Alveolar echinococcosis: update 2016

L’échinococcose alvéolaire (EA) est une cestodose larvaire rare, due au développement dans le foie de la larve d’Echinococcus multilocularis. Sa progression s’apparente à celle d’un cancer à marche lente. En France, les caractéristiques épidémiologiques de l’EA se sont modifiées récemment. Les foyers endémiques restent localisés à l’Est et au Centre du Territoire, mais la limite occidentale de la zone d’endémie française dépasse désormais Paris et concerne également les zones urbaines. Le registre français des cas indique par ailleurs une augmentation de l’incidence annuelle. Elle est probablement surtout la conséquence d’une amélioration significative des techniques de diagnostic, en particulier de l’échographie, examen de première ligne pour le diagnostic de cette affection. Le développement de thérapeutiques immunosuppressives (chimiothérapie, biothérapie) pour diverses pathologies peut fournir une deuxième explication, avec l’observation croissante de cas d’EA découverts en situation d’immunosuppression. La maladie est repérée plus tôt dans son cours évolutif, très souvent à un stade asymptomatique. L’ictère qui, par le passé, révélait l’EA dans la moitié des cas, n’est aujourd’hui que très rarement présent au diagnostic. La chirurgie est restée jusque dans les années 1980, la seule et unique solution pour son traitement, souvent palliatif. Des options complémentaires, ou distinctes, sont apparues au cours de ces trente dernières années : administration d’albendazole exerçant un effet parasitostatique, radiologie interventionnelle, transplantation hépatique en cas d’EA évoluées. Toutes ces acquisitions, associées à un diagnostic généralement plus précoce, aux progrès des techniques d’imagerie et de la chirurgie hépato-biliaire ainsi qu’à une approche multidisciplinaire de cette prise en charge, ont permis d’améliorer considérablement le pronostic.Alveolar echinococcosis (AE) is a rare larval cestodosis infestation caused by the intra-hepatic development of Echinococcus multilocularis larva. Its progression in the liver is similar to a slow-growing cancer. Recently in France, the epidemiological characteristics of AE evolved. The endemic areas are still located in the East and in the Center of the territory, but the western limit of the French endemic zone now extends beyond Paris and includes other urban areas. The French Registry also indicates an increase of the annual incidence rate. Above all, this phenomenon is probably due to the major improvement of the diagnostic techniques – especially liver ultrasound exam, that proved to be the first line exam to diagnose this parasitic disease. This could also be explained by the development of immunosuppressive therapeutics like chemotherapy or biotherapy to treat various pathological conditions, which is linked with the growing observation of AE discovered in immunosuppressive situations. Today, the disease is detected earlier in its course, most of the time at an asymptomatic stage. By the past, jaundice, which usually reflect an advanced disease was the revealing symptom in half of the cases; it is now very rarely present at the time of diagnosis. Until the 1980s, surgery remained the only solution for its treatment – which was often part of a palliative care. Additional or alternative options appeared during the last 30 years: administration of albendazole having a parasitostatic effect, interventional radiology, liver transplantation in case of advanced AE. All these acquisitions, combined with an earlier diagnosis, the progress in imagery technology and in hepatobiliary surgery, and a multidisciplinary approach of the patient’s care, contributed to improve significantly AE prognosis

Indications and results of liver transplantation for Echinococcus alveolar infection: an overview

Background: Alveolar echinococcosis (AE) of the liver, caused by the larval stage of the fox tapeworm Echinococcus multilocularis, has the characteristics of a slow-growing liver cancer. It is one of the rare parasitic diseases for which a parasitolytic drug is not yet available, and AE is lethal in the absence of appropriate therapeutic management. Complete surgical resection of the parasite at an early stage of infection provides favourable prospects for cure, but, due to a long clinical latency, many cases are diagnosed at an advanced stage, so that partial liver resection can be performed in only 35% of patients. Benzimidazole (BZM) treatment is given in inoperable cases but these compounds are only parasitostatic, and lifelong therapy is required. During the past 20 years some centres have considered liver transplantation (LT) for the treatment of incurable AE. Methods: Our review summarizes the results of this experience based on a series of 47 European patients who received transplants between 1985 and 2002, tries to specify the real place of LT for AE, and underlines the measures that could be undertaken in the future to improve the results. Results: Five-year survival was 71%. Five-year survival without recurrence was 58%. Major technical difficulties related either to previous laparotomies or to the loco-regional involvement were observed. The nine early deaths concerned AE patients with a long past-history of symptomatic AE (iterative cholangitis, secondary biliary cirrhosis). Five late deaths were directly related to ongoing AE, located in the brain in three cases, a very rare AE location that was not investigated before LT in these patients. Conclusions: In general, the pre-LT screening for distant AE metastases appeared insufficient in this series. Heavy immunosuppressive schemes, absence or delayed re-introduction of BZM after LT have clearly played a role in this unfavourable course. This unique experience indicates that, despite major technical difficulties, LT for incurable AE is feasible and could be discussed in very symptomatic cases. Before LT, interventional radiology should be preferred to repeated laparotomies. Pre-LT and post-LT BZM treatment is mandatory. A careful evaluation of possible distant metastases should be done before the decision for LT is made. After LT, the possibility of an ongoing AE must be permanently kept in mind. This could be reduced by lightening the immunosuppressants, carefully following the specific circulating antibodies, and applying a systematic radiological evaluation, not only to the graft but also to the lungs and the brai

