Search

Resume Introduction Le syndrome TAFRO est un syndrome inflammatoire systemique proche de la maladie de Castleman, associant, anasarque, fievre, insuffisance renale et/ou fibrose reticulinique et organomegalie. Nous rapportons la premiere observation de vascularite necrosante compliquant un syndrome TAFRO. Observation Une femme de 69 ans presentait amaigrissement, fievre, anasarque, splenomegalie, adenomegalies, oligoanurie et livedo necrotique extensif au decours d’une diarrhee aigue. Les examens biologiques montraient une anemie, une thrombopenie, une insuffisance renale, une hypergammaglobulinemie, un clone lymphocytaire B circulant minime, une hypoparathyroidie et une hypothyroidie auto-immune. La biopsie cutanee montrait une vascularite avec necrose fibrinoide des petits vaisseaux. Le tocilizumab, associe a des perfusions de methylprednisolone, etait inefficace. Les echanges plasmatiques associes au rituximab ont permis la remission initiale, consolidee par 6 cures de rituximab, cyclophosphamide et dexamethasone. Conclusion Le syndrome TAFRO peut s’associer a une vascularite necrosante cutanee. La resistance possible au tocilizumab merite d’etre connue.

BackgroundIdiopathic inflammatory myopathies (IIM) or myositis are a group of rare autoimmune diseases that combine muscle weakness and multi-visceral damage. The discovery of IIM-specific autoantibodies (aAbs) and their associations with clinical phenotypes has improved diagnostic and classification criteria. Faced with the large number of these aAbs, multiplexed techniques have emerged. Among them, the immunodot is simple, rapid, and inexpensive, but has been several times criticized for its lack of specificity.ObjectivesOur objective was to evaluate the current interpretation criteria of the D-Tek immunodot and to propose new interpretation rules based on clinical criteria in order to improve its reliability.MethodsWere included in this retrospective study patients tested positive result on the semi-quantitative myositis/synthetase immunodots at manufacturer threshold (≥ 5 UA), for at least one of the 15 aAbs: anti-SRP, anti-NXP2, anti-TIF1, anti-SAE (1 and 2), anti-Mi2, anti-MDA5, anti-Jo1, anti-PL7, anti-PL12, anti-EJ, anti-OJ, anti-KS, anti-ZO, anti-HA. Specificity of the immunodots was further evaluated using 60 healthy and anonymous subjects (French blood bank, Toulouse, France). The clinical diagnosis and sub-classification retained by the clinician in charge of the patient was used as a reference for attribution to the myositis/non-myositis group and subgroups. For the myositis group, 7 subgroups were considered: immune-mediated necrotizing myopathy (n=4); dermatomyositis (n=66); anti-synthetase syndrome (n=36); inclusion body myositis (n=1); overlap myositis with another connective tissue disease (n=7); polymyositis (n=8); and unclassified myositis (n=6). For the non-myositis group, patients were subdivided in 4 subgroups: autoimmune or inflammatory diseases (n=72); isolated and diffuse interstitial lung disease (n=26) not associated with other myositis criteria; other non-inflammatory myopathies (n=8) including genetic, metabolic, and toxic myopathies; and other diseases (n=36). The immunodot interpretation thresholds were evaluated both in relation to the manufacturer’s threshold, and by considering the phenotypes and clinical diagnoses using a ROC method (Youden’s index).ResultsAmong 270 patients included between 01/07/2016 and 30/06/2020, 128 (47%) were classified as myositis (median age 58 years, 60% women, 52% DM and 28% AS) and 142 (53%) in non-myositis. Among the 15 aAbs analyzed, none were detected in the healthy control group but they were represented in both myositis and non-myositis group. Among them only 2 (anti-Jo1, anti-Mi2) predominate in the myositis group, and 1 (anti-TIF1) in the non-myositis group (Fisher’s test). As quantitative values were found different for 5 aAbs (Mann Whitney test), a clinical threshold was calculated to discriminate myositis from non-myositis groups (ROC curve) allowing to determine an odds ratio (OR). Accordingly, 4/15 (%) aAbs were found associated with myositis: anti-SRP (at 28UA: OR=3.24 95% CI [1.01-10.46], p=0.048), anti-MDA5 (at 15UA: OR=4.36; 95% CI [1.19-15.99], p=0.048), anti-Mi2 (at 5UA: OR =3.24; 95% CI [1.01-10.46], p=0.026), anti-Jo1 (at 5UA: OR= 12.20; 95% CI [2.78-53.52], pConclusionIn this retrospective work, despite missing data, the clinical phenotypes of myositis patients and their distribution according to aAbs were comparable to those in the literature. Our study confirms the lack of specificity of D-tek immunodots for IIM-specific aAbs and allows establishing new thresholds improving their performance. Our results should encourage medical biologists to establish local rules of interpretation and reinforce the interest of the discussion between Clinicians and Biologists around the interpretation of these immunodots.Figure 1.A: Odd Ratio (OR) evaluated with the manufacturer threshold ≥ 5 and B: OR using Youden’s index (clinical threshold).Disclosure of InterestsNone declared

