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Postpartum ovarian vein thrombosis (POVT) is a rare but serious postpartum complication that can be life-threatening due to its embolic and septic risks. The clinical and paraclinical diagnosis is difficult because of the non-specific signs and the absence of a gold standard for imaging. There is no consensus in the literature on the treatment and follow-up of these patients. The primary objective was to specify the clinical and paraclinical signs suggestive of POVT in order to improve the diagnostic delay. The secondary objectives were to describe the extent of POVT and the proposed immediate therapeutic management.This was a 10-year retrospective study in a type III maternity hospital, from January 2010 to December 2019, where all patients with an imaging-confirmed diagnosis of POVT were included. We analysed the clinical and paraclinical data and the follow-up of the patients.We included 9 patients with a diagnostic confirmation by imaging. The mean time from first symptoms to diagnosis was 3.3 days (±3.5 days), and only 2 patients (22.2 %) had been diagnosed with POVT before imaging. All patients received curative anticoagulation and 77.8 % (n=7) received antibiotic therapy for POVT. Two patients had a complicated form, 1 with a pulmonary embolism and 1 with a urinary tract compression requiring a urinary diversion with a double J catheter. Five patients (55.6 %) had a thrombophilia check-up.The diagnosis of POVT is difficult and needs to be evoked in front of a painful symptomatology or a fever in postpartum. It can be made by ultrasound, but the injected CT scan specifying the specific search for a POVT remains the imaging examination of choice in order to confirm the diagnosis and eliminate differential diagnoses. Under curative anticoagulation and broad-spectrum antibiotic therapy, the clinical course is generally very favourable. A consultation with an internist makes it possible to define instructions for a subsequent pregnancy.

Introduction VTE‐BLEED is a validated score for identification of patients at increased risk of major bleeding during extended anticoagulation for venous thromboembolism (VTE). It is unknown whether VTE‐BLEED high‐risk patients also have an increased risk for recurrent VTE, which would limit the potential usefulness of the score. Methods This was a post hoc analysis of the randomized, double‐blind, placebo‐controlled PADIS‐PE trial that randomized patients with a first unprovoked pulmonary embolism (PE) initially treated during 6 months to receive an additional 18‐month of warfarin vs. placebo. The primary outcome of this analysis was recurrent VTE during 2‐year follow‐up after anticoagulant discontinuation, that is, after the initial 6‐month treatment in the placebo arm and after 24 months of anticoagulation in the active treatment arm. This rate, adjusted for study treatment allocation, was compared between patients in the high‐ vs. low‐risk VTE‐BLEED group. Results In complete case analysis (n = 308; 82.4% of total population), 89 (28.9%) patients were classified as high risk; 44 VTE events occurred after anticoagulant discontinuation during 668 patient‐years. The cumulative incidence of recurrent VTE was 16.4% (95% confidence interval [CI], 10.0%‐26.1%; 14 events) and 14.6% (95% CI, 10.4%‐20.3%; 30 events) in the high‐risk and low‐risk VTE‐BLEED groups, respectively, for an adjusted hazard ratio of 1.16 (95% CI, 0.62‐2.19). Conclusion In this study, patients with unprovoked PE classified at high risk of major bleeding by VTE‐BLEED did not have a higher incidence of recurrent VTE after cessation of anticoagulant therapy, supporting the potential yield of the score for making management decisions on the optimal duration of anticoagulant therapy.

Resume Introduction Le but de ce travail etait de decrire les particularites de la sclerodermie systemique (ScS) quand le diagnostic est fait apres l’âge de 70 ans. Patients et methodes Il s’agit d’une etude retrospective des patients âges de plus de 70 ans au moment du diagnostic de ScS, suivis dans un service de medecine interne entre 2000 et 2015. Les co-morbidites et les caracteristiques demographiques et cliniques ont ete analysees, ainsi que la survie a 1, 2 et 3 ans. Resultats Sur les 246 cas de ScS de notre cohorte, 27 (11 %) ont ete inclus (89 % de femmes, âges de 78,3 ± 4,5 ans). Une neoplasie synchrone etait notee chez 3 patients. La ScS etait majoritairement cutanee limitee (24/27), avec des telangiectasies (63 %), un reflux gastro-oesophagien (59 %), des ulceres digitaux (22 %), et associee a un anticorps anti-centromeres (69 % des cas). L’atteinte interstitielle pulmonaire etait peu frequente (29 %). Une hypertension arterielle pulmonaire (HTAP) etait suspectee des le diagnostic de ScS dans 14 cas (52 %), mais seuls 5 patients ont eu un catheterisme cardiaque, montrant une HTAP severe dans 3 cas. La survie etait de 85,2 % a 1 an et 66,7 % a 3 ans ; elle etait moins bonne en cas de suspicion d’HTAP (respectivement 78,6 % et 57,1 %). Conclusion Les cas de ScS diagnostiquee apres 70 ans sont majoritairement des formes cutanees limitees. Une suspicion d’HTAP est frequente, representant plus d’un cas sur 2, et l’HTAP est un mode d’entree dans la maladie a cet âge. L’association a un cancer synchrone est possible. La survie est mediocre.

Venous thromboembolism (VTE) is a frequent and serious complication in cancer patients, and the second leading cause of death in this setting. Cancer patients are also more likely to present recurrent VTE and major bleeding while taking anticoagulants. Management of VTE in these patients is always challenging and remains suboptimal worldwide. In 2013, the International Initiative on Thrombosis and Cancer (ITAC-CME) released international guidelines for the treatment and prophylaxis of VTE and central venous catheter-associated thrombosis, based on a systematic review of the literature ranked according to the Grading of Recommendations Assessment, Development, and Evaluation scale. An update of these ITAC-CME consensus guidelines, including the use of direct oral anticoagulants, was recently published. In this review, we summarize these updated guidelines. Better adherence to the international guidelines, involving an adequate educational and active implementation strategies, will substantially decrease the burden of VTE and allow to increase survival in cancer patients.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by the triad of nose bleeding, telangiectasia and familial heredity.We report the case of a patient who had idiopathic venous cerebral thrombosis complicated by a cerebral infarction treated with warfarin. In the context of a psoas hematoma by warfarine overdose and immobilization, the patient had deep vein thrombosis of the left lower limb with pulmonary embolism revealing a pulmonary arteriovenous malformation. After a reexamination, the patient clinical phenotype of HHT was confirmed genetically. The patient was treated with rivaroxaban allowing clinical improvement and partial recanalization of all thrombosis after six months. Thrombotic overisk has already been studied in HHT patients but the use of anticoagulants is at higher risk in these patients. However this patient experienced no adverse event with rivaroxaban.This is the first case described of cerebral venous thrombosis treated with rivaroxaban revealing an HHT.