Alveolar echinococcosis in solid organ transplant recipients: a case series from two national cohorts

Alveolar echinococcosis (AE) is a severe parasitic infection caused by the ingestion of Echinococcus multilocularis eggs. While higher incidence and faster evolution have been reported in immunosuppressed patients, no studies have been performed specifically on AE in transplant patients. We searched for all de novo AE cases diagnosed between January 2008 and August 2018 in solid organ transplant (SOT) recipients included in the Swiss Transplant Cohort Study and the FrancEchino Registry. Eight cases were identified (kidney = 5, lung = 2, heart = 1, liver = 0), half of which were asymptomatic at diagnosis. AE diagnosis was difficult due to the low sensitivity (60%) of the standard screening serology (Em2+) and the frequently atypical radiological presentations. Conversely, Echinococcus Western blot retained good diagnostic performances and was positive in all eight cases. Five patients underwent surgery, but complete resection could only be achieved in one case. Moreover, two patients died of peri-operative complications. Albendazole was initiated in seven patients and was well tolerated. Overall, AE regressed in one, stabilized in three, and progressed in one case, and had an overall mortality of 37.5% (3/8 patients). Our data suggest that AE has a higher mortality and a faster clinical course in SOT recipients; they also suggest that the parasitic disease might be due to the reactivation of latent microscopic liver lesions through immune suppression. Western blot serology should be preferred in this population. Finally, surgery should be considered with caution, because of its low success rate and high mortality, and conservative treatment with albendazole is well tolerated

Epidemiology, clinical features and management of autoimmune hepatitis in Switzerland: a retrospective and prospective cohort study

BACKGROUND AND AIMS: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. RESULTS: A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. Paediatric cohort: median age at diagnosis 12.5 years (range 1–17, interquartile range (IQR) 8–15), 16 (53%) girls, 6 (32%) with type 2 autoimmune hepatitis, 8 (27%) with autoimmune sclerosing cholangitis, 1 with primary biliary cholangitis variant syndrome, 4 (15%) with inflammatory bowel disease and 10 (41%) with advanced liver fibrosis at diagnosis. Adult cohort: median age at diagnosis 54 years (range 42–64, IQR 18–81), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis. The median follow-up time for the entire cohort was 5.2 years (IQR 3–9.3 years). Treatment in children: 29 (97%) children were initially treated with corticosteroids, 28 of whom received combination treatment with azathioprine. Budesonide was used in four children, all in combination with azathioprine. Mycophenolate mofetil was used in five children, all of whom had previously received corticosteroids and thiopurine. Treatment in adults (data available for 228 patients): 219 (96%) were treated with corticosteroids, mostly in combination with azathioprine. Predniso(lo)ne was the corticosteroid used in three-quarters of patients; the other patients received budesonide. A total of 78 (33%) patients received mycophenolate mofetil, 62 of whom had previously been treated with azathioprine. Complete biochemical response was achieved in 13 of 19 (68%) children and 137 of 182 (75%) adults with available follow-up data. All children were alive at the last follow-up, and none had undergone liver transplantation. Five (2%) adults underwent liver transplantation, two of whom had a fulminant presentation. Four (2%) adults with autoimmune hepatitis died (two from liver-associated causes). CONCLUSION: Patients with autoimmune hepatitis in Switzerland had clinical features similar to those in other cohorts. The proportion of patients diagnosed with primary biliary cholangitis variant syndrome was higher than expected. Autoimmune hepatitis was managed according to guidelines, except for the use of budesonide in a small proportion of paediatric patients. The outcomes were excellent, but the findings must be confirmed over a longer follow-up period

L'infection humaine par Echinococcus multilocularis: du depistage a la transplantation hepatique. Aspects epidemiologiques, cliniques et immunologiques

SIGLEINIST T 76365 / INIST-CNRS - Institut de l'Information Scientifique et TechniqueFRFranc

Hépatite B : le regard français sur les recommandations européennes

International audienc

Emergence d une nouvelle infection opportuniste chez les patients immunodéprimés (l échinococcose alvéolaire)

BESANCON-BU Médecine pharmacie (250562102) / SudocSudocFranceF