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Copyright of Nouveau Praticien Vétérinaire Canine & Féline is the property of EDP Sciences and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Immunoglobulin G4 (IgG4) disease is a recently described entity with a growing list of associated disorders. The most common manifestations are pancreatitis, infiltration of the salivary or lacrimal glands (Mikulicz syndrome) and retroperitoneal fibrosis. We report the case of a patient whose polycythaemia was secondary to IgG4 disease. Through a review of the literature, we discuss the clinical, radiological and therapeutic aspects of this rare condition. [ABSTRACT FROM AUTHOR]

Résumé: Le carcinome hépatocellulaire (CHC) se développe dans la majorité des cas sur un foie cirrhotique. Le dépistage du CHC permet un diagnostic plus précoce de CHC, un accès plus fréquent à des traitements à visée curative et une amélioration de la survie des patients. En France, le dépistage par échographie abdominale semestrielle est recommandé chez les patients cirrhotiques. Cependant, seulement 20 % des patients ont un diagnostic dans le cadre d'un programme de dépistage. En l'absence de dépistage, il peut s'agir de patients ayant une cirrhose connue mais non correctement suivie, ou de patients n'ayant pas de cirrhose ou de fibrose hépatique connue. Reconnaître les patients à risque de maladie chronique du foie et identifier la fibrose avancée chez ces patients sont des axes d'optimisation du dépistage du CHC. Hepatocellular carcinoma (HCC) develops in most cases in a cirrhotic liver. HCC screening allows for an earlier diagnosis of HCC, more frequent access to treatments with curative intents, and improved patient survival. In France, biannual abdominal ultrasound screening for HCC is recommended for cirrhotic patients. However, only 20% of patients are diagnosed with HCC in a screening program. In case of patients diagnosed outside a screening programm, it may be either because they have known cirrhosis but are not properly followed, or because they have no known cirrhosis or liver fibrosis. Recognizing patients at risk for chronic liver disease and identifying advanced fibrosis in these patients are areas for optimizing HCC screening. [ABSTRACT FROM AUTHOR]

Hepatocellular carcinoma (HCC) is a major global health challenge with rising incidence. Despite the previous approval of several novel therapeutic approaches, HCC remains the second common cause of cancer-related death worldwide. The vast majority of HCCs arises in the context of chronic fibrotic liver diseases caused by viral or metabolic etiologies. In patients with advanced liver disease the risk of HCC persists even after viral cure or control of infection. Moreover, given the change in the lifestyle and increase of obesity and metabolic disorders, HCC incidence is predicted to drastically augment in the next decade. Early detection, improvement of the screening method in patient at-risk and development of chemopreventive strategies are therefore urgently needed to reduce HCC risk. This review summarizes the major challenges in the identification of patient at risk for HCC and the emergent strategies for HCC prevention to improve patients’ outcome.

Eosinophilic fasciitis (EF) is a rare connective tissue disorder characterized by painful edema and induration of the limbs and trunk, likely associated with hypereosinophilia and hypergammaglobulinemia. EF causes arthralgia and range of motion limitation, leading to significant functional impairment and poor quality of life. Since its description by Shulman in 1974, over 300 cases have been reported. We present here a review of the latest diagnostic, pathophysiological and therapeutic developments in this disease. Magnetic resonance imaging appears useful to guide diagnosis and biopsy. Diagnosis is based on a deep skin biopsy involving the fascia, which will reveal edema, sclerofibrosis of the muscular fascia and subcutaneous tissue, and an inflammatory infiltrate sometimes composed of eosinophilic polynuclear cells. EF may occur in patients treated with immune checkpoint inhibitors and the diagnosis should be raised in case of cutaneous sclerosis in these patients. The pathophysiology of the disease remains poorly understood, and its management lacks randomized, controlled, blinded trials. First-line treatment consists in oral corticosteroid therapy, sometimes combined with an immunosuppressant, mainly methotrexate. A better understanding of the pathophysiology has opened new therapeutic perspectives and clarified the role of targeted therapies in the management of EF, such as interleukin-6 inhibitors, whose efficacy has been reported in several cases., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

"ASIA" (Auto immune/Inflammatory Syndrome Induced by Adjuvants) is the spectrum of immune-mediated diseases triggered by an adjuvant such as silicone. We report a case of pulmonary fibrosis possibly falling within the scope of ASIA associated with chronic exposure to silicone gel used in sewing machines. To our knowledge, no case of ASIA has been described following the manipulation of silicone gel from sewing machines. [ABSTRACT FROM AUTHOR]

