93.4%), 96.5% (95% CI: 92.1-98.5%), 95.6% (95% CI: 90.9-97.8%), 91.4% (95% CI: 85.6-94.9%) and 93.0% (95% CI: 87.6-96.1%) respectively. High level of agreement was found for morphology and severity of perfusion defects between modalities (Kappa=0.84 and 0.86 respectively) and on DECT images among readers (Kappa=0.94 and 0.89 respectively). A significantly sharper delineation of perfusion defects was found on DECT images (P<0.0001) using a significantly lower equivalent dose of 4.1±2.3 (SD) mSv (range: 1.9-11.85mSv) compared to an equivalent dose of 5.3±1.1 (SD) mSv (range: 2.8-7.3mSv) for SPECT-CT, corresponding to a 21.2% dose reduction (P=0.0004). CONCLUSION: DECT imaging shows strong quantitative correlations and qualitative agreements with SPECT-CT for the evaluation of lung perfusion.

Five simultaneous artificial intelligence data challenges on ultrasound, CT, and MRI.

PURPOSE: The goal of this data challenge was to create a structured dynamic with the following objectives: (1) teach radiologists the new rules of General Data Protection Regulation (GDPR), while building a large multicentric prospective database of ultrasound, computed tomography (CT) and MRI patient images; (2) build a network including radiologists, researchers, start-ups, large companies, and students from engineering schools, and; (3) provide all French stakeholders working together during 5 data challenges with a secured framework, offering a realistic picture of the benefits and concerns in October 2018. MATERIALS AND METHODS: Relevant clinical questions were chosen by the Société Francaise de Radiologie. The challenge was designed to respect all French ethical and data protection constraints. Multidisciplinary teams with at least one radiologist, one engineering student, and a company and/or research lab were gathered using different networks, and clinical databases were created accordingly. RESULTS: Five challenges were launched: detection of meniscal tears on MRI, segmentation of renal cortex on CT, detection and characterization of liver lesions on ultrasound, detection of breast lesions on MRI, and characterization of thyroid cartilage lesions on CT. A total of 5,170 images within 4 months were provided for the challenge by 46 radiology services. Twenty-six multidisciplinary teams with 181 contestants worked for one month on the challenges. Three challenges, meniscal tears, renal cortex, and liver lesions, resulted in an accuracy>90%. The fourth challenge (breast) reached 82% and the lastone (thyroid) 70%. CONCLUSION: Theses five challenges were able to gather a large community of radiologists, engineers, researchers, and companies in a very short period of time. The accurate results of three of the five modalities suggest that artificial intelligence is a promising tool in these radiology modalities.

Reduction of patient radiation dose with a new organ based dose modulation technique for thoraco-abdominopelvic computed tomography (CT) (Liver dose right index).

PURPOSE: To evaluate the radiation dose reduction, image quality and diagnostic confidence with thoraco-abdominopelvic computed tomography (TACT) using a new organ based dose modulation system (liver dose right index [Liver DRI]), compared to TACT using a standard automatic exposure control adjusting mA according to attenuation. METHODS: A total of 37 patients who had two TACT examinations on 2 different CT scanners, one using standard automatic exposure control (combination of a DoseRight automatic current selection and Z modulation) and one using Liver DRI were included. There were 19 men and 18 women with a mean age of 67.6±11.7 (SD) years (range: 36-85 years) For each patient, volume CT dose index (CTDIvol), size-specific dose estimates (SSDE) and signal-to-noise ratio (SNR) were evaluated at each anatomic level (lung, breast, liver and pelvis area) for each protocol. Two radiologists assessed independently image quality, artifacts and diagnostic confidence. RESULTS: The radiation dose decreased significantly using Liver DRI compared to standard automatic exposure control on the total scan length, lung, breast and pelvis area, with a significant CTDIvol reduction of 27% (P=0.0001), 23% (P=0.0002), 24% (P=0.0002) and 31% (P=0.0001), respectively; and a significant SSDE reduction of 23% (P=0.0001), 28% (P=0.0001), 23% (P=0.0002) and 29% (P=0.0001), respectively. No significant SNR reductions were observed in all measured tissues at the level of the aortic arch, celiac trunk and iliac bifurcation, except in the muscle (P=0.0013) and fat tissue (P=0.0052) at the level of the ureteral meatus. No significant differences were noted between both protocols in overall image quality, artifacts and diagnostic confidence with an excellent inter observer agreement between radiologists (Kappa values of 0.83, 0.85 and 0.88, respectively). CONCLUSION: Liver DRI organ based dose modulation technique allows significant dose reduction compared to standard automatic exposure control while preserving diagnostic image quality in all thoraco-abdominopelvic areas.