[Giant cell arteritis: Role of color-duplex ultrasound]. / Artérite gigantocellulaire : apport de l'écho-doppler.

Giant cell arteritis is the most common primary vasculitis of large-vessel occurring in subjects over 50 years of age. Many imaging techniques has been evaluated to improve the diagnosis of giant cell arteritis. Among these imaging techniques, ultrasound has shown good performances to detect inflammatory involvement of the temporal arteries as well as branches of the aorta. Several publications and recent EULAR recommendations have emhasized the place of this tool in the diagnosis of giant cell arteritis.

[A double-faced myocarditis: Whipple's disease or sarcoidosis?]. / Une myocardite à deux visages : maladie de Whipple ou sarcoïdose?

INTRODUCTION: Whipple's disease is a systemic infection that may mimic sarcoidosis in its initial presentation. The heart involvement is not uncommon and consists generally in an endocarditis. Myocarditis is less common and is usually accompanied by impairment of heart conduction. CASE REPORT: We report a 56-year-old man with Whipple's disease associated with a myocarditis, initially diagnosed as having a sarcoidosis with cardiac injury. The contribution of the histology and molecular biology on intestinal sampling made it possible to rectify the diagnosis. CONCLUSION: The diagnosis of Whipple's disease should be considered in the presence of a systemic granulomatosis with or without heart involvement. Early diagnosis is important because of effectiveness of antibiotic therapy.

[Anticoagulation clinics for outpatients: a 5-year experience]. / Expérience d'un centre de suivi et de conseil des traitements anticoagulants oraux en médecine de ville: résultats à cinq ans.

INTRODUCTION: Anticoagulation clinics and computerized management of chronic oral anticoagulation increase the time spent in the therapeutic range with both mortality and morbidity reduction. Usually, anticoagulation clinics are hospital-based medical care centers. We report the five-year results from a general medicine center (CSCTA) using a computer-assisted management. METHODS: A prospective cohort observational study of 530 primary care patients that were receiving long term oral anticoagulation. RESULTS: Cardiac arrhythmia (55%), heart valve disease and venous thrombo-embolic disease (30%) represented the most common indications of oral anticoagulation. Patients received fluindione, warfarin and acenocoumarol in 80%, 13% and 7%, respectively. The duration of treatment was at least one year in 54% of the cases, and was at least three years in 25% of the cases. The rate of patients that were in average within the therapeutic range (INR 2-3) was 72%, while 12% were under and 16% over the therapeutic range. Corresponding rates were 82, 17 and 1% respectively for all anticoagulation targets (INR 1.5-4.5). Twenty-six bleeding events (4.9 per 100 patient-years) and four thrombotic complications (0.75 per 100 patient-years) occurred. Life-threatening hemorrhage occurred in 1.3 per 100 patient-years. After the equilibration of the anticoagulation, the average delay of control between two consecutive INR was 19 days. CONCLUSION: The results obtained with CSCTA were similar to those reported by other anticoagulation clinics regarding hemorrhagic complications and time spent in the therapeutic range. In contrast, thrombotic events were less frequent. Because of the absence of a control group, a medico-economic analysis could not be performed.

[Aortic involvement in giant cell arteritis. A prospective follow-up of 11 patients using computed tomography]. / Aortite inflammatoire et maladie de Horton. A propos du suivi évolutif scanographique prospectif d'une série de 11 patients.

PURPOSE: To determine clinical and radiological features, using computed tomography (CT-scan) in patients with aortic involvement related to giant cell arteritis (GCA), and to assess both clinical and CT-scan outcome after therapy institution. METHODS: Aortic involvement due to GCA was investigated in all patients, using CT-scan at diagnosis, and at 3, 6 and 12 months follow-up after therapy institution. RESULTS: The 11 consecutive patients consisted of 4 men and 7 women with mean age of 64.5 years. Patients exhibited: constitutional symptoms (N=9; 82%), dorsalgia (N=3; 27%), clinical signs of GCA (N=3; 27%) and of upper limb large vessel impairment (N=6; 55%). CT-scan showed aortitis involving both thoracic and abdominal aorta (N=6; 55%), abdominal (N=2; 18%) or thoracic aorta (N=2; 18%) and thoracic aortic aneurysm (N=1; 9%). At one-year follow-up, CT-scan revealed: complete resolution (N=7; 64%) and improvement (N=3; 27%) of aortic damage; the patient, who had thoracic aortic aneurysm, underwent surgical treatment, as aortic lesion remained unchanged on CT-scan. CONCLUSION: Our study underlines that CT-scan is a helpful test in diagnosis and follow-up of aortic involvement in patients with GCA.