Cholesterol crystal embolism is a systemic pathology associated with diffuse atherosclerosis. Pathophysiology corresponds to tissue necro-inflammation secondary to arteriolar occlusion associated with microembolism from atherosclerotic plaques of large diameter arteries. The clinical presentation is heterogeneous and polymorphic. Multiple organs may be the targets, but preferential damage is skin, kidneys and digestive system. It is a serious pathology, underdiagnosed, with a poor prognosis. The risk factors for developing the disease remain the same risk factors as atheroma. The factors favouring migration of microembolism remain mainly vascular interventional procedures; easy to diagnose, they oppose spontaneous embolic migrations or secondary to the introduction of antithrombotic treatment, whose diagnosis is more difficult and the prognosis more severe. The diagnosis of the disease remains mostly a diagnosis of elimination and often refers to a bundle of clinical, biological, morphological and histologic arguments. The treatment is poorly codified and the subject of few publications. It will favour both symptomatic treatment (and mainly that of pain) and complications (high blood pressure, renal insufficiency). The aetiological support remains less consensual. The treatment of atherosclerotic plaques consists, of course, in the correction of classical cardiovascular risk factors, the introduction of a statin. It will be discussed in the implementation of surgery or angioplasty to exclude potentially responsible atherosclerotic lesions. Eviction of antithrombotic therapy should be considered in terms of the benefit-risk balance, but often in favour of maintaining it. Finally, other treatments may be proposed in a case-by-case basis, such as oral or intravenous corticosteroid therapy, colchicine or LDL aphaeresis.

Many factors can contribute to the risk of venous thrombosis observed in hemolytic diseases. Some mechanisms are related to hemolysis by itself, while others seem more specific to each disease. Despite recent advances in the quantification of this risk and in understanding its physiopathology, the association of hemolysis with venous thrombosis is often unknown. The purpose of this general review is to clarify the main pro-thrombotic mechanisms during hemolysis and to synthesize the clinical data currently available. We will focus on the main types of hemolytic pathologies encountered in current practice, namely paroxysmal nocturnal hemoglobinuria, hemoglobinopathies, auto-immune hemolytic anemia and thrombotic microangiopathies.

A large outbreak of OXA-48 carbapenemase-producing Klebsiella pneumoniae at Nantes University Hospital was investigated. The index case had no history of travel or hospitalization abroad and had been hospitalized in the internal medicine department for more than one month when the epidemic strain was isolated from a urine sample in June 2013. Seventy-two secondary cases were detected by weekly screening for gastrointestinal colonization during the two phases of the outbreak from June to October 2013 (33 cases) and from November 2013 to August 2014 (39 cases). Spread of the epidemic strain was attributed to the proximity of, and staff movement between, the infectious diseases (32 cases) and the internal medicine (26 cases) departments; 14 secondary cases were also observed in the renal transplant department following the transfer of an exposed patient from the infectious diseases department. Most of the patients (90%) were colonized and no death was linked to the epidemic strain. More than 3000 contact patients were reviewed and 6000 rectal swabs were performed. Initial control measures failed to control the outbreak owing to the late detection of the index case. The late implementation of three successive cohort units, the large number of transfers between wards, and the frequent readmission of cases contributed to the incomplete success of control measures.

To describe the clinical features and etiologies of upper limb venous thrombosis (ULVT).All patients with a clinically suspected ULVT, were included retrospectively from January to December 2016. Diagnosis of ULVT was based on doppler-ultrasonography. Clinical features, topography and symptomatic pulmonary embolism (PE) were analyzed. The sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative value (NPV) of clinical symptoms leading to ULVT suspicion were estimated by comparing patients with and without ULVT.Among 488 patients with a suspected ULVT, 160 were diagnosed with ULVT, including, 80 with deep venous thrombosis (DVT) and 80 with superficial venous thrombosis (SVT). Symptomatic PE was found in 2.5 % of cases (n=4). None of the clinical symptoms of ULVT had a sensitivity greater than 40 %. For DVT, presence of superior vena cava syndrome had a 100 % PPV, 71.6 % NPV and 100 % Sp. For SVT, the presence of an cord-like induration had a 85.7 % PPV, 75.3 % NPV and 98.4 % Sp. An endovenous device was present in 87.5 % of DVT and 97.5 % of SVT cases. Malignant hemopathy was found in 43.8 % and 31.3 % of cases of DVT and SVT, respectively. Sepsis and solid neoplasia were present in 25 % and 15 % of cases of ULVT, respectively. Peripherally inserted central catheter or implantable sites were present in 40 % and 17.5 % of DVT patients. No solid neoplasia, hematological malignancy or thrombophilia were diagnosed in patients with ULVT.An endovenous device was involved in 92.5 % of cases of ULVT. The prevalence of symptomatic PE was low. Hematological malignancies, sepsis and neoplasia were the most common conditions present in patients with ULVT.

Venous thromboembolism (VTE) is a frequent and serious complication in cancer patients, and the second leading cause of death in this setting. Cancer patients are also more likely to present recurrent VTE and major bleeding while taking anticoagulants. Management of VTE in these patients is always challenging and remains suboptimal worldwide. In 2013, the International Initiative on Thrombosis and Cancer (ITAC-CME) released international guidelines for the treatment and prophylaxis of VTE and central venous catheter-associated thrombosis, based on a systematic review of the literature ranked according to the Grading of Recommendations Assessment, Development, and Evaluation scale. An update of these ITAC-CME consensus guidelines, including the use of direct oral anticoagulants, was recently published. In this review, we summarize these updated guidelines. Better adherence to the international guidelines, involving an adequate educational and active implementation strategies, will substantially decrease the burden of VTE and allow to increase survival in cancer patients.

The aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70.This is a retrospective study of patients aged above 70 years at the time of diagnosis of SSc and followed at an internal medicine unit between 2000 and 2015. Co-morbidities and clinical characteristics were analyzed, as well as survival at 1, 2 and 3 years.Of 246 patients, 27 (11%) were included (89% women, 96% Caucasians, age 78.3±4.5 years). Synchronous cancer was noted in 3 patients. SSc was mostly limited cutaneous only (24/27), with telangiectasia (63%), gastroesophageal reflux (59%) and digital ulcers (22%), and was associated with anti-centromere antibody (69%). Interstitial lung disease was not frequent (29%). Pulmonary arterial hypertension (PAH) was suspected at diagnosis of SSc in 14 cases (52%), but only 5 patients had undergone heart catheterization, with severe PAH in 3 cases. Survival at 1 and 3 years was 85.2% and 66.7%, and was worse in the case of suspected PAH, at 78.6% and 57.1% respectively.Cases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.

Renovascular hypertension accounts for 5-10 % of hypertensioncases in children; there is currently noconsensus on treatment. Here, we report on our clinical experience with this disease and outline the different pathways in which to investigate it. We report retrospectively on ten children diag- nosed with renovascular hypertension at the University Hos- pital of Nantes from 2001 to 2012. The main findings were obtained by fortuitous screening of children aged 2 months to 14 years old with neurofibromatosis (n=2) and fibromuscular dysplasia (n=8). The hypertension was always severe yet asymptomatic. Lesions were complicated in nine out of ten cases and included bilateral, multiple, mid-aortic syndrome and aneurysm. Doppler ultrasound associated with computed tomographyallowedfor aprecisediagnosisinsevenout often cases. Where ambiguities persisted, they were highlighted by arteriography, the gold standard investigation. Medical treatment was insufficient, leading to invasive procedures in nine out of ten children: 2 nephrectomies, 2 autotransplantations, and 21 repetitive percutaneous translu- minal angioplasties.After invasiveprocedures,blood pressure control improved in four cases and was resolved in three. Conclusion: Arteriography remains to be the gold standard technique for renovascular hypertension in children and can be combined with angioplasty when medical treat- ment is rendered obsolete. The role of computed tomog- raphy is controversial. Despite the heterogeneity of the children studied, we present a general medical and ther- apeutic management pathway for the treatment of this disease.

Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies.

Resume L’infection est une complication rare mais tres severe des protheses vasculaires. Nous rapportons l’observation d’une infection a Staphylococcus aureus d’une prothese iliofemorale, sept ans apres son implantation, revelee par un purpura vasculaire localise au membre inferieur droit. Cette observation est singuliere par sa presentation clinique bruyante a distance du geste chirurgical. En effet, le mode de revelation varie selon le delai d’apparition de l’infection, soit precoce et typique dans les quatre mois apres l’implantation, soit tardive avec des manifestations clinicobiologiques atypiques et aspecifiques. Cette manifestation clinique inhabituelle a type de purpura doit etre reconnue comme d’origine septique, et malgre les progres therapeutiques, la morbi-mortalite reste elevee.

Many cutaneous adverse events have been identified with recently developed targeted treatments. Some of them are common and specific, like paradoxical psoriasiform eruptions with anti-TNFα, papulopustular eruptions and paronychias with EGFR inhibitors and peculiar hand-foot skin reactions with multitargeted kinase inhibitors sorefenib and sunitinib. Patients treated with these recently available biologics need a careful monitoring.

Antiangiogenic agents are an innovative oral chemotherapy prescribed in metastatic renal cancer and gastrointestinal stromal tumors (GIST). These molecules have several side effects. A woman, with moderate hypertension and severe Thevenard's ulceromutilating acropathy, presented renal cancer with lung metastasis. She was treated by antiangiogenic therapy (sunitinib). Under this treatment, she presented some large, extensive, severe and necrotizing ulcerations of both hands and feet, exacerbated with a sepsis. Sunitinib was stopped and antibiotics were combined with surgical trimming leading to clinical remission and complete healing. Sunitinib inhibits both tumor angiogenesis and tumor cell proliferation, but also the preexisting microcirculation. In our case, severe neuropathy caused neurovascular dysregulation which, together with hypertensive microangiopathy, led to a severe hand-foot skin reaction. This microangiopathy worsened under anti-VEGF therapy. The clinical severity was linked to the severity of the neuropathy. To avoid having serious cutaneous consequences, neuropathy and microangiopathy have to be diagnosed before introducing antiangiogenic therapy.

Leiomyosarcomas are rare malignant mesenchymal tumors. We report the case of a 55-year-old man with a lung metastasis from an iliofemoral leiomyosarcoma mimicking a deep venous thrombosis (DVT). Vascular leiomyosarcoma occurs in the wall of large vessels, and usually presents as unexplained DVT. To date, few cases of peripheral leiomyosarcomas have been described in the literature. This observation demonstrates the usefulness of Positron Emission Tomography (PET), beyond oncologic staging, in such indications as the workup of an unexplained DVT. In this report, PET prevented a misdiagnosis of DVT by showing a peculiar cockade appearance of the vessel. Compared with conventional imaging, PET can provide complementary information. The specificity of such information still needs to be assessed.