BackgroundSystemic sclerosis (SSc) is reported to be a secondary cause of cryoglobulinemia as well as cryofibrinogenemia. However, prevalence, clinical implication and associated pronostic of cryoprecipitates in SSc are unknown.ObjectivesTo describe the prevalence, the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia associated with systemic sclerosis.MethodsWe included all adult (≥18 years) consecutive SSc patients from the Systemic Scleroderma Toulouse Cohort (SSTC) [1] for whom a cryoglobulin and/or cryofibrinogen measurement was carried out at the immunology laboratory of the Toulouse University Hospital between June 1, 2005 and May 31, 2018 and at least one follow-up visit. We compared SSc-patients characteristics’ with and without cryoglobulinemia > 50 mg/l and with and without cryofibrinogenemia. Survival analysis based on presence of cryoglobulin cryofibrinogen was performed using the Kaplan-Meier method. Univariable and multivariable Cox proportional hazards models (ascending step-by-step method) were used to determine baseline variables associated with cryoglobulin or cryofibrinogen presence.Results166 patients were included in the study. 74.6% of patients were women, with a average age at diagnosis of 51.2 years-old. 24% were diffuse cutaneous subtypes and 71.6% limited cutaneous subtypes. Anti-centromere and anti-Scl70 were respectively positive in 44.5% and 21.6% of cases. All these patients were assessed for cryoglobulin detection and 75 cryofibrinogen detection in serum. 43.3% had a cryoglobulinemia >50 mg/l. 46.6% had cryofibrinogenemia. Patients with cryoglobulinemia >50 mg had more cardiac diastolic involvement (22.8% vs. 5.1% p=0.0395). In the multivariate analysis, diastolic involvement (HR=6.23; p=0.0331) was an independent predictor of cryoglobulin >50 mg/l. Survival at 10 years was better for patients with cryoglobulinemia >50 mg/l (log-rank 0.0363) (Figure 1). Survival at 5 and 10 years was 97.6% and 88.8% respectively in patients with cryoglobulinemia >50 mg/l versus 91.9% and 78.4% in patients with cryoglobulinemia 50 mg/l was negatively associated with mortality (HR: 0.09; p=0.03). The presence of cryofibrinogenemia was not associated with any clinical, biological or morphological features. In the multivariate analysis, no variable was predictive of the presence of cryofibrinogenemia in patients with SSc. The presence of cryofibrinogenemia had no influence on the mortality of these patients.ConclusionIn SSc patients, the presence of cryoglobulin >50 mg/l is an independent predictive factor of cardiac diastolic involvement and is associated with a better survival. However, cryofibrinogenemia does not influence clinical phenotype or impact mortality in SSc patients.References[1]De Almeida Chaves S, Porel T, Mounié M, Alric L, Astudillo L et al. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality. Arthritis Res Ther. 2021 Dec 7;23(1):295. doi: 10.1186/s13075-021-02672-y.Disclosure of InterestsNone declared