[Helicobacter pylori prevalence in Raynaud's disease]. / Prévalence des infections à Helicobacter pylori au cours de la maladie de Raynaud.

PURPOSE: Recent studies have suggested that the prevalence of Helicobacter pylori may be more frequent in patients with primary Raynaud's phenomenon (PRP) compared to healthy subjects. These data prompted us to conduct this prospective study, in order to assess the prevalence of H. pylori infection in a large series of patients with PRP. METHODS: Forty consecutive patients with a definite diagnosis of PRP were included in the study. The findings in the PRP patients were compared with those of 80 age- and sex-matched healthy subjects. H. pylori infection was diagnosed using serology and urease breath test. RESULTS: The prevalence of H. pylori infection was as high as 12.5% in PRP patients using both serology and urease breath test, whereas it was found to be 16.7% and 18%, respectively, in healthy controls. CONCLUSION: As prevalence of H. pylori infection was similar in PRP patients compared to controls (P=0.53 and 0.43, respectively), our data underscore that H. pylori infection may not play a role in the genesis of PRP-related vascular complication onset. Interestingly, PRP patients exhibited more commonly digestive symptoms consistent with H. pylori infection compared to controls (P<0.05).

[Infectious or inflammatory aortitis? One case report]. / Aortite infectieuse ou inflammatoire ? A propos d'un cas.

INTRODUCTION: The infectious or inflammatory nature of an aortitis is difficult to assert because the microbiological results are often negative. The development of an aneurysm under treatment is rare, but requires a change in the therapeutic strategy and the etiologic diagnosis needs to be discussed again. EXEGESIS: We report the case of a 69-year-old woman treated by corticotherapy for an aortitis thought to be inflammatory, who required emergency surgery when a dissected aneurysm appeared. The peroperative samples were positive to Streptococcus pneumoniae using polymerase chain reaction and allowed a change of the diagnosis. The patient evolved favorably under antibiotic therapy. CONCLUSION: The decision to treat an aortitis by corticotherapy must be made with caution even if the microbiological tests are negative.

[Association of sarcoidosis and Takayasu's arteritis: an additional case report]. / Association sarcoïdose et maladie de Takayasu: une nouvelle observation.

INTRODUCTION: Takayasu arteritis has been described in association with various auto-immune disorders (mainly inflammatory digestive tract diseases). However, only few cases of Takayasu arteritis associated with sarcoidosis have been reported, raising the question of an association by chance. EXEGESIS: We report the case of a 26-year old woman with a 1-year history of sarcoidosis, who presented with a right painful upper limb, revealing inflammatory humeral, axillary and subclavian arteritis related to Takayasu arteritis. The patient was successfully treated with steroids. CONCLUSION: Our case report suggests that both Takayasu arteritis and sarcoidosis may be related, and that Takayasu arteritis or Takayasu arteritis-like granulomatous vasculitis may be, in fact, a complication of sarcoidosis. It also indicates that a complete vascular clinical examination should be performed in patients with sarcoidosis, in order to detect asymptomatic underlying inflammatory arteritis.

[Tuberculosis peritonitis: an always present disease. About 4 new cases]. / La tuberculose péritonéale: une entité toujours présente. A propos de quatre observations.

INTRODUCTION: Tuberculous peritonitis, a major problem in developing country, occurs preferentially in immigrant population and in patients with acquired immune deficiency syndrome (AIDS). Although rare in France, it did not disappear and epidemiological, clinical and therapeutic approach deserve to be reminded. EXEGESIS: We reported 4 patients (immigrants in two cases), occurred in caucasian and African persons (one with AIDS). Disease was characterized by fever, abdominal pain, anorexia, weight loss and ascites. Biological and radiological were unconclusive. Cell count analysis from ascitic fluid show a lymphocytic predominance with negative direct smear for Ziehl-Neelsen strain. Tuberculous peritonitis was established with combined visual and histological diagnosic laparoscopic examination. CONCLUSION: These observations have the interest to underline that tuberculous peritonitis must be evoked in case of lymphocytic ascitis. We believe an aggressive diagnostic approach, particulary with peritoneal biopsy, is warranted for the diagnosis of tuberculous peritonitis. Validity of PCR amplification is ascitic fluid still needs to be established.

