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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.

Resume Les auteurs rapportent deux cas de syndrome de Lambert-Eaton associes a un cancer bronchique a petites cellules. Les donnees concernant le diagnostic clinique sont rappelees. Les acquisitions recentes sur le caractere auto-immun de cette affection, les relations entre le syndrome paraneoplasique et le cancer a petites cellules sont discutees. Ce syndrome est lie a la presence d'anticorps qui bloquent les canaux calciques voltage-dependants des terminaisons nerveuses. Les cellules du cancer a petites cellules expriment des canaux calciques, ces determinants antigeniques induisent probablement la production des anticorps. L'hypothese de l'origine auto-immune de ce syndrome paraneoplasique repose sur la notion d'antigenicite croisee.

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.

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.

Although suspected, a viral etiology has never been proven in giant cell arteritis (GCA). We tested for viruses known to induce multinucleated giant cells in human pathology, which include the parainfluenza viruses (HPIV), respiratory syncytial virus, measles virus, herpesviruses type 1 and 2, and the Epstein-Barr virus.A multicenter case-control study on incident cases of temporal arteritis (TA) and polymyalgia rheumatica (PMR). Population based age and sex matched controls were randomly selected. Serological tests for IgG and IgM directed against the viruses listed above were performed, on blood samples taken at the time of clinical diagnosis.Three hundred five new patients were included over a 5 year period, of whom 159 presented with positive biopsy TA, 70 with negative biopsy TA, and 76 with negative biopsy PMR. Thirty-eight percent of cases versus 20.9% of controls were positive for IgM directed against HPIV (p = 0.00005). The association was stronger in the positive TA subgroup [positivity rate 43.31%; odds ratio with controls 2.89 (95% CI 1.82-4.60, p = 0.000006)] than in the PMR or negative biopsy TA subgroups. Only HPIV type 1 was associated with the disease, regardless of the season or the geographical origin of the cases. Positivity rates for HPIV types 2 and 3 and for the other viruses tested were similar in cases and controls.Our findings suggest that reinfection with HPIV type 1 is associated with the onset of GCA in a subset of patients, particularly in cases with positive TA biopsy.

To assess the potential pathogenetic role of cardiovascular risk factors measured at diagnosis in temporal arteritis.Four hundred newly diagnosed patients and their age- and sex-matched controls randomly selected from the general population were included in a prospective multicentric cas-control study.In female patients, smoking increased the disease risk by a factor of 6 (95% CI: 2-17, p = 0.00006), and by a factor of 17 in case of10 pack-years. Pre-existing peripheral arterial disease increased the risk by a 4.5-fold factor (95% CI: 2-11, p = 0.0003), both in patients with a positive and those with a negative biopsy. Smoking alone appeared as a risk factor for polymyalgia rheumatica (OR: 3,64, 95% CI: 1.07-12.40). In male patients, none of the cardiovascular risk factors studied was positivity associated with the disease.Smoking and a pre-existing peripheral arterial disease are independently associated with temporal arteritis in women. Risk factors for disease with an imbalanced sex ratio should be studied separately for each sex in matched case-control studies in order to avoid matching bias.

To assess the role of cardiovascular risk factors, measured at the time of diagnosis, in the pathogenesis of giant cell (temporal) arteritis (GCA).Four hundred new patients with GCA or polymyalgia rheumatica (PMR) and population-based, individually age- and sex-matched controls were included in this multicenter, prospective case-control study. Each participant was evaluated by review of the medical history and by clinical and laboratory assessments.Among women, smoking was associated with a 6-fold increase in risk (P = 0.00006, 95% confidence interval [95% CI] 2-17), heavy smoking with a 17-fold increase in risk, and previous atheromatous disease with a 4.5-fold increase in risk (P = 0.0003, 95% CI 2-11) of GCA in both the biopsy-positive and biopsy-negative GCA groups; only smoking appeared to be a risk factor for PMR in women (odds ratio 3.64, 95% CI 1.07-12.40). Among men, no risk factor was found to be significant.Smoking and previous arterial disease were independently associated with GCA in women. In order to avoid matching bias, risk factors for diseases with an unbalanced sex distribution should be studied separately in each sex, using a sex-matched, case-control study design.

To assess the prevalence and thrombogenic role of anticardiolipin antibodies (aCL) in giant cell arteritis.Two hundred eighty-four patients with newly diagnosed temporal arteritis or polymyalgia rheumatica and 210 age- and sex-matched controls randomly selected from the general population were included in a multicenter, prospective case-control study. Blood samples were obtained at the time of diagnosis, data on initial clinical features were collected in a questionnaire, and temporal artery biopsy findings were reviewed by an experienced pathologist.Anticardiolipin antibodies were present in 20.7% of patients compared with 2.9% of controls (P = 1.45 x 10(-9)). The prevalence of aCL was higher in samples found positive for temporal arteritis than in those found negative on biopsy (31.2% versus 16.7%; P = 0.04), and was similarly higher in the biopsy-positive temporal arteritis group compared with the polymyalgia rheumatica and control groups. Although aCL were associated with thrombotic complications in univariate analysis, the positivity of the biopsy findings remained the only predictive variable in stratified analysis.In giant cell arteritis, aCL seem to function as reactive antibodies in relation to endothelial lesions.

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.

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.

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.

We report a case of painless giant cell thyroiditis with inflammatory syndrome, hypothyroidism, and multinodular goiter regressive after steroid treatment. The association with headache rises the question of its relation with giant cell arteritis.