The management of head and neck cancers is multidisciplinary, often relying on the use of combined treatments to maximize the chances of cure. Combined treatments are however also responsible for cumulative side effects. The aim of reconstructive surgery with a flap is to restore a function lost with the loss of substance from the tumor resection. However, changes in reconstructive surgery have impact of postoperative radiotherapy planning. The optimization of imaging protocols for radiotherapy planning should make it possible to identify postoperative changes and to distinguish flaps from surrounding native tissues to delineate the flaps and document the spontaneous evolution of these flaps or dose-effect relationships in case of radiotherapy. Such changes include atrophy, fibrosis of soft tissue flaps and osteoradionecrosis of bone flaps. Radiotherapy optimization also involves standardization of the definition of target volumes in situations where a flap is present, a situation that is increasingly common in routine care. This evolution of practice, beyond the essential multidisciplinary consultation meetings defining treatment indications, requires a close radio surgical collaboration with respect to technical aspects of the two disciplines. Doing so, anticipation of relapse and toxicity profiles could possibly lead to propose strategies for personalized de-escalation of multimodal treatments through interdisciplinary trials., (Copyright © 2024 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

INTRODUCTION: Chronic hepatitis C (CHC) can cause severe complications, including fibrosis and cirrhosis. Very little is known about the prevalence of these complications in the Moroccan population. METHODS: the prevalence of liver fibrosis and cirrhosis using a non-invasive blood test (FibroTest and ActiTest) was studied in 699 Moroccan patients with CHC for 4 years (from January 2014 to December 2017). The serum immunological markers: α2-macroglobulin, haptoglobin, apolipoprotein A1 were analyzed nephelometrically on BN ProSpec® System. The serum biochemical markers: γ-glutamyltransferase, alanine aminotransferase, and bilirubin were performed using the VITROS® Chemistry System Ortho Clinical Diagnostic. A 699 patients with CHC were identified. RESULTS: the overall prevalence of cirrhosis (F4) was estimated at 31.8%. 13.9% of patients with cirrhosis had a risk of developing esophageal varices and a 3.3% risk of developing primary liver cancer. The association between cirrhosis and age showed an increase in prevalence after age 55 years old [OR=7.68(95%CI=4.9-12.2); p=0.0001]. No significant association for cirrhosis was found for sex. CONCLUSION: according to the results of FibroTest, 32% of patients with CHC had cirrhosis. The older age was independently associated with liver cirrhosis.

The present study determined the regenerative effect of bone marrow-derived stem cells (BMSCs) on thioacetamide (TA)-induced liver fibrosis in rats. A total of 30 male Wistar rats were randomly divided into sham control and treatment groups. The rats of the sham control group were subdivided into three groups and sampled on the 14th, 18th, and 20th weeks after fibrosis induction. The rats of the treatment group were subdivided into two groups and sampled on the 4th and 6th weeks after BMSCs treatment. Fibrosis was induced by the intraperitoneal administration of 200 mg/kg of TA twice a week for a period of 14 weeks. All the animals underwent liver function tests and histopathologic evaluation 4 and 6 weeks after BMSCs transplantation. The BMSCs were characterized using osteogenic induction and reverse transcription-polymerase chain reaction. The BMSCs were plastic adherent, spindle-shaped, and positive for osteogenic differentiation. They expressed CD73 and were negative for CD45. The infiltration of inflammatory cells and deposition of collagen fibers were noticed after TA administration. A significant decline in inflammatory cells and a healing process were detected 4 weeks after cell transplantation. The amelioration in hepatic tissue was significant 6 weeks after cell therapy. Following the injection of BMSCs, a nonsignificant decrease was visible in aspartate transaminase level; however, this decline was significant for alanine aminotransferase level. The alkaline phosphatase and albumin levels showed an increasing trend after cell administration. The transplantation of BMSCs resulted in a significant regenerative effect after hepatic injuries. Therefore, it was shown that BMSCs transplantation can open a new window and be a therapy of choice in the amelioration of liver fibrosis.

Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disease derived from biliary dysgenesis secondary to ductal plate malformation; it often coexists with Caroli’s disease, von Meyenburg complexes, autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). Although CHF was first named and described in detail by Kerret al. in 1961, Its pathogenesis still remains unclear. The exact incidence and prevalence are not known, and only a few hundred patients with CHF have been reported in the literature to date. However, with the development of noninvasive diagnostic techniques such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), CHF may now be more frequently detected. Anatomopathological examination of liver biopsy is the gold standard in diagnosis of CHF. Patients with CHF exhibit variable clinical presentations, ranging from no symptoms to severe symptoms such as acute hepatic decompensation and even cirrhosis. The most common presentations in these patients are splenomegaly, esophageal varices, and gastrointestinal bleeding due to portal hypertension. In addition, in younger children, CHF often is accompanied by renal cysts or increased renal echogenicity. Great variability exists among the signs and symptoms of the disease from early childhood to the 5th or 6th decade of life, and in most patients the disorder is diagnosed during adolescence or young adulthood. Here, we present two cases of congenital hepatic fibrosis in 2-years-old girl and 12-year-old male who had been referred for evaluation of an abdominal distension with persistent hyper-transaminasemia and cholestasis, the diagnostic was made according to the results of medical imaging (CT or MRI), a liver biopsy, and genetic testing.