Incidence and prognosis of cancer associated with bilateral venous thrombosis: a prospective study of 103 patients.

BACKGROUND: A strong association between bilateral deep vein thrombosis (DVT) and cancer had been found in one retrospective study. To confirm this finding, consecutive patients with an objective diagnosis of bilateral DVT were followed over 12 months. PATIENTS AND METHODS: One-hundred and three patients, hospitalized for bilateral DVT, were included in the study. Twenty-six patients (25.2%) were already known to have a cancer, 26 (25.2%) had a previous history of venous thromboembolic disease, 44 (42.7%) had a symptomatic pulmonary embolism. The patients were scheduled to be prospectively followed up at 3, 6 and 12 months as outpatients. Information on recurrence, evidence of a new overt cancer and the cause of death were recorded for all patients. RESULTS: A new cancer was diagnosed in 20 (26%) of the 77 patients without known cancer at admission. The risk of cancer was significantly more important in idiopathic thrombosis than in patients with secondary thrombosis (40.5% vs. 12.5%; odds ratio 4.8, 95% confidence interval 1.4, 18.8). Seventy percent of the cancers discovered had already spread. Age, gender, presence of pulmonary embolism, recurrence and location of the thrombosis were not statistically associated with the risk of cancer. The 1-year survival rates of patients with a previously known cancer and patients with a newly discovered cancer were, respectively, 26% and 35% (P = 0.33). CONCLUSIONS: Bilateral DVT is a significant risk indicator of malignancy. Cancer is present in 45% of patients with bilateral DVT and is associated with a poor prognosis.

Tolerance of Iloprost and results of treatment of chronic severe lower limb ischaemia in diabetic patients. A retrospective study of 64 consecutive cases.

BACKGROUND: The aim of this study was to assess the tolerance and report obtained results with a stable prostacyclin analogue (iloprost) in diabetic patients with severe forms of permanent lower limb ischaemia. METHODS: Sixty-four consecutive unselected patients, in stage III and IV of Leriche and Fontaine, turned down for vascular surgery after angiography and treated with iloprost during 28 days, were enrolled in this study. Patients were followed-up clinically (ischemic pain, trophic change, walking distance) and with transcutaneous oxymetry (D28). Long-term assessment (6 and 12 months) was expressed as rate of death, major amputation and of live patients with viable limbs and walking. There was no manifestation of intolerance to iloprost. Were considered as responders patients offering a lack or significant decrease in pain, a reduction of trophic lesions and improvement or recovery of walking. RESULTS: Response at two months is lasting: 29 responders (45.3%) and 35 non-responders (54.7%). At 6 months and one year, we observed that 8 (12.5%) and 15 (24.1%) patients respectively had died; 19 (29.6%) and 22 (34.3%) patients underwent major amputation, but 41 (64%) and 34 (53.1%) patients were still alive with their limb and conservative walking. In responder group, at 6 months, 28 (96.5%) patients were alive without amputation for only 13 (37.1%) among non-responders. At one year, 79.3% of the responders and 31.4% of the non-responders were alive without amputation. A total loss of walking, a segmental amputation and a previous amputation of opposite limb were more often noted in no responder group. But no predictive factor was referred to TcPO(2) in particular. Results ware similar in the group of 136 non diabetic patients treated during the same period (67.9% alive with limb at 6 months). CONCLUSIONS: This retrospective study, despite its limitations, underlines the clinical particularities of critical ischaemia in diabetics and the good tolerance to iloprost. This point allowed patients, in non-surgical chronic critical ischaemia, to avoid being confined to bed and to access to benefits of a early physiotherapy, in association with local treatment. However, no predictive criterion of long-term results could be established, except initial clinical severity and clinical change one month after treatment.

Endothelins. A potential target for pharmacological intervention in diseases of the elderly.

