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Resume Introduction. – La thrombose intracardiaque est une complication rare de la maladie de Behcet (MB), qui peut se presenter comme une tumeur intracardiaque. Sa decouverte precede, dans la moitie des cas, le diagnostic de MB. La mortalite elevee peut etre en rapport avec des complications postchirurgicales et/ou une atteinte associee des arteres pulmonaires. Exegese. – Nous rapportons l'observation d'une femme francaise caucasienne de 31 ans, aux antecedents de maladie thromboembolique veineuse (MTEV), qui a subi une chirurgie apres la decouverte d'une tumeur du ventricule droit. Il s'agissait d'un thrombus organise avec fibrose endomyocardique et dans les suites operatoires nous avons diagnostique une MB. L'evolution a ete favorable sous traitement medical associant une corticotherapie, de la colchicine et des antivitamines K (AVK), sans recidive quatre ans apres. Conclusion. – La decouverte d'une masse intracardiaque chez un sujet jeune doit faire evoquer le diagnostic de thrombus cardiaque et de maladie de Behcet, meme en l'absence de facteur ethnique ou geographique predisposant.

The optimal duration of oral anticoagulant therapy is a matter of debate. It is essential to balance the desired effect of the anticoagulants in reducing recurrences against the risk of major bleeding. Recent data suggest that it is necessary to tailor the duration of anticoagulation individually according to the topography of deep vein thrombosis (DVT) and the presence of risk factors. A six-week treatment for patients with isolated calf DVT is sufficient. For proximal DVT and/or pulmonary embolism, a short anticoagulant course seems sufficient in patients with temporary risk factors (three months) and a longer anticoagulant course (six months at least) is recommended for cases with permanent risk factors or idiopathic DVT. The inherited or acquired hypercoagulable states can be divides into those that are common and associated with a modest risk of recurrence (i.e. isolated factor V Leiden or G20210A prothrombin gene) and those are uncommon but associated with a high risk of recurrence (i.e. antithrombin, protein C or S deficiencies and anticardiolipin antibodies). Thus, the presence of one of these last abnormalities favours more prolonged anticoagulant therapy. For the high-risk of recurrence patients, there is a paucity of evidence based medicine particularly for patients with biological thrombophilia, and randomised controlled trials in this population are required. An assessment of low- or fixed-dose oral anticoagulation is also necessary in order to reduce the bleeding risk.

Objective To describe the clinical, biologic, and histologic features of temporal artery biopsy (TAB)–localized systemic necrotizing vasculitides (SNV), and to assess their frequency among elderly patients undergoing TAB for suspected giant cell (temporal) arteritis (GCA). Methods The frequency of a TAB localization of SNV was prospectively assessed in a multicenter study of elderly patients undergoing TAB for suspected GCA. All patients with SNV fulfilling the American College of Rheumatology criteria for a specific vasculitic syndrome and with evidence of vasculitis on TAB were included in a retrospective, descriptive study. Results SNV was diagnosed based on the TAB in 1.4% of the patients with suspected GCA and in 4.5% of the positive (inflamed) TAB specimens. We retrospectively selected 27 patients (18 female, 9 male; mean ± SD age 62 ± 15 years, range 22–79 years) with SNV and TAB-localized vasculitis. Only 2 of these patients were known to have SNV before TAB localization. Twenty-two patients (81%) had cephalic symptoms, including jaw claudication in 33%, clinically abnormal temporal arteries in 33%, and neuro-ophthalmologic symptoms in 11%. All patients had systemic symptoms suggestive of SNV and histologically proven NV in the TAB specimens (70%) or elsewhere in other biopsy sites (74%). Abnormal biologic results suggestive of SNV were present in 17 patients (63%). For 4 patients, the TAB-documented involvement led to initial misdiagnoses of GCA, and systemic manifestations that developed under steroid therapy revealed the correct diagnosis. The final diagnoses of the patients were polyarteritis nodosa (PAN) (n = 11), Churg-Strauss syndrome (n = 6), micropolyangiitis (n = 3), Wegener's granulomatosis (n = 3), hepatitis B virus–related PAN (n = 2), hepatitis C virus–related cryoglobulinemic vasculitis (n = 1), and rheumatoid vasculitis (n = 1). Conclusion TAB-localized SNV presents a major diagnostic dilemma because it can mimic GCA. Careful analysis of clinical, biologic, and histologic data should lead to the correct diagnosis and help guide the clinician's choice of appropriate therapy.