Background: Anti-centromere antibodies, anti-topoisomerase-1 antibodies (ATA), and anti-RNA-polymerase III antibodies are three Systemic Sclerosis (SSc)-specific autoantibodies. Their detection is helpful in determining the prognosis. We aimed to evaluate whether ATA levels were associated with disease severity at diagnosis or disease progression during follow-up in ATA positive patients., Methods: We conducted a single-centre French retrospective observational study, between 2014 and 2021. ATA positive patients fulfilling the ACR/EULAR 2013 classification criteria for SSc with a minimal follow-up of 1 year and 2 ATA dosages were included. SSc patients with high IgG ATA levels at baseline (>240IU/mL) were compared with SSc patients with low levels (≤240IU/mL), at inclusion and at 1 and 3 years. A variation of at least 30 % of ATA levels was considered significant., Results: Fifty-nine SSc patients were included and analysed. There was a predominance of women and of patients with diffuse interstitial lung disease. Patients with high ATA levels exhibited a higher skin sclerosis assessed by the modified Rodnan skin score (P=0.0480). They had a lower carbon monoxide transfer coefficient (P=0.0457), a lower forced vital capacity (FVC) (P=0.0427) and more frequently had a FVC under 80 %, when compared to patients with low ATA levels (P=0.0423). Initial high ATA levels were associated with vascular progression at one year (21.95 % vs. 0 %; P=0.0495)., Conclusion: ATA levels are associated with skin sclerosis and vascular progression in SSc. Beyond the detection of ATA, quantifying this autoantibody might be of interest in predicting disease severity and prognosis in SSc., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Systemic sclerosis (SSc) is a rare connective tissue disease characterized by inflammation, fibrosis, and autoimmunity. Despite few clinical trials when compared to other autoimmune diseases, SSc has benefited from renewed interest over the past ten years and a large number of clinical trials have been performed or are underway. We present here the results of the trials published in the last 5 years in ScS according to the chosen endpoint criteria and describe the trials in progress or expected in the years to come., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Résumé: Depuis son apparition au début du XXIe siècle, le FibroScan™ a pris un essor considérable. C'est un appareil présent sur tous les continents du globe et c'est une technique qui a ouvert une nouvelle avenue : la mesure de l'élasticité hépatique ou de la dureté du foie. La mesure de l'élasticité hépatique est devenue l'hépatoscope indispensable en hépatologie, au même titre que l'examen clinique ou les examens complémentaires biologiques, morphologiques ou endoscopiques. Après l'engouement initial, et les dérives de l'adolescence, le FibroScan™ est devenu maintenant adulte avec des qualités et des défauts qui font tout son intérêt et son utilité. Le FibroScan™ continue d'évoluer avec de nouvelles fonctions qui sans aucun doute nous permettront de mieux appréhender le diagnostic des maladies du foie. Et si on se donnait rendez-vous dans dix ans ? Since its appearance at the beginning of the 21st century, FibroScan™ has grown considerably. It is a device present on all the continents of the globe and it is a technique which opened a new avenue : the measurement of liver stiffness. The measurement of liver stiffness has become the hepatoscope in hepatology, in the same way as clinical examination or additional biological, morphological or endoscopic examinations. After the initial craze and the excesses of adolescence, the FibroScan™ is now an adult with qualities and flaws that make it so interesting and useful. FibroScan™ continues to evolve with new functions that will allow us to better perform the diagnosis of liver disease. What's about a new meeting in 10 years? [ABSTRACT FROM AUTHOR]