BackgroundThe prevalence of cardiac involvement in systemic sclerosis (SSc) varies in the literature between 3% and 44% and represents a leading cause of mortality in this disease. The incidence of severe cardiac involvement and the factors associated with the occurrence of severe cardiac involvement are not known in the literature.ObjectivesThe objective of this study was to evaluate the incidence, prognosis and factors associated with the occurrence of severe cardiac involvement during SSc course.MethodsWe conducted a retrospective, bi-centric study from January 1, 1966 to December 31, 2018. The patients included had a diagnosis of SS according to the ACR/EULAR 2013 criteria. The primary endpoint was the occurrence of severe cardiac involvement. Cardiac involvement was defined by the presence of at least one of the following elements: death of cardiovascular origin, left ventricular ejection fraction less than or equal to 50%, abnormality of at least 3 measurement parameters of diastolic function, global longitudinal strain less than or equal to 18 in absolute value, ventricular tachycardia, ventricular extrasystoles requiring intervention or elevated troponin. Patients with associated myositis and whose only criterion for cardiac involvement was elevated troponin were not included in the group with cardiac involvement. Severe cardiac involvement was defined by the occurrence of hospitalization for cardiovascular reasons or by death of cardiovascular origin. Univariable and multivariable Cox proportional hazards models were used to determine variables associated with severe cardiac involvement occurrence. Survival analysis was performed using the Kaplan-Meier method with comparisons performed using the log rank test.ResultsFour hundred and fifty-nine patients with SSc were included and were followed for a median of 7.1 years [3.1; 13.3]. The median age of our population was 54 years old. There were 81% of women, 77% of patients had limited cutaneous SSc, 15% diffuse cutaneous SSc and 8% SSc sine scleroderma. Of the 459 patients, 105 (23%) had cardiac involvement and 56 (12%) severe cardiac involvement. The incidence of severe cardiac involvement was 2.42 per 100 patient years. Ninety-six hospitalizations were recorded, including 40 (42%) for acute heart failure, 19 (20%) for arrhythmia, 5 (5%) for acute pericarditis, 6 (6%) for acute myocarditis and 14 (15 %) for coronary artery disease (acute coronary syndrome, myocardial infarction or coronary revascularization). The independent factors associated with severe cardiac involvement in SSc were age over 54 years at SSc-diagnosis (OR = 3.21 95% CI [1.73; 5.95], p < 0.001), the presence of myositis (OR = 5.01 95% CI [1.89; 13.28], p = 0.001), pericardial involvement (OR = 3.79 95% CI [2.05; 7.03]; p < 0.001) or scleroderma renal crisis (OR = 4.72 95% CI [2.05; 10.92], p < 0.001). The survival rate of patients with severe cardiac involvement was 70% at 5 years and 53% at 10 years. Patients with severe cardiac involvement had a mortality risk three times greater than patients without severe cardiac involvement, HR = 3.1 (95% CI [1.7; 5.7], pFigure 1.Survival of patients with severe cardiac involvement of systemic scleroderma. HR: Hazard ratio; 95% CI: 95% Confidence interval; Nb at risk: Number at riskConclusionWe need to focus our clinical attention on diagnosing and manage cardiac involvement in SSc, as severe cardiac involvement is not uncommon and is responsible for a poor prognosis.Disclosure of InterestsNone declared

TAFRO syndrome is a systemic inflammatory syndrome in the spectrum of Castleman's disease, associating thrombocytopenia, anasarca, fever, renal failure and/or reticulin myelofibrosis and organomegaly. Its association with necrotizing cutaneous vasculitis has not yet been reported.A 69-year-old woman presented with weight loss, fever, anasarca, organomegaly, lymphadenopathy, anuria and extensive necrotic livedo occurring after acute diarrhea. Biology showed anemia, thrombocytopenia, renal failure, hypergammaglobulinemia, a circulating B-lymphocyte clone, hypoparathyroidism and autoimmune hypothyroidism. The skin biopsy showed small vessel vasculitis with fibrinoid necrosis. Methylprednisolone infusions associated with tocilizumab were ineffective and the patient became anuric. Rituximab and plasma exchanges associated to corticosteroids allowed remission for 2 months. Combination of rituximab, cyclophosphamide and dexamethasone resulted in a prolonged remission.We report here the first case of severe cutaneous necrotizing vasculitis in a patient suffering from TAFRO syndrome. The possible resistance to tocilizumab should be known.

Les enquetes de la Societe francaise d’endoscopie digestive (SFED), initiees en 1998 et realisees annuellement depuis 2001, ont pour objectif de decrire les caracteristiques des patients beneficiant d’une coloscopie, les conditions de prise en charge, le rendement diagnostique et les eventuelles complications. A partir d’un questionnaire envoye par voie electronique, la pratique de l’endoscopie est evaluee pendant cinq jours consecutifs. Les donnees de l’enquete sont extrapolees sur la population totale des 2 600 gastroenterologues pratiquant des endoscopies digestives, puis comparees aux annees d’exercice precedentes. L’enquete a ete realisee du 16 au 22 janvier 2017. L’enquete SFED 2017 sur l’exercice de la coloscopie en France met en evidence plusieurs points: une participation en hausse, une augmentation du nombre de coloscopies realisees, une amelioration constante de la qualite des coloscopies, liee a plusieurs facteurs: une meilleure prise en charge des patients avant l’examen, une meilleure adaptation des produits de preparation en fonction du profil des patients, l’utilisation plus importante des laxatifs chez les patients constipes avant examen, un meilleur respect des recommandations sauf pour la gestion des traitements anticoagulants avant examen. Les donnees fournies par les enquetes de la SFED representent un outil important dans l’evaluation de l’activite endoscopique en France et sont une image quasi unique en Europe. L’amelioration de la qualite permet d’ameliorer les performances de la coloscopie et montre la pertinence de respecter au mieux les recommandations.

