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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.

Resume Propos. – La duree optimale du traitement anticoagulant oral apres un premier episode de maladie thromboembolique veineuse reste encore controversee. Il est essentiel dˈevaluer conjointement lˈeffet positif des anticoagulants qui reduisent lˈincidence des recidives et leur effet negatif qui est la survenue de complications hemorragiques iatrogenes. Lˈobjectif de cet article est de faire une mise au point sur lˈetat actuel des connaissances dans ce domaine. Actualites et points forts. – Les donnees recentes, fondees sur les resultats dˈessais controles, montrent quˈil est necessaire dˈenvisager une gestion personnalisee de la duree du traitement anticoagulant oral en fonction de la gravite de la maladie thromboembolique veineuse et de la presence ou non de facteurs de risque. Ainsi, six semaines de traitement sont suffisantes chez les patients avec une thrombose veineuse profonde surale isolee. Au decours dˈune thrombose veineuse profonde proximale et/ou dˈune embolie pulmonaire, une duree de traitement breve (trois mois) semble suffisante chez les patients avec un facteur de risque temporaire, mais une duree de traitement plus prolongee (au minimum six mois) est necessaire chez ceux avec un facteur de risque permanent ou une maladie thromboembolique veineuse idiopathique. Les thrombophilies biologiques acquises ou hereditaires doivent etre reparties entre celles qui sont frequentes mais associees a un risque faible de recidive (facteur V Leiden heterozygote isole ou mutation du gene de la prothrombine) et celles qui sont plus rares mais associees a un risque eleve de recidive (deficit en antithrombine, proteine C ou S, anticorps anticardiolipines). Ainsi la presence de l’une de ces dernieres causes de thrombophilie justifie un traitement anticoagulant oral prolonge. Perspectives et projets. – 1) Chez les patients avec un risque eleve de recidive thromboembolique, surtout ceux avec une thrombophilie biologique, les donnees actuelles de la science sont insuffisantes et de nouveaux essais controles sont indispensables. Afin d’essayer de reduire le risque hemorragique, une evaluation de faible dose (INR cible 1,5–2,0) ou de dose fixe d’anticoagulants oraux sont necessaires. 2) D’apres les donnees actuellement disponibles, la duree optimale du traitement anticoagulant oral est incertaine dans certains cas. Nous avons realise une meta-analyse d’apres les donnees resumees de la litterature qui suggere qu’une duree de traitement prolongee est superieure a une duree plus breve. Nous proposons de realiser une meta-analyse internationale a partir des donnees individuelles des patients inclus dans les essais, et cette approche nous permettra d’obtenir des conclusions plus solides sur cette problematique tres importante en pratique clinique.

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.

Cardiac thrombosis is a rare complication of Behçet's disease (BD), which may present as a cardiac tumor. Its discovery precedes, in half of the cases, the diagnosis of BD. The high mortality may be associated to postsurgical complications and/or an associated involvement of pulmonary arteries.We present the case of a 31 years old Caucasian French woman, with a history of venous thromboembolic disease, who had surgery after the discovery of a right ventricle tumor. That was an organised thrombus with endomyocardial fibrosis and a diagnosis of Behçet's disease was made after the surgery. The outcome was favourable under medical treatment associating corticosteroids, colchicine and antivitamin K (AVK), without relapse four years later.The discovery of an intracardiac mass in a young patient must evoke the diagnosis of cardiac thrombus and Behçet's disease, even in the absence of predisposing ethnic or geographic factor.

The overprevalence of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) in women remains unexplained. Microchimerism pathogenicity has been discussed in some systemic diseases. We tested history of pregnancy as a risk factor for GCA.Prospective, multicenter case-control study with multiple, age-matched, control groups. Patients have been included in 40 different centers. The first control group has been randomly selected in the general population, consecutive hospitalized patients in two geographically distant departments of internal medicine made up the second and third ones.Three hundred and fifteen patients (249 GCA and 66 PMR), 242 general population controls, 333 in the first hospitalized control group, and 355 in the second, have been included in the 1991-1998 period. Pregnancy has been constantly protective against GCA/PMR (Wilcoxon rank sum test: P = 0.0001, 0.0005, and 0.054, respectively, for the three control groups), more particularly for parity equal or greater than 4 (OR = 0.32, 95% CI: 0.18-0.57, P = 0.00003; OR = 0.44, 95% CI: 0.26-0.74; P = 0.0009, and OR = 0.42; 95% CI: 0.25-0.71, P = 0.0006, respectively). In multivariate analysis, risk for GCA on pre-existing degenerative, vascular disease is decreased by half for each pregnancy (OR = 0.49, 95% CI = 0.27-0.90, P = 0.022).Contrary to the initial hypothesis, multiparity is a protective factor against GCA. Mechanism is unknown.