Resume Propos L'atteinte inflammatoire des vaisseaux de gros calibre est retrouvee chez 10 a 20 % des patients ayant une maladie de Horton. L'atteinte aortique en represente la forme la plus grave avec des risques de rupture d'anevrisme et/ou de dissection aortique, pouvant compliquer ou reveler la maladie. L'aortite de la maladie de Horton concerne davantage l'aorte thoracique, mais l'atteinte abdominale est egalement possible. Il n'existe que peu de donnees sur l'evaluation de l'atteinte de l'aorte abdominale au stade initial de la maladie. Patients et methodes Il s'agit d'une etude prospective monocentrique qui a inclus consecutivement 30 patients au moment du diagnostic de maladie de Horton, prouvee par biopsie d'artere temporale, entre mai 1998 et mai 2002. Chaque patient a fait l'objet d'un recueil de donnees cliniques et biologiques puis a beneficie d'un examen systematique de l'aorte abdominale par echographie-doppler avec recherche d'anevrisme, d'ectasie, d'epaississements parietaux, et d'un halo hypoechogene periaortique. Resultats Parmi les 30 patients de cette serie (25 femmes et cinq hommes, âge moyen 68,5 ans), quatre (13 %) avaient un anevrisme de l'aorte abdominale de petite taille (23 a 27 mm de diametre), etendu sur 2 a 5,5 cm. Un epaississement parietal d'au moins 3 mm etait note chez 17 patients (68 %). Un halo hypoechogene periluminal, mesurant 4 a 8 mm etait note chez dix patients (33 %). Ce signe du halo etait note chez trois des quatre patients avec anevrisme. Conclusion L'atteinte aortique est une complication potentiellement grave de la maladie de Horton. La question du depistage systematique de cette complication reste posee. Notre etude montre que l'echographie-doppler permet de deceler des anomalies morphologiques de l'aorte abdominale des le stade initial de la maladie. Ces anomalies a type d'anevrisme debutant, d'epaississement parietal, et de halo periaortique, pourraient correspondre a des localisations inflammatoires de la maladie. Des etudes complementaires sont necessaires pour en preciser la specificite et la gravite potentielle.

Resume Propos. – Dans 10 a 15 % des cas, les hepatites granulomateuses sont idiopathiques. Quand elles sont symptomatiques, notamment avec une fievre prolongee, un traitement d'epreuve est discute. Le but de cette etude est de decrire les modalites de traitement d'epreuve que proposent les internistes francais devant une telle situation clinique. Methodes. – Il s'agit d'une enquete de pratique realisee au sein de la Societe nationale francaise de medecine interne (SNFMI), sous la forme d'un questionnaire anonyme a propos d'une observation clinique fictive d'hepatite granulomateuse idiopathique. Ce questionnaire a ete propose aux membres de la SNFMI lors des congres de juin et decembre 2004. La decision de mise en route d'un traitement ainsi que les modalites de ce traitement, etaient laissees a l'appreciation des internistes interroges. Resultats. – Trente-six internistes ont repondu a cette enquete. Dans la situation proposee, 89 % des internistes interroges instaurent un traitement d'epreuve. Dans 18 cas sur 36 (50 %), un traitement d'epreuve antituberculeux est propose en premiere intention (quadritherapie dans 11 cas). Dans sept cas (19 %), un traitement d'epreuve par prednisone serait prescrit, aux doses de 0,4 mg/kg par jour ( n = 1) et 1 mg/kg par jour ( n = 6). Pour sept internistes interroges (19 %), un traitement d'epreuve par cyclines serait indique, aux doses de 100 a 400 mg/j. La mediane de la duree de ce traitement d'epreuve serait de 28 jours (extremes = 8–252 jours). Les indicateurs d'efficacite de ce traitement sont : fievre (69 %), poids (59 %), taux de proteine C-reactive (59 %), et bilan hepatique (53 %). En cas d'echec du premier traitement d'epreuve, les praticiens interroges en prescrivent un deuxieme dans 69 % des cas : prednisone a la dose de 0,4 a 2 mg/kg par jour (72 %), traitement antituberculeux (16 %), cyclines 200 mg/j (12 %), d'une duree mediane de 28 jours. Sept des praticiens (19 %) ont propose de poursuivre une association de deux traitements d'epreuve. Discussion. – Devant une hepatite granulomateuse idiopathique, les internistes interroges proposent quatre options therapeutiques : l'abstention, le traitement antituberculeux, le traitement par cyclines, la corticotherapie. Le traitement antituberculeux de premiere intention est une proposition coherente du fait de la prevalence elevee de la tuberculose. Les donnees de la litterature concernant les traitements empiriques par corticoides ou par cyclines sont peu nombreuses. Les modalites precises de la conduite d'un traitement d'epreuve doivent etre validees. Conclusions. – La prise en charge des hepatites granulomateuses idiopathiques est difficile. Notre etude montre que les pratiques therapeutiques des internistes sont heterogenes. La proposition dominante est celle d'un traitement d'epreuve antituberculeux de premiere ligne, a evaluer au bout de quatre semaines, avec corticotherapie en cas d'echec (prednisone a la dose quotidienne de 1 mg/kg). A defaut de representer un consensus d'experts, notre etude jette les bases de l'elaboration de recommandations de pratique clinique face a une situation rare, complexe et heterogene, typiquement du ressort de la medecine interne.

Resume L’antithrombine (AT) appartient a la famille des inhibiteurs de serine protease. Son deficit constitutionnel est fortement thrombogene, notamment sur le plan veineux. Le deficit peut etre soit quantitatif (type I) soit qualitatif (type II) et differentes mutations sont desormais decrites. Le deficit de type II heparin binding site (HBS) correspond a une anomalie de liaison de l’AT a l’heparine. Ce deficit, a l’etat homozygote, est souvent lie a des manifestations thromboemboliques veineuses recidivantes. De tres rares cas de thromboses arterielles recidivantes sont decrits, principalement chez des enfants ou adultes jeunes. Nous rapportons l’observation d’une jeune patiente presentant des thromboses arterielles recurrentes dans le cadre d’un deficit de type II de type HBS, avec presence de la mutation Leu99Phe a l’etat homozygote deja rapportee dans la litterature. Cette observation illustre l’indication du bilan biologique de thrombophilie chez des patients jeunes, presentant des thromboses arterielles inexpliquees.

Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided.A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line.Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls.The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered.