Introduction Le syndrome TAFRO, decrit en 2010, est une forme particuliere de la maladie de Castleman multicentrique sans proliferation HHV8, associant notamment thrombopenie, anasarque, fievre, myelofibrose, insuffisance renale et organomegalie. Son association a une vascularite avec necrose cutanee est inedite. Observation Une femme âgee de 69 ans, caucasienne et ayant presente de nombreux antecedents oncologiques (tumeur carcinoide pulmonaire il y a 20 ans, dysplasie colique de haut grade il y a 4 ans, cancers du poumon, du sein et de l’endometre dans sa fratrie), etait admise en medecine interne pour amaigrissement, fievre a 38,5 °C et dyspnee d’installation progressive depuis trois mois. L’examen clinique montrait un livedo douloureux extensif avec acrocyanose marquee. Ce livedo devenait necrotique a la face interne des cuisses. Une anasarque apparaissait progressivement. Les examens biologiques montraient : anemie microcytaire non hemolytique (8 g/dL), thrombopenie d’aggravation progressive (nadir : 13 G/L), TCA allonge (ratio 1,62) avec anticoagulant circulant de type lupique, creatininemie a 275 umol/L, proteinurie moderee et hematurie microscopique, CRP a 314 mg/L, hypergammaglobulinemie de profil oligoclonal (26 g/L). Un clone lymphocytaire B circulant (CD19+ CD5+ CD23− FMC7+ CD79b− kappa+ ; 1,3 % des lymphocytes ; 19/uL) etait mis en evidence ainsi qu’une cryoglobulinemie de type II (composant monoclonal IgG lambda ; taux : 110 mg/L) avec cryofibrinogenemie moderee (54 mg/L), C3 0,92 g/L, C4 0,15 g/L. On retrouvait egalement une hypoparathyroidie et une hypothyroidie avec anticorps anti-TPO. La serologie VIH, la PCR HHV8, les ANCA et les ACAN etaient normaux. Les taux seriques d’erythropoietine et de VEGF (284 pg/mL) egalement. Les examens radiologiques et scintigraphiques montraient, en plus de l’anasarque, une hepatosplenomegalie et des adenomegalies hypermetaboliques sus- et sous-diaphragmatiques. La biopsie cutanee montrait une vascularite leucocytoclasique avec necrose fibrinoide et des infiltrats lympho-histiocytaires perivasculaires. La biopsie osteo-medullaire montrait une moelle hyperplasique avec dysmyelopoeise. Deux biopsies ganglionnaires, dont une mediastinale de petite taille, ne montraient qu’une inflammation non specifique. La biopsie hepatique montrait une fibrose portale. Le diagnostic de syndrome TAFRO etait pose. Une corticotherapie avec bolus de methylprednisolone etait initiee, totalement inefficace, rapidement associee a du tocilizumab. En l’absence d’efficacite apres 4 jours, etaient realises 4 echanges plasmatiques avec administration de rituximab 375 mg/m2 a j1, j7, puis une fois par mois. Une epuration extra renale etait necessaire pendant trois semaines. L’immunodepression favorisait une aspergillose pulmonaire d’evolution lentement favorable. L’evolution etait marquee apres deux mois, en fin d’intercure du rituximab, par la rechute de la fievre, du syndrome inflammatoire, de l’insuffisance renale et de la thrombopenie. Le traitement etait alors intensifie par 6 cures de rituximab, cyclophosphamide et dexamethasone, sans nouvelle rechute (recul de 6 mois). Aucun cancer n’a ete mis en evidence malgre une recherche approfondie. Discussion Chez cette patiente, les criteres de syndrome TAFRO proposes en 2015 par Masaki et al. sont remplis, avec trois criteres majeurs (fievre, thrombopenie et anasarque) et trois criteres mineurs (organomegalie, insuffisance renale progressive et hyperplasie medullaire). A l’inverse, les criteres de Iwaki et al. ne sont pas remplis en raison de l’absence de preuve anatomopathologique de maladie de Castleman. L’hypothyroidie est frequemment retrouvee dans le syndrome TAFRO, comme dans le syndrome POEMS dont les tableaux cliniques sont parfois chevauchants. La physiopathologie n’est pas connue. La resistance du TAFRO au tocilizumab comme l’efficacite du rituximab ont deja ete rapportes dans la litterature. L’anakinra, le bortezomib et des immunosuppresseurs conventionnels ont egalement ete decrits comme efficaces. L’association de ce syndrome avec une vascularite necrosante cutanee est inedite. A l’inverse, elle a ete rapportee plusieurs fois dans la maladie de Castleman. On peut ici evoquer le role possible de la cryoglobulinemie et du cryofibrinogene. Conclusion Nous rapportons ici le premier cas de syndrome TAFRO associe a une vascularite necrosante cutanee. L’interet de la recherche d’un clone circulant, meme minime, et la resistance possible au tocilizumab malgre le caractere tres inflammatoire meritent d’etre connus.