The existence of vasoconstrictive factors originating from the endothelium was confirmed by the description of endothelin, a 21-amino-acid peptide derived from a series of precursors, preproendothelin and a 38-amino-acid big endothelin. Three isoforms of endothelin, endothelin-1, -2 and -3, and 3 receptors (ETA, ETB and ETC) have been described and cloned. The cellular mode of action of endothelin seems to involve the modulation of intracellular calcium (through inositol trisphosphate, diacylglycerol and phospholipase C) and activation of calcium channels. The effects of endothelin are predominantly on the cardiovascular system. Its major effect is vasoconstriction, both systemic and pulmonary, with additional positive chronotropic and inotropic effects on the heart. It has also been implicated in homeostatic regulation of kidney microcirculation, and has powerful mitogenic effects on fibroblasts and smooth muscle cells. Many additional effects have been described on the endocrine system and on other systems. However, the clinical relevance of such effects is uncertain. Increased plasma endothelin levels have been reported in many diseases, but as yet it is not certain whether they are a cause or a consequence of the pathology. Pathologies most probably related to endothelin dysfunction are the vasospastic diseases, especially vasospasm after subarachnoid haemorrhage. Endothelin could be implicated to a lesser measure in diseases typical of the elderly population, such as hypertension or atherosclerosis. Drugs are being developed which act on endothelin metabolism, the most promising of which appear to be the inhibitors of endothelin converting enzyme and endothelin receptor antagonists. Some already existing drugs, such as calcium channel blockers or angiotensin converting enzyme inhibitors, probably act at least in part by interfering with endothelin metabolism or effects.

A shock associated with adult-onset Still's disease.

Only two cases of adult-onset Still's disease associated with shock have been previously described. We report a case of shock in a man with adult-onset Still's disease and discuss the relationship between the two processes by assessing tumor necrosis factor-alpha, procalcitonin and interleukin-6 concentrations.

[Hormone replacement therapy in menopause, hemostasis and thromboembolic risk]. / Hormonothérapie substitutive de la ménopause, hémostase et risque thrombo-embolique.

The association between oral contraceptives and the risk of thrombosis is now well documented. Conversely, post-menopausal hormonal therapy with doses and types of estrogen vastly different, seems not conducive of venous thromboembolism. Clotting factor abnormalities which have occasionally been observed in patients receiving hormone replacement therapy appear to be relatively unimportant clinically. Since no study is available on the assessment of the risk of thrombosis with these treatment, any hormone replacement therapy should be contraindicated as a principle, in both patients with recurrent venous thrombosis with or without known hemostatic abnormality. This attitude could be modified, if the therapy is justified by osteopenia or clinical complaints, after consulting specialist in gynecology, endocrinology, angiology or rheumatology, informed of the risk arising from such hormonal therapies. In theses particular cases, hormonal therapy with the fewest metabolic effects could be used.

[Tolerance and therapeutic results of iloprost in obliterative arteriopathy in lower limbs at the severe chronic ischemia stage. A retrospective study of 29 consecutive cases]. / Tolérance et résultats thérapeutiques observés de l'iloprost dans l'artériopathie oblitérante des membres inférieurs au stade d'ischémie chronique sévère.

The aim of this retrospective, study was to assess the tolerance and therapeutic effect of a stable prostacyclin (iloprost) analog in severe forms of permanent lower limb ischemia. Ninety consecutive unselected patients, in Leriche and Fontaine stages III or IV, turned down for vascular surgery after angiography and treated with iloprost for 28 days were enrolled in the study. Patients were followed up clinically (ischemic pain, trophic changes, walking distance) and with transcutaneous oxymetry (D28). Long-term assessment (mean 2 years) was expressed as rates of death, major amputation and "patients alive with limb". There were no manifestations of intolerance to iloprost. At two months, 42 out of 90 patients (47%) were considered as responders because of a lack (n=36) or significant decrease (n=6) in pain, reduction of trophic lesions and conservative walking. At long term (6 months, one and two years) we observed that 10 (11%), 17 (20%) and 22 (25%) patients respectively had died, 24 (27%), 26 (30%) and 28 (32%) patients underwent major amputation, but 60 (68%), 54 (62%) and 49 (56%) patients still alive with their limb and conservative walking. No predictive factors were noted, but diabetic patients without microangiopathy or recent bypass occlusions (respectively 43% and 56% out of patients were alive with limb at 6 months) were associated with bad results. This retrospective study, despite its limitations, underlines the good tolerance to, and effectiveness of iloprost in non surgical chronic critical ischemia. However, no predictive criterion of long-term effectiveness could be established, except initial clinical severity and clinical change one month after treatment.