Introduction Cutaneous arterio-venous malformations (AVM) are high-flow vascular malformations made up of a direct link between arteries and veins without intermediary capillary space. ‘Distal limb's AVM’, which mean involving hands or feet, are rare and their functional prognosis is often poor. Little is known about their early clinical symptoms. The objectives of this study were to identify early clinical symptoms of distal limb's cutaneous AVMs and to determine their long-term clinical outcome. Methods A retrospective study was carried out including adult patients who had distal limb's AVM, who were followed up between January 2000 and November 2013 in two regional tertiary care centres. The information was collected from patients' clinical records and completed by a structured telephone questionnaire. Results Nineteen patients were included in the study: four (21%) with foot AVM and 15 (79%) with hand AVM. The first clinical symptoms were as follows: swelling (47%), pain (47%), one or several venous dilatations (37%) and rarely abnormal skin colour, hyperthermia and pulsating sensation. The median diagnosis delay was 9 years after the onset of first manifestations. Amongst the 17 patients who underwent a treatment, 53% had embolotherapy session(s), 12% surgery and 35% had both. After an average follow-up of 57.6 months, 31% of the 13 patients contacted who were receiving treatment were in complete remission; 31% had partial remission; 15% had relapse after initial improvement and 23% had treatment failure. Overall, 74% of patients had a serious development of the AVM: 37% had digital or hand amputation, and 42% remained symptomatic and/or unstable. Conclusion This study suggests that initial manifestations of distal limb's AVMs are discreet and non-specific, leading to a diagnosis delay of about 10 years, with poor prognosis. Doctors should evoke the diagnosis earlier, when these symptoms are shown: pain and/or swelling, sometimes with a large vein.

Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies.

Prosthetic graft infection after vascular reconstruction is a rare but serious complication. We report a case of infection occurring late after implantation of an iliofemoral prosthetic vascular graft. The Staphylococcus aureus infection was revealed by vascular purpura localized on the right leg 7 years after implantation of a vascular prosthesis. This case illustrates an uncommonly late clinical manifestation presenting as an acute infection 7 years after the primary operation. In this situation, the presentation differs from early infection, which generally occurs within the first four postoperative months. Diagnosis and treatment remain a difficult challenge because prosthetic graft infection is a potentially life-threatening complication. Morbidity and mortality rates are high. Here we detail specific aspects of the clinical and radiological presentation.

Objectif Nous decrivons le cas du diagnostic antenatal d’une thrombose de la veine cave inferieure (VCI) et de la veine renale (VR) droite chez un fœtus d’une patiente de 37 ans a 33 semaines d’amenorrhees (SA). Observation Une patiente de 37 ans primigeste nullipare avec un antecedent familial au premier degre de thrombose veineuse profonde consulte a 33 SA pour baisse des mouvements actifs fœtaux (MAF). Une echographie revele chez le fœtus une asymetrie renale et une thrombose de la VR droite. Il est propose une surveillance clinique et echographique rapprochee. A 39 SA, l’accouchement est declenche pour une pre-eclampsie debutante et nait une petite fille de 3,505 kg en bonne sante. Un echo-Doppler realise a j1 retrouve un rein droit atrophie, la VR droite et la VCI sus-renale ne sont pas visualisees. Un scanner thoraco-abdomino-pelvien injecte realise a j6 confirme la presence d’un thrombus calcifie de la VCI sur 25 mm de hauteur, une thrombose de la VR droite et une asymetrie renale. Le diagnostic de phlebolithe est evoque devant le caractere ancien et il est decide de ne pas anticoaguler le nourrisson. Le suivi a un mois retrouve un bon developpement staturo-ponderal, l’absence de retard psychomoteur et une fonction renale normale. L’echo-Doppler retrouve une thrombose stable. Un bilan de thrombophilie met en evidence une mutation heterozygote du facteur V et une proteine S a la limite basse de la norme. Discussion La thrombose de la VCI antenatal est une pathologie vasculaire rare mais avec une prevalence difficilement evaluable car souvent asymptomatique. Elle est frequemment associee a une thrombose de la VR. L’etiologie est inconnue. Certains cas decrits dans la litterature retrouvent des facteurs de risque maternels tels que le diabete, l’hypertension arterielle, une thrombophilie familiale. Le diagnostic est souvent fortuit mais a ete decrit chez des patientes presentant une diminution des MAF et un travail premature. Il n’existe pas de recommandations quant a la therapeutique mais on propose le plus souvent une surveillance clinique, biologique et echographique. Conclusion Le diagnostic de thrombose de la VCI peut se faire en antenatal et est souvent d’evolution asymptomatique. Il n’existe pas de traitement specifique. A l’âge adulte, certaines agenesies de la VCI ne seraient-elles pas finalement en lien avec une thrombose de la VCI comme decrit ci-dessus avec involution de celle-ci ?