Introduction Le syndrome TEMPI est une entite rare (16 cas rapportes) associant polyglobulie a taux serique d’erythropoietine (EPO) eleve, telangiectasies cutanees, gammapathie monoclonale et parfois, epanchements perirenaux et shunts intrapulmonaires. Nous rapportons ici un nouveau cas. Observation Une femme de 51 ans, caucasienne, menopausee depuis 6 ans, etait adressee en medecine interne pour polyglobulie avec erythermalgie depuis deux ans. Elle avait presente dans la meme periode plusieurs episodes de goutte articulaire, deux episodes d’insuffisance renale aigue transitoires avec lombalgie bilaterale (sans etiologie retrouvee) et de frequentes hemorragies sous-conjonctivales. L’examen physique revelait la presence de nombreuses telangiectasies predominant au niveau du tronc et d’une erythrose palmoplantaire. Il n’y avait pas de signe de cirrhose clinique, biologique ni morphologique. Les examens biologiques montraient une polyglobulie JAK2 negative (hemoglobinemie [Hb] 19,4 g/dL, hematocrite 60 %), un taux d’EPO tres eleve (525 mUI/mL ; N Les epreuves fonctionnelles respiratoires avec gazometrie et l’oxymetrie nocturne eliminaient une hypoxemie chronique. L’IRM cerebrale, le scanner thoraco-abdomino-pelvien et la tomographie par emission de positons eliminaient une tumeur secretant de l’EPO renale, hepatique ou de type hemangiome cerebelleux. L’exploration de l’axe hormonal gonadotrope etait normale. La recherche de shunt intrapulmonaire ou d’epanchement perirenal etait negative. Le traitement initial associait saignees, acide acetylsalicylique a dose antiagregante et febuxostat. Celui-ci a permis un controle de la polyglobulie (Hb 15 g/dL malgre reticulocytose a 210 G/L) et induit une carence martiale (ferritine Le diagnostic de syndrome TEMPI, pose secondairement, a fait modifier le traitement pour 8 cycles de bortezomib, cyclophosphamide et dexamethasone, permettant l’arret des saignees et la normalisation de l’electrophorese des proteines. Discussion Le syndrome Telangiectasia, Erythrocytosis with elevated erythropoeitin, Monoclonal gammapathy, Perinephric fluid collections, Intrapulmonary shunting (TEMPI) a ete decrit en 2011 par Sykes et al. chez des patients âges principalement de 40 a 60 ans. Seize observations ont ete rapportees a ce jour. La gammapathie monoclonale etait le plus frequemment de type IgG kappa (7 cas sur 13). Les taux seriques d’EPO sont toujours superieurs a 78 mUI/mL et plus souvent a 5000 mUI/mL. Les epanchements perirenaux ont ete rapportes dans 13 cas et les shunts intrapulmonaires, pouvant induire une insuffisance respiratoire, dans 9 cas. La goutte, non rapportee a ce jour dans le syndrome TEMPI, est une complication habituelle des polyglobulies essentielles. Les insuffisances renales transitoires pourraient etre liees a une precipitation urinaire d’acide urique. Les hemorragies sous-conjonctivales n’ont pas ete rapportees dans ce syndrome. Plusieurs traitements antiplasmocytaires ont ete utilises dans le syndrome TEMPI, comme le bortezomib, le daratumumab, le lenalidomide et l’autogreffe de cellules souches. Tous montrent une amelioration spectaculaire de la plupart des manifestations, c’est pourquoi ce syndrome est classe parmi les gammapathies monoclonales de signification clinique (MGCS). Conclusion Du fait de traitements specifiques, le syndrome TEMPI merite d’etre connu des cliniciens. L’originalite de notre observation est la presence d’une goutte compliquant la polyglobulie ainsi que la caracterisation du clone plasmocytaire. Il faut evoquer un syndrome TEMPI devant l’association d’une polyglobulie a EPO elevee, de telangiectasies et d’une gammapathie monoclonale.