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., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Chronic elevation of transaminases is recurring issue in primary care. This article aims to be practical. It offers a quick reminder of epidemiology, pathophysiology, concept of normal values of transaminases, main causes (alcohol, fatty liver disease, viral hepatitis) and first-line assessment. Then, we will go further in less frequent causes, extra-hepatic causes and additional tests. We will precise what is the role of general practitioner in this care pathway and when to address patient to specialist., (Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Résumé La sarcoïdose est une granulomatose de cause inconnue avec une atteinte pulmonaire quasi-constante et une évolution difficile à prédire. Le diagnostic est retenu dans un triple contexte devant un tableau clinico-radiologique le plus souvent évocateur, la mise en évidence de granulomes typiques sans nécrose caséeuse par des biopsies de lésions superficielles, per-endoscopiques bronchiques ou encore ganglionnaires médiastinales sous contrôle echoendoscopique et l’exclusion des autres causes de granulomatose pouvant donner un même tableau. La corticothérapie constitue le traitement de première ligne en cas d’indication thérapeutique, situation rencontrée chez la moitié des patients. Les traitements de deuxième et de troisième ligne sont respectivement les agents cytotoxiques et les biomédicaments anti-TNF-α. Les formes pulmonaires sévères — fibrose sarcoïdienne, hypertension pulmonaire, sténoses bronchiques et greffe aspergillaire — sont les premières causes de morbi-mortalité. Les enjeux dans l’atteinte pulmonaire sont de ne pas méconnaître un diagnostic alternatif, de savoir quand et comment traiter les patients et de cibler au mieux ceux à risque de développer des formes sévères. Sarcoidosis is a granulomatous disease of unknown cause with almost constant lung involvement and a natural history difficult to predict. Diagnosis is based on triple context with an evocative clinical and radiological presentation, the demonstration of non-caseating and typical granulomas by biopsy samples of superficial lesions, perendoscopic bronchial biopsies or endobronchial ultrasound-guided transbronchial needle aspiration and the exclusion of other causes of granulomatosis. Corticotherapy is the first-line of treatment and is indicated in almost half of patients. Second – and third-line of treatments are respectively cytotoxic agents and TNF-α blockers. Severe pulmonary sarcoidosis including fibrosis, pulmonary hypertension, bronchial stenosis and aspergillosis are the leading causes of morbidity and mortality. The challenge in pulmonary involvement management is especially not to miss an alternative diagnosis, to know when and how to treat patients and to better target those at risk of developing severe forms. [ABSTRACT FROM AUTHOR]

Résumé Dans les classifications de l’OMS des néoplasies myéloprolifératives, l’analyse d’une biopsie ostéo-médullaire est un examen de premier plan nécessaire au diagnostic et à l’évaluation du pronostic. L’entité « pré-fibrose », décrite depuis plusieurs années comme distincte de la myélofibrose primitive avec myélofibrose franche, fait son entrée dans la version la plus récente de ces classifications, publiée en 2016. La place de la biopsie dans le diagnostic de maladie de Vaquez est également renforcée. Des comités de relecture regroupant des hémato-pathologistes se mettent en place afin d’améliorer la reproductibilité des critères histologiques. Summary In WHO classifications of myeloproliferative neoplasms, a bone marrow biopsy at diagnosis is mandatory in almost all cases to classify the disease as well as to evaluate the prognosis. The “pre-fibrotic” form, which has been described for several years as distinct from primary myelofibrosis with overt myelofibrosis, is included in the most recent version of these classifications published in 2016. The importance of the biopsy in the diagnosis of polycythemia vera is also growing. Re-evaluation committees bringing together hematopathologists are set up to improve the reproducibility of these histological criteria. [ABSTRACT FROM AUTHOR]

Résumé L’évaluation de la sévérité de la fibrose hépatique est un paramètre essentiel dans le suivi des maladies chroniques du foie. Elle permet selon l’étiologie en cause, d’évaluer le pronostic et représente un critère décisionnel pour la mise en place d’un traitement voire d’une stratégie de dépistage des complications de la cirrhose lorsqu’elle est mise en évidence. Dans les hépatites virales, le résultat de l’évaluation de la fibrose conditionne l’instauration d’un traitement antiviral, critère actuellement remis en cause par les experts. La biopsie hépatique est le test de référence pour l’appréciation de l’étendue de la fibrose et de l’inflammation, mais possède certaines limites. De nombreuses méthodes non invasives ont été développées, incluant des paramètres biologiques et des techniques basées sur la mesure de l’élasticité hépatique. Ces méthodes sont très performantes pour le diagnostic de fibrose avancée/cirrhose et possèdent une exactitude légèrement inférieure pour l’identification des stades précoces. Les combinaisons de tests améliorent sensiblement les performances diagnostiques, et réduisent le recours à la biopsie hépatique. L’utilisation de ces méthodes n’est validée que dans une seule indication en france (hépatite chronique C non traitée), mais les sociétés savantes, nationales et internationales, les intègrent régulièrement dans leurs recommandations de bonne pratique, au-delà des indications validées par les instances sanitaires. Avec l’évolution des pratiques cliniques, ces méthodes doivent répondre à de nouvelles attentes, notamment en termes d’aide au pronostic clinique et de prédiction de la réponse aux traitements. Summary The assessment of liver fibrosis is an important step in the follow up of chronic liver diseases. The severity of fibrosis is a strong argument for initiating a strategy for the screening of complications of cirrhosis and has a useful prognostic value. It can also help to identify which patients should sooner receive antiviral therapy, although this indication tends to be contested in chronic viral hepatitis. Liver biopsy has been the gold standard for estimating the extent of fibrosis and inflammation, although it has some limitations. Non invasive methods were developed, including blood tests and liver stiffness measurement. These methods have been shown to perform well in ruling in cirrhosis, with a less ability to discriminate between intermediate stages. Several tests combinations improve the performances of individual tests, hence avoiding liver biopsy in many cases. In france, health authorities validated noninvasive methods in chronic hepatitis C solely, but these tests are regularly integrated by international associations in their clinical guidelines, extending their use in many other etiologies. Nowadays, since clinical recommendations are moving in this field, non invasive methods have to respond to other attempts, particularly by predicting clinical outcomes of fibrosis and treatment response in viral hepatitis. [ABSTRACT FROM AUTHOR]