Aim: To assess clinical features, treatment and outcome of Hypothalamo-pituitary (HP) sarcoidosis and to determine whether HP is associated with a particular clinical phenotype of sarcoidosis. Design: Multicentric retrospective study. Methods: Retrospective chart review. Each patient was matched with two controls. Results: Twenty-four patients were identified (10 women, 14 men). Their median age at the sarcoidosis diagnosis was 31.5 years (range: 8–69 years). HP involvement occurred in the course of a previously known sarcoidosis in 11 cases (46%), whereas it preceded the diagnosis in 13 patients (54%). All but two patients had anterior pituitary dysfunction, 12 patients presented with diabetes insipidus. The most common hormonal features were gonadotropin deficiency ( n = 21), TSH deficiency ( n = 15) and hyperprolactinemia ( n = 12). Magnetic Resonance Imaging (MRI) revealed infundibulum involvement ( n = 8), pituitary stalk thickness ( n = 12) and involvement of the pituitary gland ( n = 14). All but two patients received prednisone. After a mean follow-up of 4 years, only two patients recovered from hormonal deficiencies. MRI abnormalities improved or disappeared in 12 cases under corticosteroid. There was no correlation between the hormonal dysfunctions and the radiologic outcomes. Patients with HP sarcoidosis had significantly more frequent sinonasal localizations and neurosarcoidosis and required a systemic treatment more frequently than controls. Conclusion: Although HP sarcoidosis is unusual, physicians should be aware that such specific localization could be the first manifestation of sarcoidosis. HP involvement is associated with general severity of sarcoidosis. MRI abnormalities can improve or disappear under corticosteroid treatment, but most endocrine defects are irreversible.

International audience; OBJECTIVE: To describe effectiveness, steroid-sparing effect, and tolerance of the antiproliferative immunosuppressant mycophenolate mofetil (MMF) in neurosarcoidosis. METHODS: We describe a retrospective case series of 10 consecutive patients with a diagnosis of neurosarcoidosis who were treated with MMF, alone or in association with corticosteroids, in our teaching hospital. RESULTS: At the time of our study, the mean duration of MMF treatment was 21 months. All but one patient with CNS involvement (n = 8) were in remission (except for hormonal dysfunction) which was complete in 6 patients. MMF was efficient as single-agent induction therapy in one patient. The 3 patients who received MMF as a maintenance therapy after initial response to corticosteroids did not relapse even though steroids were stopped. Out of 4 subjects who demonstrated insufficient response to prior therapy including corticosteroids and immunosuppressive agents, 3 demonstrated significant clinical and radiologic improvement. However, the 2 patients who presented muscular sarcoidosis did not respond to MMF. Among patients treated with steroids at MMF introduction and after excluding those with sarcoid myopathy, the mean dose of corticosteroids was 6 mg/day at the end of the follow-up while it was 59 mg/day at the initiation of MMF. No significant side effects were observed. CONCLUSIONS: These data suggest that MMF is effective in CNS sarcoidosis but not in sarcoid myopathy, with a corticosteroid sparing effect and a better tolerance profile than other immunosuppressive agents.

Resume Deux cas de femmes jeunes, avec occlusion arterielle aigue du membre superieur droit et diagnostic concomitant de maladie de Basedow sont rapportes. Il s'agit vraisemblablement d'embolies arterielles sur trouble du rythme paroxystique, sans notion de cardiopathie. Le bilan thyroidien doit etre rajoute a la liste des examens complementaires a pratiquer au cours de la demarche etiologique d'une occlusion arterielle chez un sujet jeune sans facteur de risque cardio vasculaire.

The optimal duration of oral anticoagulant therapy after a first episode of venous thromboembolism (VTE) is still a matter of debate. It is essential to balance the desired effect of the anticoagulants in reducing recurrences against the risk of major bleeding. The aims of this paper are to describe the current concepts in this field. Recent data, based on randomized controlled trials, suggest that it is necessary to tailor the duration of anticoagulation individually according to the topography of VTE and the presence of risk factors. A 6-week treatment for patients with isolated calf vein thrombosis is sufficient. For proximal thrombosis and/or pulmonary embolism, a short anticoagulant course is sufficient in patients with temporary risk factors (3 months), and a longer anticoagulant course (6 months at least) is recommended for cases with permanent risk factors or idiopathic VTE. For these high-risk of recurrence patients, an assessment of low- or fixed-dose oral anticoagulation is necessary in order to reduce the bleeding risk. It is not possible to precisely determine the optimal duration with the available data. We have already performed a meta-analysis on summary data that suggests a long course of oral anticoagulant therapy is superior to a short course. An individual meta-analytic approach is needed to draw more precise conclusions on an interesting and important clinical topic.