Surgery for low rectal cancer remains a challenge when a standard laparoscopic approach is used. Transanal endoscopic total mesorectal excision (TME) has been shown to be feasible and to be associated with a low conversion rate. Combining the transanal and transabdominal single-port approaches (with an abdominal single port implanted in the future stoma and extraction site) could allow TME with minimal wound trauma, low morbidity, and faster recovery. The aim of the current study was to assess the short- and mid-term results of this technique. We conducted a prospective single-centre study of consecutive patients presenting with low rectal cancer requiring a conservative proctectomy with a manual coloanal anastomosis between January 2012 and April 2015. During the study period, 41 patients were recruited. Conversion to open surgery was required in only one patient (2.4%). The median operating time was 358.5 min (range 300–600 min). Partial intersphincteric resection was necessary for 15 patients (36.6%). The specimens were mostly extracted via the abdominal access (n = 34) without wound complications. The mean number of lymph nodes harvested was 12.7 (range 6–24 lymph nodes). Specimens were graded as complete (n = 31) or nearly complete (n = 10) in all of the patients, and the circumferential resection margin positivity was 4.9%. Intraoperative morbidity rate was 4.9%, and the 30-day morbidity rate was 24.4% (n = 10). Sixty per cent (n = 6) of the patients with 30-day morbidity were Dindo I–II. At a median follow-up of 29 months, overall and disease-free survival rates were 97.5 and 80.5%, respectively. The stoma-free survival rate was 95.1%. Combining an endoscopic transanal TME and a single laparoscopic ileostomy-site proctectomy is a promising minimally invasive approach for the treatment of low rectal cancer.

Introduction La cholecystite aigue et l’arterite temporale sont rares au cours de la granulomatose eosinophilique avec polyangeite (EGPA). Observation Un patient de 64 ans aux antecedents d’appendicectomie, hernie hiatale et polypectomie presente une toux chronique avec une tracheo-broncho-malacie a l’endoscopie bronchique. La toux cede initialement sous corticoides et antibiotherapie. Trois mois plus tard, il est hospitalise pour toux, arthromyalgies diffuses, oedemes prurigineux et petechies des membres inferieurs. Il existe une hypereosinophilie a 7250/mm 3 . Les serologies parasitaires sont negatives. Le traitement par corticotherapie a 1 mg/kg/j pendant 15 jours et antiparasitaire d’epreuve est inefficace. Il est adresse en medecine interne pour toux et dysesthesies des pieds. L’examen retrouve une douleur en hypochondre droit, des arthromyalgies, des crepitants des bases. Il decrit des cephalees avec claudication de la mâchoire. Est apparue une multinevrite avec atteinte des nerfs median droit et sciatique poplite interne gauche confirmee par l’electromyographie. Il persiste une hypereosinophilie a 2880 elements/mm 3 et CRP a 134 mg/L malgre la corticotherapie. L’echographie montre une vesicule biliaire alithiasique avec un epaississement et un dedoublement parietal. Le bilan hepatique est normal. Un traitement medical initial par antibiotherapie est decide. Une biopsie cutanee des lesions purpuriques montre une necrose fibrinoide de la paroi capillaire avec infiltrats eosinophiles. Les ANCA sont positifs au titre de 800, de type cANCA, anti-MPO a 3,7 AI. La biopsie d’artere temporale montre une arterite necrosante segmentaire et focale sans cellule geante et nombreux polynucleaires eosinophiles. Il n’y a pas d’atteinte renale ni cardiaque. Une EGPA est diagnostiquee. Un traitement par bolus de prednisone a 500 mg/j et cyclophosphamide a 600 mg/kg sont realises, permettant la disparition rapide de la toux, des cephalees avec claudication des mâchoires, des douleurs abdominales, de l’hypereosinophilie et du syndrome inflammatoire. L’etat du patient s’aggrave brutalement avec recidive violente de la douleur abdominale et defense generalisee. Le scanner abdominal et la cœlioscopie confirment une peritonite par perforation de la vesicule biliaire. L’anatomopathologie revele une cholecystite aigue necrosante a eosinophiles avec images de vascularite. Les suites operatoires sont simples, le traitement par corticoides et cyclophosphamide est maintenu. Discussion Ce patient presente une EGPA a atteintes pulmonaire, neurologique peripherique, cutanee et digestive et une arterite temporale necrosante symptomatique. Il s’agit d’une forme severe avec un Five-Factor Score a 1, resistante a la corticotherapie a 2 mg/kg/j. Il existe plusieurs cas decrits d’arterite temporale necrosante sans cellules geantes dans l’EGPA. Serrano-Castro PJ et al. decrivent 5 cas [1] . Il s’agit souvent de patients ayant des symptomes de maladie de Horton et une hypereosinophilie, avec decouverte d’EGPA a l’anatomopathologie. Endo T et al. evoquent l’existence d’une entite clinique distincte d’EGPA avec atteinte des vaisseaux de moyen calibre [2] . Mais cette atteinte pourrait aussi constituer un facteur pronostique d’EGPA. La cholecystite aigue dans l’EGPA est rarement decrite, de diagnostic souvent echographique, sans consensus therapeutique medical ou chirurgical. L’atteinte digestive fait partie des facteurs pronostiques de Five-Factor Score avec indication a une immunosuppression par cyclophosphamide ou rituximab [3] . Dans notre cas, cette complication digestive etait grave avec peritonite biliaire, survenue sous fortes doses de corticoides et cyclophosphamide. On peut se demander si la corticotherapie a favorise la perforation ou si le traitement n’a pas ete suffisant pour stopper le processus de necrose fibrinoide de la paroi vesiculaire. La cholecystectomie precoce, qui avait ete discutee, aurait evite la perforation vesiculaire et pourrait etre proposee aux patients symptomatiques. Inversement, le risque de complication peroperatoire en phase aigue d’une vascularite n’est pas connu, notamment sur le plan infectieux. Conclusion Nous rapportons un cas d’EGPA avec deux atteintes rares : l’arterite temporale necrosante symptomatique et la cholecystite necrosante perforee. L’existence d’une arterite temporale dans l’EGPA traduit une atteinte des vaisseaux de moyen calibre qui ne fait pas partie des criteres pronostiques classiques. Elle etait neanmoins associee ici a des atteintes graves. La prise en charge des cholecystites de l’EGPA par chirurgie precoce ou apres traitement medical doit etre discutee au cas par cas au regard du risque de perforation avec complications potentiellement severes.