Résumé La transplantation hépatique (TH) s’est accrue en France ces dernières années grâce à l’augmentation du nombre d’organes prélevés, l’utilisation de greffons marginaux et l’élargissement des critères cliniques et biologiques des patients figurant sur liste d’attente. L’évaluation d’un patient candidat à la TH fait appel à des scores clinico-biologiques tels que le score MELD (Model for End Stage Liver Disease) . Ce score peut être modifié, en lien avec la pathologie hépatique concernée (composante expert, natrémie), ou en association à d’autres scores comme le score α-foetoprotéine. La clairance au vert d’indocyanine, par sa mesure simplifiée, gagne un nouvel intérêt pour évaluer la fonctionnalité du foie. Il n’existe pas encore de « Donor Risk Index» évaluant avec fiabilité la qualité du foie du donneur. Dans les suites immédiates de la TH, la biologie joue un rôle essentiel pour surveiller les receveurs à haut risque de mortalité, pour dépister en urgence la non-fonction primaire du greffon, la thrombose de l’artère hépatique et pour contribuer au diagnostic des infections. La biologie joue un rôle moindre dans les lésions d’ischémie-reperfusion et les sténoses biliaires. Après le premier mois post-TH, la surveillance biologique régulière permet le diagnostic de récidive de la maladie initiale et du rejet chronique. Elle estime la fonctionnalité du foie et pourrait évaluer le développement de fibrose avec l’aide de marqueurs sanguins non invasifs associés ou non à l’élastométrie. Summary Liver transplantations have increased in France over the past ten years thanks to the extended pool of liver donors, the use of marginal organs and an enlarged set of clinic and biological criteria for patients on the waiting list. The selection of patients eligible for liver transplantation uses clinical and biological scores as MELD score (Model for End Stage Liver Disease). This score can be modified in relation to liver pathology (expert composant, natremia) or associated with additional scores such as α-foetoprotein score. The green indocyanin clearance, with a simplified measurement, gains a new interest for the estimation of liver functionality. A Donor Risk Index able to assess with a sufficient fiability the liver donor quality has not yet been defined. Biology has an essential place in the immediate post-TH period to detect urgent complications in some recipients with high risk of mortality, primary non function of the graft, artery thrombosis and also infections. Biology plays a minor role in other early complications as ischemia-reperfusion and biliary stenosis. For post-transplantation follow up in the longer term, a regular biology assessment enables the diagnosis of recurrent diseases or chronic rejection. It allows to estimate the graft functionality and could assess a fibrosis development, using blood non-invasive biomarkers associated or not with elastometry. [ABSTRACT FROM AUTHOR]