The optimal duration of oral anticoagulant therapy after a first episode of venous thromboembolism 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 randomised controlled trials, suggest that it is necessary to tailor the duration of anticoagulation individually according to the topography of venous thromboembolism 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 seems 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 venous thromboembolism. The inherited or acquired hypercoagulable states can be divided 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 that 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.1) For patients at high risk of recurrence, 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. 2) It is not always possible to precisely determine the optimal duration with the available data. We have performed a meta-analysis on summary data which suggests that 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 and we propose to perform this analysis in a international collaborative group.

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

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).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.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), micropoly-angiitis (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).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.

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.

The growing number of reports of surgery for papillary fibroelastomas of the heart led us to evaluate the diagnostic potential of ultrasonography in patients with cerebral or coronary signs and to assess the efficacy of anticoagulant therapy in preventing recurrent cerebral ischemia and disease progression after resection.Ten cases of echographically diagnosed fibroelastoma of the heart treated by surgery were analyzed together with cases reported in the literature.Transesophageal echography has been shown to be the superior method. Surgical resection has given good results and the postoperative course is always excellent. Recurrent embolism occurred in two of our cases despite well-conducted anticoagulation.Surgical resection should be performed as early as possible because anticoagulation does not appear to sufficiently protect against embolic events, particularly cerebral events.

Venous thromboembolism disease is frequently seen in medical practice. Its morbidity, mortality and long-term sequels, as well as its haemorrhagic iatrogenic complications, represent a major problem of public health. For therapeutic management, we can usually use in medical practice non-fractionated heparin or low molecular weight heparin, sometimes substituted by antivitamin K therapy. Vena cava filter, surgical thrombectomy or thrombolytic therapy are rarely used. Elastic contention should by systematically prescribed. Therapeutic guidelines have been published on the recommendation furnished by randomised controlled trials. Low molecular weight heparin and early substitution by antivitamin K permitted an ambulatory treatment for deep vein thrombosis. The optimal duration of anticoagulant therapy is still controversial. Adequate biological survey causes decreasing incidence of haemorrhagic complications.

We report two observations of young women with concomitant diagnosis of acute arterial occlusion of the upper limb and Graves' disease. The occlusion was probably related to arterial embolism on paroxysmal atrial fibrillation, without underlying cardiopathy. Dosage of thyroid hormones should be performed in case of arterial occlusion in young subjects without cardiovascular risk factors.

The outcome of 75 cases of post-radiotherapy pericarditis severe enough to lead to surgery, was reviewed from 1970 to September 1995. Four clinical forms were identified: acute pericarditis resistant to medical treatment (12 cases), large chronic pericardial effusions resistant to medical treatment (16 cases), chronic, compressive effusions (35 cases) and signs of pericardial constriction (12 cases). The medical conditions irradiated were Hodgkin's disease (41 cases), followed by carcinomas (27 cases), malignant lymphomas, thymoma and seminoma. Forty-three deaths were observed during the study period (only 4 unrelated to the neoplasia or radiotherapy) after a mean interval of 7.4 years after radiotherapy and 2.5 years after surgical biopsy: 25 were related to cardiopulmonary complications of radiotherapy: 14 were due to recurrence of the malignant disease or the appearance of another tumour. Fifty-two pericardial effusion drainage procedures and 28 pericardectomies were performed, due to the necessity for reoperation. The authors have tried to determine therapeutic indications based on recognised physiopathological data and the anatomical aspects of the four different clinical forms. The prognosis is affected by post-radiotherapy fibrosis which extends beyond the pericardium to involve other cardiac structures and the pulmonary parenchyma. However, it must be emphasised that the initial survival of all these patients was due to the radiotherapy.

Every clinician managing a patient with venous thromboembolism of the lower limbs is faced with two opposing problems: first the risk of antivitamin K induced haemorrhage requires adequate but not excessive hypocoaguability of limited duration (international normalized ratio between 2 and 3); second the threat of recurrence requiring an adequate level of hypocoaguability of sufficient duration. Recently reported clinical data have greatly changed management attitudes. Current recommendations favour a 6 week regimen of antivitamin K for distal venous thrombosis in patients with thromboembolism of lower limb veins without any other aggravating factor and a 12 week regimen for proximal vein thrombosis or pulmonary emboli, although the therapeutic efficacity and risk remain to be demonstrated with precision. In France, we are conducting a multicentric controlled study with sufficient power (1800 patients) comparing parallel groups of patients: those with distal deep venous thrombosis treated 6 versus 12 week regimens, those with proximal deep venous thrombosis and/or pulmonary emboli treated 12 versus 24 week regimens. The "DOTAVK" study (Durée Optimale du Traitement Antivitamine K) involves patients with first time venous thrombosis or pulmonary emboli and no underlying neoplasia or coagulation disease. The two criteria of outcome are haemorrhage complications and thromboembolic recurrence during treatment and the first year after treatment withdrawal.