Pinede L, Duhaut P, Cucherat M, Ninet J, Pasquier J, Boissel JP (Hopital Edouard Herriot and Unite de Pharmacologie Clinique, Lyon, France). J Intern Med 2000; 247: 553–562. Objective. To assess the length of oral anticoagulant therapy (short versus long duration) after a first episode of venous thromboembolism (VTE). Design. Meta-analysis of randomized controlled trials, comparing two durations of anticoagulation, identified in 1999 by a computerized search of the Cochrane Controlled Trial Register, Medline and Embase, completed by an extensive review of the references of pertinent articles. Setting and subjects. The meta-analysis was performed on literature data. Seven published controlled trials were included. Relative risks with 95% confidence intervals were computed using the relative risk logarithm method. Statistical significance was set up at 0.01 for the test of association. Main outcome measures. Outcomes are major haemorrhage and recurrence after a 12-month follow-up. Results. For the recurrence end-point (sample size of 2304 patients), a duration treatment of 12–24 weeks seems preferable to a 3–6 week regimen, with a relative risk (RR) of 0.60 (95% CI: 0.45–0.79, P

OBJECTIVE To assess the potential role of allo-immunization, either by former pregnancies, or by a history of blood transfusion, in the pathogenesis of giant cell arteritis and polymyalgia rheumatica. METHODS Two hundred and eighty-five incident female cases and 186 age-matched, population-based female controls were prospectively included in a multicentre case-control study. RESULTS The number of pregnancies was significantly lower in cases than in controls (nulliparous: 21.55% vs 12.90%; > or =4 pregnancies: 16.25% vs 27.42%; Wilcoxon rank sum test: P = 0.0019) in biopsy-proven or negative temporal arteritis and, to a lesser extent, in polymyalgia rheumatica. No difference was found for history of blood transfusion. Pregnancies remained negatively associated with the disease in a multivariate analysis including cardiovascular risk factors such as smoking or a pre-existing peripheral vascular disease. CONCLUSION Former pregnancies are not a risk factor for giant cell arteritis. Pregnancies may be protective thanks to an effect of the associated hyperoestrogenic state against alterations of the artery wall, as suggested in animal models.

We report two cases of fatal necrotizing vasculitis in patients under 50, with intestinal gangrena resistant to immunosuppressive treatment, plasmapheresis and hyperbar oxygen. Although showing signs of necrotizing arteritis, biopy failed to recognize any specific feature of known vasculitis.

Jaw claudication and clinically abnormal temporal artery are shown to be highly specific of giant cell arteritis with positive biopsy, whereas headache and fever, more commonly seen in negative biopsy arteritis, may reflect the inclusion of non-related pathologies in this group.

We report a set of clinical criteria easily collected at the time of patient's examination, which allow the diagnostic of Giant Cell Arteritis with a sensitivity of 85 % and a positive predictive value of 72,5 % when the biopsy is negative.

We report one case of biopsy-proved large cell arteritis with polyneuropathy and intestinal involvement leading in 15 days to mesenteric ischemia, small bowel perforation, multivisceral failure and death in spite of the administration of high-dose steroids, symptomatic treatment and abdominal surgery.

We report a series of 40 cases of infectious endocarditis, diagnosed on a 10-year period in a department of Internal Medicine. Initial clinical features were not suggestive of endocarditis and led to delayed hospitalization and diagnosis.

33 % of patients giant cell arteritis (Ta and PMR) versus 9 % of controls have been tested positive for anticardiolipine antibodies at the time of diagnosis. Positive patients presented severe vascular complications.

We report a case of a 60-year old woman, with a tracheal stenosis as the first sign of Wegener's granulomatosis. This is the twelfth observation described in the literature.

We report a case of digital necrosis after treatment with diclofenac, regressive after cessation of the drug and steroid therapy. Biological findings were those of a chemically induced lupus.

We report 2 complications of celiac disease, which were the first sign of the disease previously unknown : a B lymphoma and an adenocarcinoma of the small intestine.

From a analysis of 36 personal observations of Wegener's Granulomatosis and a review of the literature, we have studied the clinical manifestations and laboratory and histopathological findings of the disease. We emphasize the importance of antibodies against neutrophil cytoplasm and surgical pulmonary biopsy in affirming the diagnosis.