Résumé L’exploration biochimique du foie est particulièrement importante étant donné ses fonctions de synthèse, de stockage, d’élimination et de transformation de molécules biologiques. L’exploration des fonctions excréto-biliaires passe par les dosages sériques des différentes formes de la bilirubine, des acides biliaires, des enzymes PAL, GGT et 5’-Nuc. Elle est utile au diagnostic d’une cholestase et à la classification des ictères. Les fonctions de synthèse du foie sont explorées par le dosage de l’albumine et la réalisation de tests d’hémostase, qui jugeront du degré d’insuffisance hépatique. Le foie a aussi d’importantes fonctions d’épuration qui pourront être étudiées par le dosage de l’ammoniémie et la réalisation de tests d’épuration comme à la BSP ou à l’ICG. La cytolyse hépatique est classiquement étudiée par la mesure d’enzymes plasmatiques comme les transaminases (ASAT et ALAT), la LDH et ses iso-enzymes, la GLDH ou encore l’α-GST. L’inflammation est étudiée par la mesure de la VS et l’électrophorèse des protéines sériques. La fibrose hépatique bénéficie de dosages de protéines de la matrice extracellulaire, souvent présentés sous forme de scores de fibrose. La détection de l’alcoolisme s’est aussi améliorée par de nouvelles approches avec le test CDT (« Carbohydrate Deficiency Transferrin »), plus spécifique que l’augmentation du « volume globulaire moyen » (VGM) et de l’activité de la « Gamma-Glutamyl-Transpeptidase » (GGT). Summary Biochemistry of the liver is particularly relevant because liver exerts many important functions such as synthesis, stocking, elimination and transformation of biomolecules. The biochemical exploration of bile secretion needs the determination in serum of various bilirubin forms, bile acids, and enzymes such as PAL, GGT and 5’-Nuc, all of them being useful for the diagnosis of cholestasis and to the classification of icterus. The functions of the liver are explored by the dosage of both albumin and hemostasis factors reflecting the level of liver failure. The liver exerts also important purification functions which can be explored by the determination of ammonium, and the realization of BSP or ICG tests. Liver cytolysis is classically studied by transaminases (SGOT and SGPT), LDH and its iso-enzymes, GLDH and even α-GST. Inflammation is studied with the measure of the sedimentation rate and the electrophoresis analysis of serum proteins. Liver fibrosis has now the benefit of the dosages of proteins from the extracellular matrix, often gathered in fibrosis scores. The detection of alcoholism is easier with the development of CDT test (« Carbohydrate Deficiency Transferrin »), more specific than the elevated « Mean Corpuscular Volume » (MCV) and the « Gamma-Glutamyl-Transpeptidase » (GGT) activity. [ABSTRACT FROM AUTHOR]

Introduction: While short-term results of lung transplantation have improved considerably, long-term survival remains below that achieved for other solid organ transplants., Current Knowledge: The main cause of late mortality is chronic lung allograft dysfunction (CLAD), which affects nearly half of the recipients 5 years after transplantation. Immunological and non-immune risk factors have been identified. These factors activate the innate and adaptive immune system, leading to lesional and altered wound-healing processes, which result in fibrosis affecting the small airways or interstitial tissue. Several phenotypes of CLAD have been identified based on respiratory function and imaging pattern. Aside from retransplantation, which is possible for only small number of patients, no treatment can reverse the CLAD process., Perspectives: Current therapeutic research is focused on anti-fibrotic treatments and photopheresis. Basic research has identified numerous biomarkers that could prove to be relevant as therapeutic targets., Conclusion: While the pathophysiological mechanisms of CLAD are better understood than before, a major therapeutic challenge remains., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

La fibrose rétropéritonéale (ou maladie d'Ormond) est une pathologie rare et insidieuse. Elle réunit un ensemble d'affections caractérisées par la présence d'un tissu fibro-inflammatoire aberrant. Le défi, pour le clinicien, réside dans la distinction entre sa forme primitive et ses formes secondaires à des pathologies infiltratives, infectieuses ou malignes. La fibrose rétropéritonéale secondaire à la tuberculose est rarement rapportée dans la littérature, son diagnostic est difficile et ses mécanismes physiopathologiques ne sont pas clairs. Nous rapportons dans cette observation le cas d'un patient âgé de 53 ans présentant des rachialgies inflammatoires en rapport avec une fibrose rétropéritonéale secondaire à une spondylite tuberculeuse débutante traitée efficacement par les antibacillaires. [ABSTRACT FROM AUTHOR]

Résumé La maladie associée aux IgG4 est une entité de reconnaissance récente. Elle se caractérise par l’atteinte d’un ou de plusieurs organes avec cliniquement des lésions tumorale et histologiquement un infiltrat lymphoplasmocytaire, associé à une fibrose. Dans le sang, dans près de 70% des cas, on retrouve une augmentation de la concentration des IgG4. Les principaux organes atteints sont le pancréas (pancréatite auto-immune de type 1), les voies biliaires, les glandes salivaires et lacrymales, le rétropéritoine, mais quasiment tous les organes peuvent être atteints. La physiopathologie est encore incomplètement comprise et implique de nombreux mécanismes de l’immunité innée et acquise. Le diagnostic actuel repose sur une connaissance des principaux organes atteints, le dosage sérique des IgG4 et sur une analyse histologique complète et rigoureuse. La quantification des plasmablastes semble prometteuse particulièrement en cas de dosage des IgG4 normal. La maladie associée aux IgG4 est très cortico-sensible. Dans les situations où le traitement est insuffisant, le rituximab est un traitement proposé. Summary Immunoglobulin G4- related disease (IgG4-RD) is a recent entity. It is characterized clinically, by involvement of one or more organs with tumor-like lesions and lymphoplasmablast infiltrate associated with fibrosis on biopsies. Elevated serum IgG4 concentration is found in many but not all patients. The main organs affected are the pancreas (type 1 autoimmune pancreatitis), bile ducts, salivary and lacrimal glands, retro-peritoneum, but all organs can be affected. The IgG4-RD pathophysiology remains incompletely understood and involves many mechanisms of innate and acquired immunity. The current diagnosis is based on knowledge of the main organs affected, serum IgG4 concentration and a rigorous histological analysis. Quantification of circulating plasmablasts looks promising especially in patients with normal IgG4 concentration in serum. IgG4-RD is a steroid-sensitive disease, Others immunosuppressive treatments, as rituximab, could also be proposed. [ABSTRACT FROM AUTHOR]