We studied clinical outcome and clinico-virological factors associated with hepatitis B virus reactivation (HBV-R) following cancer treatment in hepatitis B virus surface antigen (HBsAg)-negative/anti-hepatitis B core antibodies (anti-HBcAb)-positive patients. Between 11/2003 and 12/2005, HBV-R occurred in 7/84 HBsAg-negative/anti-HBcAb-positive patients treated for haematological or solid cancer. Virological factors including HBV genotype, core promoter, precore, and HBsAg genotypic and amino acid (aa) patterns were studied. Patients presenting with reactivation were men, had an hepatitis B virus surface antibody (HBsAb) titre 1 line of chemotherapy (CT) significantly more frequently than controls. All were treated for haematological cancer, 3/7 received haematopoietic stem cell transplantation (HSCT), and 4/7 received rituximab. Using multivariate analysis, receiving >1 line of CT was an independent risk factor for HBV-R. Fatal outcome occurred in 3/7 patients (despite lamivudine therapy in two), whereas 2/4 survivors had an HBsAg seroconversion. HBV-R involved non-A HBV genotypes and core promoter and/or precore HBV mutants in all cases. Mutations known to impair HBsAg antigenicity were detected in HBV DNA from all seven patients. HBV DNA could be retrospectively detected in two patients prior cancer treatment and despite HBsAg negativity. HBV-R is a concern in HBsAg-negative/anti-HBcAb-positive patients undergoing cancer therapy, especially in males presenting with haematological cancer, a low anti-HBsAb titre and more than one chemotherapeutic agent. HBV DNA testing is mandatory to improve diagnosis and management of HBV-R in these patients. The role of specific therapies such as rituximab or HSCT as well as of HBV aa variability deserves further studies.

L’echoendoscopie a permis une meilleure identification de l’extension parietale et ganglionnaire des tumeurs du tractus digestif et de la sphere bilio-pancreatique.

Background and study aims The aim of this study was to evaluate the efficacy and outcomes of treatment by endoscopic mucosal resection (EMR) of patients with high-grade dysplasia (HGD) or carcinoma. Patients and methods Between January 1995 and January 2002, 50 patients (35 men, 15 women) were treated by EMR for 52 sessile polyps. The median size of the polyps was 27.5 mm (range 10-60). The "lift and cut" EMR technique was used. If the lesion was poorly differentiated or infiltrated the muscularis mucosae to more than 1000 microm, the patient was referred for colectomy. In the other cases, follow-up was proposed. Results Complications occurred in 9.6 % of cases and were always treated conservatively. The rate of endoscopically complete resection was judged to be 98.1 %. Argon plasma coagulation was applied to the margins of the lesion in 21.6 % of cases. Histological examination showed 38 HGDs and 14 carcinomas. Seven patients had a lesion reaching the deep or lateral margin; four were referred for surgery; two patients for whom surgery would have been high risk were followed up, and both developed local recurrence; and one patient was followed up, without recurrence, because infiltration was less than 1000 microm. A total of 43 patients were followed up after complete excision. Two patients died during follow-up; neither death could be reliably attributed to colorectal carcinoma. Seven patients were lost during the follow-up. For 34 patients, information from a mean follow-up of 17.3 months (6 - 57) was available and recurrence was observed in five cases (15 %). Conclusions EMR appears to be a safe and efficient treatment of HGD and early colorectal cancer. However, correct analysis of submucosal infiltration is essential to assess the completeness of the resection.

Le developpement de l’echoendoscopie (EE) a permis de mieux preciser l’extension parietale et ganglionnaire des tumeurs gastrointestinales et pancreatiques. Neanmoins, l’EE ne peut affirmer le caractere malin ou benin d’adenopathies, de masses pancreatiques ou de compressions extrinseques du tube digestif. Le developpement depuis plus de 15 ans de l’echoendoscopie sectorielle lineaire, a permis de realiser des biopsies guidees de telles lesions [1,2, 5, 6, 9, 10, 16–18].