La maladie de Rendu-Osler ou telangiectasies hereditaires hemorragiques (THH) est caracterisee par la presence de fistules arterio-veineuses (FAV) viscerales. Il n’a pas ete rapporte a ce jour de localisation renale de FAV dans le cadre d’une THH. Nous presentons le cas de FAV renales traitees avec succes par radio-embolisation chez une patiente fortement suspecte de THH. Il s’agit d’une patiente de 35 ans presentant des coliques nephretiques typiques a repetition, toujours a droite, et dont l’echographie puis TDM abdominale a mis en evidence des FAV renales bilaterales. Elle a presente des epistaxis a repetition dans l’adolescence, plus espacees actuellement. Ses antecedents incluent une chirurgie non compliquee d’un kyste benin de l’ovaire droit et un accouchement non complique ; elle n’a jamais presente de traumatisme ou de chirurgie renale. Il n’y a pas d’antecedents familiaux de THH, mais son demi-frere cote maternel a presente des epistaxis a repetition a l’adolescence. Cliniquement, elle presente quelques telangiectasies sur les pourtours du nez, sans telangiectasies muqueuses de la bouche, ni sur la pulpe des doigts ; l’examen physique cardiopulmonaire est normal. NFS et ferritinemie sont normales. La TDM thoraco-abdomino-pelvienne d’extension ne met en evidence que les FAV renales sans FAV hepatique ni pulmonaire. L’echocardiographie revele un hyperdebit a 7,5 L/min avec test a bulle negatif confirmant l’absence de micro-FAV pulmonaire. La patiente repondant a 3 criteres de Curacao, le diagnostic de THH est suspecte. L’analyse genetique n’a pas permis de mettre en evidence de mutation dans 12 genes impliques dans les maladies angio-genetiques hereditaires (ACVRL1, ENG, RASA1, SMAD4, GDF2, BMPR2, CAV1, KCNK3, EIF2AK4, SMAD9, TEK, TBX4) mais ceci ne remet pas en cause le diagnostic clinique de cette maladie et laisse la place a l’identification de nouveaux genes. Le traitement de cette FAV renale, consistant en une vaso-occlusion renale droite a l’aide de plug, est un succes : absence de recidives de coliques nephretiques, pas d’alteration de la fonction renale et diminution significative du debit cardiaque a 5,8 L/min. La THH pourrait donc s’accompagner de FAV renale. Cette atteinte doit etre recherchee devant certains symptomes evocateurs. Un traitement endovasculaire peut etre propose dans cette localisation en cas de symptome comme dans les autres atteintes d’organe plus classiques.

Objectif L’objectif etait d’etudier les caracteristiques des patients, les modalites de prescription, de surveillance et d’evaluation du traitement par iloprost intraveineuse, en 2004 et 2014. Materiel et methode Nous avons realise une etude observationnelle, longitudinale, descriptive, retrospective, monocentrique sur les cohortes de patients ayant recu de l’iloprost en 2004 et en 2014, dans le service de medecine vasculaire du centre hospitalier universitaire de Nantes. Nous avons recueilli les caracteristiques des patients, les indications, et les modalites du bilan pretherapeutique, de prescription et d’evaluation du traitement. Resultats En 2004, 19 patients avaient recu le traitement, contre 27 en 2014. Les indications n’etaient pas differentes entre 2004 et 2014, 9/19 vs 14/27 ischemies de repos, 9/19 vs 12/27 ulceres digitaux et 1/19 vs 1/27 en prevention de crise hivernale. Le bilan pretherapeutique comportait toujours la realisation d’un ECG, d’une mesure de la clairance de la creatininemie, mais la mesure de la TcPO2 de repos n’avait pas toujours ete effectuee en cas d’ischemie de repos, 8/9 en 2004 et 9/14 en 2014. La dose prescrite etait toujours adaptee a la tolerance clinique et a la clairance de la creatininemie. Un relais en hospitalisation a domicile (HAD) avait ete effectue chez 6 patients en 2014. L’evaluation de l’efficacite se faisait de maniere subjective et non standardisee sur des donnees cliniques. Il n’y a pas eu de complications majeures (syndrome coronaire aigu ou hemorragie grave). Conclusion Les pratiques de prescription de l’iloprost sont conformes a l’autorisation de mise sur le marche. Les indications, la tolerance et l’evolution des patients sous traitement n’etaient pas differentes entre 2004 et 2014. Une evaluation objective et standardisee du traitement meriterait d’etre mise en place. Enfin, le recours a l’HAD apparaitrait comme une alternative pour reduire la duree d’hospitalisation.

Introduction La thrombose de Mondor (TM) est une maladie rare, caracterisee par une thrombose veineuse superficielle (veines sous-cutanees), spontanement regressive. Decrite initialement au niveau thoracique, elle touche rarement la veine dorsale du penis (ou ses collaterales). Nous rapportons le cas de 2 patients avec une TM penienne, vus en consultation de medecine vasculaire. (1) M. P., 40 ans, presente un œdeme sur la face laterale de la verge, sans erytheme ni douleur, suite a une hyperactivite sexuelle. Il n’a pas eu d’episode infectieux genito-urinaire recent. On note un episode similaire 3 ans auparavant, resolutif en moins de 24 h. Le Doppler retrouve une thrombose d’une collaterale de la veine dorsale de la verge. Un bilan de thrombophilie est realise, retrouvant une mutation heterozygote du facteur II. Le patient est mis sous anticoagulation curative pendant 21 jours. Les symptomes regressent en 1 mois, parallelement a une diminution volontaire de l’activite sexuelle. (2) M. K., 36 ans, consulte pour l’apparition brutale d’un cordon indure penien, non douloureux, associe a un œdeme du prepuce. Il n’est pas retrouve d’hyperactivite sexuelle, mais une activite sportive inhabituellement intense dans les jours precedents (cyclisme). Un traitement anti-inflammatoire oral permet une regression des symptomes. L’œdeme reapparait 1 semaine apres arret du traitement ; il est alors realise un bilan retrouvant la presence d’IgM anti-cardiolipine. Le patient est mis sous anticoagulation curative par heparine avec relais AVK, pendant 3 mois. L’œdeme regresse en 5 semaines. Discussion La TM penienne est une entite peu connue, ayant fait l’objet de peu de publications, notamment sur sa prise en charge, avec moins de 60 cas decrits et une seule etude observationnelle retrospective. Un facteur mecanique de frottement est souvent retrouve, avec au premier rang une activite sexuelle intense. Les experts recommandent actuellement un traitement medical par anti-inflammatoire topique ou per os. Les anticoagulants ne semblent pas avoir de place. Le traitement chirurgical (thrombectomie) a ete rapporte de maniere exceptionnelle (4 patients). Nos 2 patients ont ete anticoagules par exces, induit par les anomalies des bilans de thrombophilie (egalement prescrits par exces). Conclusion La TM n’est pas une indication a un bilan de thrombophilie, source d’exces de traitement par anticoagulation, et donc de risque hemorragique, alors que l’evolution est spontanement favorable.