[Erythermalgia and systemic lupus erythematosus]. / Erythermalgie et lupus erythémateux disséminé.

We report three cases of erythermalgia associated with systemic lupus erythematosus corresponding to different clinical situations in such an association. The first patient developed erythermalgia during the course of systemic lupus erythematosus. In the second, erythermalgia preceded other symptoms of systemic lupus erythematosus by four years. In the third, erythromelalgia was not related to a flare-up of systemic lupus erythematosus, but to thrombocythemia, a complication of immunosuppressive therapy. These cases permit a discussion on terminology and classification of erythromelalgia and erythermalgia. However, more than terminology or classification into three types or into adult-onset and early-onset (childhood) erythromelalgia, the important is to consider primary and secondary forms. We used a classification into two types: primary (or erythermalgia) with subdivision into sporadic and familial subtypes, and secondary with subdivision into erythermalgia related to myeloproliferative disorders and erythermalgia related to other diseases, such as systemic lupus erythematosus, or to drugs (erythermalgia-like syndrome).

[Childhood acromelalgia a propos of a case revealing Fabry's disease]. / Acromélalgies de l'enfant a propos d'une observation révélatrice de maladie de Fabry.

The occurrence of an acrosyndrome (Raynaud's phenomenon, erythermalgia, acrodynia...) in childhood may be the first manifestation of a general disease. Though it can be an early onset Raynaud's disease, it could also be the first sign of a connective tissue disease (juvenile polyarthritis, mixed connectivitis...) or of a overload disorder. We report a case of childhood-onset acromelalgia leading to the discovery of Fabry's disease. This chromosome X-linked hereditary disorder, resulting in the ubiquitous accumulation of neutral sphingolipids, is usually rapidly suspected by the finding of "boxer-short" angiokeratoma. Diagnosis is confirmed by the ophthalmic examination (cornea verticillata), by the pathological examination of a skin sample, and by the measure of alpha-galactosidase A activity. Treatment is usually only symptomatic, but the discovery of the mutations responsible for the disease could open the way to specific therapy.

[Horton's disease with involvement of the arterial trunks of the arms. Pathogenic role of anticardiolipin antibodies? Report of two cases]. / Maladie de Horton avec atteinte des troncs artériels des membres supérieurs. Rôle pathogène des anticorps anticardiolipine? A propos de deux observations.

Diffuse arterial involvement in temporal arteritis is well known but the intimate mechanisms of vasculopathy is unknown. Recently the presence of anticardiolipin antibodies (aCL) has been recognized in giant cell arteritis. We report two cases of temporal arteritis with diffuse arterial involvement associated with aCL (axillary arteries in the two cases associated with femoral arteries in one). During corticosteroid and anticoagulant therapy, a rapid improvement was noted with regression of upper-limb ischemia. One month later, the aCL were absent. These cases confirm the presence of aCL in giant cell arteritis with diffuse arterial involvement. These antibodies might imply severe vascular damage and could play a role in pathogenesis of the vasculopathy of temporal arteritis. Their presence suggests the necessity of anticoagulant therapy at the beginning of corticosteroid therapy.

[Acquired autoantibodies against human factor VIII: a new case]. / Auto-anticorps acquis anti-facteur VIII humain: une nouvelle observation.

The finding of a prolonged partial thromboplastin time and a normal prothrombin time localized the coagulation defect to the intrinsic or contact activation limb of the coagulation cascade. Because the situation is a source of hemorrhage, a rational and rapid approach is necessary with measure of factors of intrinsic limb of coagulation (especially factors VIII and IX, for the diagnosis of classic hemophilia), study of von Willebrand factor and search of coagulation factor inhibitors. We report the case of a 25-year old woman with high titer postpartum antibody against factor VIII for illustrating the diagnosis approach.