Résumé La cicatrice chéloïde est une pathologie de la cicatrisation cutanée. Il s’agit d’une prolifération fibreuse du derme liée à une accumulation de fibres de collagène secondaire à une plaie cutanée. Son aspect clinique et anatomo-pathologique est caractéristique bien qu’elle soit souvent confondue avec une cicatrice hypertrophique. Le principal mécanisme physiopathologique est un dérèglement de la synthèse et de la destruction du collagène lié à une hyperactivité du TGFβ. Dans cette première partie, sont expliquées les caractéristiques cliniques, épidémiologiques, physiopathologiques et histologiques des cicatrices chéloïdes. Summary Keloid scars are a dysregulated response to cutaneous wound healing and are characterized by excessive deposition of collagen. Clinical and histological aspects are typical but they are often confused with hypertrophic scars. Principal pathogenesis is abnormal regulation of the collagen equilibrium because of TGFβ. In this first part, clinical characteristics, physiopathology and histology of keloid scars are explained. [ABSTRACT FROM AUTHOR]

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Résumé Toute hépatite C chronique doit faire l’objet, avant l’initiation d’un traitement, d’un bilan, incluant le diagnostic sérologique et virologique, et la détermination de la sévérité de la maladie. Si la ponction biopsie hépatique reste la règle, les instances sanitaires encouragent toutefois le recours aux marqueurs non invasifs spécifiques pour uniformiser les résultats cliniques. Summary In cases of chronic hepatitis C, a thorough assessment must always be carried out before any treatment begins. This assessment must include a serological and virological diagnosis and the seriousness of the disease must be established. While a liver biopsy is still the rule, health authorities encourage the use of specific non-invasive markers to standardise the clinical results. [ABSTRACT FROM AUTHOR]

Résumé Objectifs de l’étude Les techniques de radiothérapie conformationnelle impliquent souvent la ré-irradiation des volumes irradiés à quelques secondes ou minutes d’intervalle. Ce « microfractionnement » est susceptible d’altérer la réponse des tissus cibles au rayonnement. D’autre part, les techniques de pencil beam scanning (PBS) qui se développent en protonthérapie, mettent en œuvre des microfaisceaux dans lesquels le débit de dose peut atteindre 200 Gy/s, soit 1000 fois plus qu’en radiothérapie classique. Les conséquences de ces nouvelles modalités d’administration de la dose sur l’efficacité antitumorale aussi bien que sur la réponse des tissus sains, n’avaient pas été investiguées sur des modèles animaux. Afin de combler cette lacune, nous avons développé une méthodologie (« flash ») permettant d’irradier des souris en un temps très court (inférieur à 500 ms) à haut débit de dose (supérieur ou égal à 40 Gy/s). Matériel et méthodes Nous avons exploré, dans ces conditions, le développement de la fibrose pulmonaire radio-induite après irradiation thoracique bilatérale. Résultats L’analyse des résultats montre que l’irradiation flash n’induit pas de fibrose pulmonaire aux doses où l’irradiation conventionnelle en produit 100 % et réduit de manière spectaculaire l’expression du TGF-bêta et le taux d’apoptose dans le tissu pulmonaire. En revanche, aucune perte d’efficacité antitumorale n’a été observée sur trois modèles tumoraux exogéniques ou syngénique implantés en xénogreffes ou greffe orthotopique et l’escalade de dose qu’autorise l’épargne des tissus sains permet dans certains cas d’atteindre une cure complète. Conclusion En définitive, l’irradiation flash permet d’accroître le différentiel tumeurs/tissus sains à l’avantage de ces derniers, suggérant un potentiel prometteur en radiothérapie anticancéreuse. Purpose Pencil beam scanning and filter free techniques may involve dose-rates considerably higher than those used in conventional external-beam radiotherapy. Our purpose was to investigate normal tissue and tumour responses in vivo to short pulses of radiation. Material and methods C57BL/6J mice were exposed to bilateral thorax irradiation using pulsed (at least 40 Gy/s, flash) or conventional dose-rate irradiation (0.03 Gy/s or less) in single dose. Immunohistochemical and histological methods were used to compare early radio-induced apoptosis and the development of lung fibrosis in the two situations. The response of two human (HBCx-12A, HEp-2) tumour xenografts in nude mice and one syngeneic, orthotopic lung carcinoma in C57BL/6J mice (TC-1 Luc+), was monitored in both radiation modes. Results A 17 Gy conventional irradiation induced pulmonary fibrosis and activation of the TGF-beta cascade in 100% of the animals 24–36 weeks post-treatment, as expected, whereas no animal developed complications below 23 Gy flash irradiation, and a 30 Gy flash irradiation was required to induce the same extent of fibrosis as 17 Gy conventional irradiation. Cutaneous lesions were also reduced in severity. Flash irradiation protected vascular and bronchial smooth muscle cells as well as epithelial cells of bronchi against acute apoptosis as shown by analysis of caspase-3 activation and TUNEL staining. In contrast, the antitumour effectiveness of flash irradiation was maintained and not different from that of conventional irradiation. Conclusion Flash irradiation shifted by a large factor the threshold dose required to initiate lung fibrosis without loss of the antitumour efficiency, suggesting that the method might be used to advantage to minimize the complications of radiotherapy. [ABSTRACT FROM AUTHOR]