Objectif Ameliorer les pratiques concernant la pose, le suivi et la prise en charge des complications des catheters veineux centraux hors services de reanimation (CVCHSR). Materiel et methode Au CHU de Nantes, depuis 2013, un groupe de medecins s’est constitue pour evaluer les pratiques d’usage des CVCHSR dont le recours devient de plus en plus frequent. Apres avoir evalue les besoins, valide les indications et uniformise les pratiques de pose, la necessite de disposer d’une synthese pratique des recommandations issues des societes scientifiques sur les sujets de qualite, de prevention et de prise en charge des complications (infectieuse, mecanique et vasculaire) a permis une nouvelle phase. Apres la redaction de fiches pratiques abordant chaque sujet, y compris les thematiques vasculaires, une evaluation a donc ete menee avant puis apres la diffusion des fiches techniques et pratiques, d’une part, et une intervention ciblee dans chaque service prescripteur, d’autre part. Les resultats des questionnaires sont compares. Resultats Les questionnaires ont ete renseignes avant diffusion des recommandations pour 76 prescripteurs et apres pour 43. Les differentes cibles de thematiques vasculaires ont ete nettement ameliorees, tant concernant la prevention (84 % contre 58 %), la reconnaissance de signes suspects de thrombose que sur la methode diagnostique (86 % contre 75 %) et la prise en charge (44 % contre 4 %). Le niveau de satisfaction des prescripteurs a egalement progresse (3,5/4 contre 2,6/4). Conclusion Le medecin vasculaire doit participer a l’amelioration des pratiques concernant les CVCHSR, tant sur les complications thrombotiques que lors de l’evaluation initiale avant pose. L’integration de la medecine vasculaire aux groupes de travail ou de qualite est primordiale pour la redaction de recommandations de bonnes pratiques des CVCHSR locales ou nationales.

Many cutaneous adverse events have been identified with recently developed targeted treatments. Some of them are common and specific, like paradoxical psoriasiform eruptions with anti-TNFα, papulopustular eruptions and paronychias with EGFR inhibitors and peculiar hand-foot skin reactions with multitargeted kinase inhibitors sorefenib and sunitinib. Patients treated with these recently available biologics need a careful monitoring.

Introduction Les malformations arterio-veineuses (MAV) cutanees sont des malformations vasculaires a flux rapide composees d’arteres et de veines connectees entre-elles sans lit capillaire intermediaire. Les « MAV distales des membres », c’est-a-dire atteignant les mains ou les pieds sont rares et souvent de mauvais pronostic. Leurs manifestations cliniques precoces sont mal connues. Les objectifs de notre etude etaient d’identifier les manifestations cliniques precoces des MAV cutanees distales des membres et d’evaluer leur pronostic a long terme. Patients et methodes Notre etude retrospective a inclus les patients adultes atteints d’une MAV distale de membre suivis dans deux centres specialises entre janvier 2000 et novembre 2013. Observations Les informations etaient recueillies dans les dossiers des patients et lors d’un questionnaire telephonique standardise. Resultats L’etude a inclus 19 patients : 4 (21 %) avaient une MAV du pied et 15 (79 %) de la main, dont 8 sur 15 (53 %) avec une atteinte digitale. Aux dernieres nouvelles, un tiers des MAV etaient compliquees, avec deux MAV de stade 1 (11 %), onze de stade 2 (58 %), cinq de stade 3 et une de stade 4. Les premieres manifestations cliniques etaient : une tumefaction localisee (47 %), des douleurs (47 %), une/des veine(s) dilatee(s) (37 %), et plus rarement : une coloration anormale, une hyperthermie locale et une sensation de battement. Le delai diagnostique etait de 10,4 ans apres l’apparition des premieres manifestations cliniques. Sur le plan therapeutique, 47 % des patients avaient ete traites par embolisation(s), 10,5 % par chirurgie, et 32 % par embolisation(s) et chirurgie. Des complications severes du traitement etaient observees dans 32 % des cas et une amputation avait ete realisee dans 37 % des cas. Apres un suivi moyen de 57,6 mois, parmi les 13 patients traites que nous avons pu contacter, 31 % etaient en remission complete, 31 % en remission partielle, 15 % en recidive apres amelioration, et 23 % etaient en echec therapeutique. Discussion Notre etude est la premiere a s’interesser aux manifestations inaugurales des MAV distales de membres. Elle suggere que les signes cliniques sont longtemps discrets et aspecifiques, entrainant un retard diagnostique de plus de 10 ans, et que le pronostic de ces MAV est globalement pejoratif. Conclusion Le clinicien doit donc evoquer precocement le diagnostic de MAV distale de membre, devant des douleurs et/ou une tumefaction parfois associee a une veine dilatee pour ne pas retarder la prise en charge.

Antithrombin (AT) is a natural anticoagulant and serine protease inhibitor. Hereditary AT deficiencies are uncommon and are associated with a higher risk of venous thrombo-embolism (VTE) compared to other thrombophilias. They are either quantitative (type I) or qualitative (type II) and various mutations have been reported. The II HBS deficiency is caused by a defect in the heparin-binding region (HBS) of AT. This homozygous deficiency increases the risk of VTE. The association with arterial thrombosis is weak but a few cases have been reported. We report a 20-year-old female with recurring arterial thrombosis associated with an AT type II HBS deficiency, due to a homozygous mutation Leu99Phe. In young patients, with recurrent and unexplained arterial thrombosis, a biologic thrombophilia has to be comprehensively searched.

Antiangiogenic agents are an innovative oral chemotherapy prescribed in metastatic renal cancer and gastrointestinal stromal tumors (GIST). These molecules have several side effects. A woman, with moderate hypertension and severe Thevenard's ulceromutilating acropathy, presented renal cancer with lung metastasis. She was treated by antiangiogenic therapy (sunitinib). Under this treatment, she presented some large, extensive, severe and necrotizing ulcerations of both hands and feet, exacerbated with a sepsis. Sunitinib was stopped and antibiotics were combined with surgical trimming leading to clinical remission and complete healing. Sunitinib inhibits both tumor angiogenesis and tumor cell proliferation, but also the preexisting microcirculation. In our case, severe neuropathy caused neurovascular dysregulation which, together with hypertensive microangiopathy, led to a severe hand-foot skin reaction. This microangiopathy worsened under anti-VEGF therapy. The clinical severity was linked to the severity of the neuropathy. To avoid having serious cutaneous consequences, neuropathy and microangiopathy have to be diagnosed before introducing antiangiogenic therapy.