La ponction guidee par echoendoscopic est frequemment realisee aujourd’hui pour obtenir une cytologie ou une histologie a partir des masses mediastinales ou des adenopathies des tumeurs pancreatiques, qu’elles soient solides ou kystiques.

We report the case of a 60-year-old-woman with a myeloma who was hospitalized with a cholestasis. An endoscopic retrograde cholangiopancreatography was scheduled under general anaesthesia with oral intubation. As the biliary prothesis was placed an air embolism happened. The symptomatic treatment allowed a complete recovery. This complication is rare. The pathophysiology is not well known, we discuss the possible mechanisms.

Summary Introduction The accuracy of endoscopic ultrasound (EUS) for the diagnosis and staging of pancreatic ductal adenocarcinoma (PDA) has been confirmed. Chemo-radiotherpay (CRT) induces tumor changes which can limit the accuracy of EUS. The aim of our study was to analyze the efficacy of EUS following neoadjuvant CRT comparing findings with the pathology results. Patients and methods From November 1996 to October 2003, 45 patients with histologically proven and EUS-staged PDA were treated with neo-adjuvant CRT and radical surgery. All were restaged before surgery using both EUS and computed tomography. Fifteen patients were found to have developed distant metastases. Thirty patients finally underwent pancreaticoduodenectomy (N = 24) or distal pancreatectomy (N = 6). Results Following CRT, tumor stage was correctly assessed in 12 patients (40%). The most frequent misinterpretation was overestimation of tumor size (N = 13, 43.3%). Locoregional vascular invasion of veins was suspected by EUS in 13 patients (43.3%) but surgical findings and the histological examination were both negative. Node status was correctly assessed in 27 patients (90%) but nodal involvement was found on the histological specimen in only 3 patients. Conclusion Preoperative EUS after neoadjuvant CRT for PDA does not enable reliable definitive selection of patients for surgery, probably due to radiation-induced pancreatic changes.

L’echoendoscopie (EE) constitue un outil indispensable pour le bilan d’extension des neoplasies gastro-intestinales et pulmonaires. L’etude du parenchyme hepatique peut modifier la prise charge therapeutique par la decouverte de lesions infracentimetriques non visualisees sur les examens radiologiques standards. Si le diagnostic de telles lesions implique une modification therapeutique, une confirmation histologique est alors strictement necessaire par la realisation d’une ponction echoguidee. L’opportunite d’analyser de facon tres precise la partie posterieure du lobe gauche ainsi que le hile hepatique permet la realisation de prelevements de tumeurs hepatiques primitives ou secondaires d’abord transcutane difficile, avec une morbidite faible. Certains auteurs ont evoque la possibilite de realiser des biopsies hepatiques sous EE en cas de contre-indication a la ponction percutanee (ascite, troubles de l’hemostase). Ceci doit etre evalue sur des effectifs plus importants.

Le developpement des techniques de traitement endoscopique des cancers superficiels du tube digestif (mucosectomie endoscopique) impose, avant de mettre en route de tels traitements, d’avoir un bilan loco-regional (parietal et ganglionnaire) aussi precis que possible. En effet, la mucosectomie endoscopique (ME) ne peut s’appliquer qu’a des tumeurs n’ayant qu’un faible risque de diffusion ganglionnaire; or ce risque est tres lie a l’infiltration locale de la lesion et seules les lesions limitees a la muqueuse voire au premier tiers de la sous-muqueuse (sm1) pourront beneficier d’un traitement endoscopique. La question posee est de savoir si l’echoendoscopie (EE) peut visualiser une infiltration de la sous-muqueuse (c’est-a-dire le franchissement de lamuscularis mucosae) de maniere fiable. Par ailleurs, la place de l’EE est a moduler en fonction de l’organe en cause (œsophage, estomac, colon et rectum). The development of the techniques of endoscopic treatment of superficial cancers of the digestive tract (endoscopic mucosectomy) requires before starting a loco-regional assessment (parietal and lymph nodes) as precise as possible. Indeed, the endoscopie mucosectomy (ME) can apply only to tumours with a low risk of lymph node invasion, but this risk is quite related to the local infiltration of the lesion and only the lesions limited to the mucous membrane or to the first third of the submucosa (sml) will be treated by EMR. The question is to know whether echoendoscopy (EUS) can visualize an infiltration of the submucosa (i.e. crossing the muscularis mucosae) in a reliable way. In addition, the place of EE is to be modulated according to the organ concerned (oesophagus, stomach, colon and rectum).