Résumé La fibrose systémique néphrogénique est une entité rare touchant les patients en insuffisance rénale. Elle serait liée à une toxicité au gadolinium utilisé comme produit de contraste en imagerie par résonance magnétique. Les manifestations cliniques sont variables. Les lésions cutanées sont inaugurales, à types d’induration et d’épaississement cutané débutant souvent au niveau des membres inférieurs et s’étendant aux membres supérieurs. Les manifestations systémiques sont liées à l’extension de la fibrose. Le spectre des atteintes cutanées et systémiques ne cesse d’évoluer. Nous rapportons un nouveau cas de fibrose systémique néphrogénique où les lésions cutanées débutent au niveau du membre supérieur droit, sous forme de nodules indurés et ulcérés. Le diagnostic est histologique. Nephrogenic systemic fibrosis is a rare entity occurred in patients with renal failure. It is related to toxicity to gadolinium, which is used as a contrast agent in magnetic resonance imaging. Clinic manifestations are variable. They begin by a thickening of the skin in the lower limbs and extending to the upper limbs. We report a new case of nephrogenic systemic fibrosis where cutaneous lesions begin in the upper right limb as indurated and ulcerated nodules. The diagnosis is histological. [ABSTRACT FROM AUTHOR]

Cardiovascular magnetic resonance has emerged as a very helpful tool for the interventional cardiologists not only in the assessment and treatment of coronary artery disease, but also in the evaluation of various structural cardiac diseases. The main pulse sequences are standardised, acquired during short breath-holds, and include steady-state free precession cines, dynamic myocardial first-pass perfusion imaging during contrast injection, and late enhancement imaging for the identification of myocardial substrates. Less than 30-minute CMR studies are now available for the most common clinical indications. More recently, T1 and T2 parametric myocardial maps are promising for detailed myocardial tissue characterisation (edema, replacement fibrosis, diffuse interstitial fibrosis). Technical aspects will not be addressed with particular emphasis on clinical applications., Competing Interests: Déclaration de liens d'intérêts Les auteurs déclarent n'avoir aucun lien d'intérêt en relation avec ce manuscrit., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Primary tumors are a rare phenomenon, while cardiac metastasis is rather more frequent. Cardiac masses and metastasis are usually first detected by echocardiography, which allows to describe and detail the size, the insertion, the mobility, the relation with cardiac and non-cardiac structure, and local invasion. Myocardial contrast echocardiography is able to improve tissue characterization of the masses. Transthoracic echocardiography evaluation is generally completed by cardiac magnetic resonance and computed tomography scanner, which give some important and additional information on tissue characteristics and tissue property such as vascularity and fibrosis. [ABSTRACT FROM AUTHOR]

Résumé: La fibrogenèse sous-épithéliale est une composante du remodelage tissulaire souvent observée au cours des pathologies allergiques touchant les muqueuses, tel l’asthme. Les acteurs de l’inflammation allergique, en particulier les cytokines Th2 et les cellules inflammatoires, sont en effet capables de favoriser la fibrose tissulaire à différents niveaux, et ce soit directement, soit de façon dépendante de la production de facteurs pro-fibrosants, tel le TGF-ß1. L’effet bénéfique des glucocorticoïdes sur la fibrose tissulaire n’ayant pas été clairement démontré, il est nécessaire de mieux appréhender la contribution de la réponse allergique à ce processus afin d’identifier de nouvelles cibles moléculaires pour une intervention pharmacologique visant à en empêcher la progression, voire la faire régresser. [ABSTRACT FROM AUTHOR]