Leiomyosarcomas are rare malignant mesenchymal tumors. We report the case of a 55-year-old man with a lung metastasis from an iliofemoral leiomyosarcoma mimicking a deep venous thrombosis (DVT). Vascular leiomyosarcoma occurs in the wall of large vessels, and usually presents as unexplained DVT. To date, few cases of peripheral leiomyosarcomas have been described in the literature. This observation demonstrates the usefulness of Positron Emission Tomography (PET), beyond oncologic staging, in such indications as the workup of an unexplained DVT. In this report, PET prevented a misdiagnosis of DVT by showing a peculiar cockade appearance of the vessel. Compared with conventional imaging, PET can provide complementary information. The specificity of such information still needs to be assessed.

Objectif Evaluer les elements cliniques, les modalites diagnostiques et therapeutiques, mais aussi l’evolution des hypertensions reno-vasculaires (HTARV). Materiel et methode Etude retrospective monocentrique des patients hospitalises pour HTARV entre 2002 et 2013. Les dossiers obtenus a partir des donnees du codage informatique sont ensuite analyses puis valides. Resultats Sur 92 dossiers avec codage d’HTARV, 47 patients ont un diagnostic confirme d’HTARV, dont 25 hommes et 22 femmes d’âge moyen de 52,7 ans, dont 6 mineurs et 10 adultes de moins de 45 ans. Le diagnostic d’HTARV a ete porte devant une HTA resistante dans 29,8 % des cas, une aggravation de la fonction renale sous inhibiteur du systeme renine-angiotensine et des OAP « flash » dans 17 % et 14,9 % des cas respectivement. Dix-sept pour cent des patients avaient un hyperaldosteronisme secondaire. L’ensemble des patients ont beneficie pour le diagnostic d’une echographie doppler. Le recours a l’angioscanner a eu lieu dans 66 % des cas. L’angio-IRM a ete plus rarement realisee (12,7 %). Un pourcentage de 74,5 % des patients (soit 35 patients) a ete traite par une angioplastie et 23,5 % (soit 11 patients) par un traitement medical seul. Un patient a eu une prise en charge chirurgicale. Le suivi median est de 36 mois. Un patient a ete perdu de vue. Trois deces ont ete recenses dans le suivi (2 dans le groupe traite par angioplastie et un parmi les patients traites par les medicaments seuls). Absence de difference statistiquement significative dans notre serie en termes d’efficacite sur la pression arterielle et la fonction renale entre les patients du groupe angioplastie associee au traitement medical et ceux du groupe traitement medical seul (71,4 % et 54,5 % respectivement, p = 0,78). Parmi les patients ayant beneficie d’une angioplastie, 25 patients (71,4 %) ont ete ameliores (diminution de la pression arterielle et/ou diminution du nombre de traitements antihypertenseurs et stabilisation de la fonction renale) sur une periode de suivi moyen de 43,6 mois. Conclusion La prise en charge de l’HTARV dans notre centre est conforme aux donnees de la litterature. Un tiers des patients ne presente qu’une hypertension resistante. Les modalites diagnostiques reposent sur l’echographie, souvent completee par un scanner. Notre etude bien que retrospective, avec un faible effectif, temoigne de l’efficacite et de l’absence de complications de l’angioplastie dans l’HTARV.

Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis.This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta.Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm.Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

Ten to fifteen percent of granulomatous hepatitis are idiopathic. If symptoms like prolonged fever are present, empirical treatment is discussed. The goal of this study is to describe the empirical treatment proposed in this situation by French specialists of internal medicine.We conducted a practice investigation among the French national society of internal medicine (SNFMI), using an anonymous questionnaire that related a case of idiopathic granulomatous hepatitis. This questionnaire was proposed to all French internists present at the SNFMI congress in June and December 2004. French specialists of internal medicine had to answer if they would prescribe an empirical treatment and if so, to specify this treatment.Thirty-six French specialists of internal medicine answered to the questionnaire. In the proposed situation, 89% of them initiate an empirical treatment. In 18/36 cases (50%), a first-line anti-tuberculosis empirical treatment is proposed (quadritherapy in 11 cases). In 7 cases (19%), an empirical treatment with prednisone, 0.4 mg/kg/d (N=1) and 1 mg/kg/d (N=6), would be prescribed. Seven internists (19%) would prescribe an empirical treatment with cyclins at the dose of 100 to 400 mg/d. Median duration of the empirical treatment would be 28 days (range: 8-252d). The evaluation parameters mentionned are: fever (69%), weight (59%), seric level of C-reactive protein (59%), and liver biology (53%). In case of failure of first-line empirical treatments, 69% of all questionned internists prescribe a second-line treatments: prednisone at the dose of 0.4 to 2 mg/kg/d (72%), anti-tuberculosis treatments (16%), cyclins 200 mg/d (12%), with a median duration of 28 days. Seven internists (19%) propose to combine two empirical treatments.Faced with a problem of idiopathic granulomatous hepatitis, French internists questionned propose four therapeutics options: no treatment, anti-tuberculosis treatment, cyclins or steroids treatment. First-line anti-tuberculosis treatment is a coherent proposition regarding to the high prevalence of tuberculosis. There are only few data available concerning empirical treatment with steroids or cyclins. Specific proposition of such empirical treatments should be defined.The management of idiopathic granulomatous hepatitis is difficult. Our study shows that therapeutics practices of French internists are heterogenous. The main proposition consists in a first-line anti-tuberculosis empirical treatment, that has to be evaluated after four weeks, and switched with steroids (prednisone, 1 mg/Kg/d) in case of failure. This study is not an expert proposition but contributes to suggest clinical practice guidelines for a rare, complex, heterogenous, and typically internist situation.