[Upper-extremity venous thrombosis: A retrospective study about 160 cases]. / Épidémiologie des thromboses veineuses des membres supérieurs : étude rétrospective de 160 thromboses aiguës.

AIM: To describe the clinical features and etiologies of upper limb venous thrombosis (ULVT). METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Late-onset systemic sclerosis: A retrospective study of 27 patients diagnosed after the age of 70]. / Sclérodermie systémique de révélation tardive : étude rétrospective de 27 patients diagnostiqués après l'âge de 70 ans.

BACKGROUND: The aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Radiographic presentation of musculoskeletal involvement in Werner syndrome (adult progeria).

Werner syndrome (i.e., adult progeria) is a rare autosomal recessive disorder caused by mutations of the WRN gene, which is characterized by the premature appearance of features associated with normal aging and cancer predisposition. Patients with Werner syndrome can present with musculoskeletal complaints, associated with suggestive radiographic features with a potential prognostic or therapeutic impact. This review illustrates the main radiographic features of Werner syndrome, focusing on the musculoskeletal system, such as soft-tissue calcification, muscular atrophy, osteoporosis, foot deformities, osteitis and osteomyelitis, and bone or soft-tissues malignancies. The identification of these features by radiologists can therefore be useful in the clinical screening of Werner syndrome.

[Superior vena cava syndrome induced by pacemaker probes, 12 years after introduction]. / Un syndrome cave supérieur 12ans après l'implantation d'un pacemaker.

Superior vena cava syndrome is a rare disease, most often found to result from a malignant process, which causes extrinsic compression of the superior vena cava. In recent years, there has been an increase of superior vena cava syndrome related to medical devices (implantable site, pacemaker [PM], central venous line for parenteral nutrition...). We report the case of a 37-year-old patient who developed a superior vena cava syndrome 12 years after implantation of a PM. The diagnosis was established on venography after two negative venous-CT focused on the superior vena cava. The superior vena cava syndrome improved immediately after angioplasty and stenting covering the PM probes at the superior vena cava/brachiocephalic venous trunk junction.

[Necrotizing hand-foot skin reaction induced by antiangiogenic in a patient with Thevenard neuroacropathy]. / Syndrome pied-main nécrotique sous antiangiogéniques chez une patiente porteuse d'une acropathie ulcéromutilante de Thévenard.

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.

[Risk of venous thromboembolism in acute medical illnesses. Part 2: Situations at risk in ambulatory, hospital and internal medicine settings]. / Risque thromboembolique veineux au cours des affections médicales aiguës. Partie 2: situations à risque en milieu ambulatoire, en milieu médical hospitalier et en médecine interne.

PURPOSE: The increased risk of thromboembolism in acute medical illnesses (AMI) is difficult to assess because of the diversity of medical conditions. The first part of this review of the literature was dedicated to methods of risk analysis based on our current pathophysiological knowledge. This second part describes more specifically the risk of venous thrombosis linked to AMI in hospital, ambulatory and internal medicine settings. CURRENT KNOWLEDGE AND KEY POINTS: The incidence of venous thromboembolism is higher in hospital than in ambulatory setting, albeit the latter remains significant. Stroke and affections leading to intensive care management represent conditions at great risk. Several mechanisms leading to a prothrombotic state have been identified, explaining the increased risk observed during relapses of pathologies specifically treated in internal medicine such as lupus erythematosus, Wegener granulomatosis, inflammatory bowel diseases and Behcet's disease. FUTURE PROSPECTS AND PROJECTS: Next to the pathophysiological understanding of venous thrombosis, the assessment of the specific thrombogenic burden of an AMI is an additive tool to screen medical patients at high risk. This systematic review of the literature shows uncertainties towards some risk factors as bedrest or acute inflammatory response. Taking into account the methodological difficulties inherent to prospective and epidemiological studies, a meta-analysis focusing on these factors would be useful to refine prevention guidelines for venous thromboembolism in medical setting.

[Screening of abdominal aortic involvement using Doppler sonography in active giant cell (temporal) arteritis at the time of diagnosis. A prospective study of 30 patients]. / Dépistage de l'atteinte aortique abdominale par échographie-doppler au diagnostic de maladie de Horton. Etude prospective de 30 patients.

BACKGROUND: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Risk factors for venous thromboembolism in hospitalized patients in internal medicine: case-control study of 150 patients]. / Facteurs de risque de la maladie thromboembolique veineuse chez des malades hospitalisés en médecine interne: une enquête cas-témoins sur 150 patients.

BACKGROUND: Risk factors for the thrombo-embolic disease are not yet completely validated in medical setting. Indeed, there is a lack of scientific data concerning their prevalence and their thrombogenic weight. METHODS: We carried out a prospective case-control study in an internal medicine unit where general acute pathologies are recruited. We have included two patients without thrombosis for each patient admitted for a thrombo-embolic event. In all patients, at admission, clinical and biological risk factors for thromboembolism were systematically analysed in a physiopathological and chronological way. RESULTS: 450 patients were included during the 27 months period of the study. Among these patients, 150 were hospitalised for a deep venous thrombosis of the leg or/and a pulmonary embolism. Height risk factors have demonstrated their high thrombogenic potential: paralysis of the legs (OR = 13; 95% CI: 1.4 to 5.1), post-thrombotic syndromes (OR = 13; CI: 1.7 to 4.3), extrinsic compressions (OR = 8; CI: 1.3 to 5.4), pregnancy (OR = 6; CI: 1.2 to 6), malignancy occurred one year before (OR = 4; CI: 1.1 to 6.7), personal history of deep vein thrombosis (OR = 3; CI: 1.6 to 4.5), varicosity (OR = 2.5; CI: 1.2 to 6.1) and venous insufficiency (OR = 1.7; CI: 1 to 7.4). Four parameters, generally considered as risk factors, didn't prove here their pathogenic power: the age over 60, obesity (BMI > 27), bedrest and the presence of an on-going malignancy. CONCLUSIONS: Only risk factors with high thrombogenic power (as paralysis or extrinsic compression for example) could be identified by this kind of study performed in polypathological patients. In this particular population, usually recognised risk factors as age, obesity and bedrest are not determinant parameters for the thomboembolic risk evaluation.

Popliteal artery thrombosis secondary to exostosis of the tibia.

This report describes an exceptional case of popliteal artery thrombosis secondary to exostosis of the superior extremity of the superior tibia in a young adult. Correct diagnosis was made during re-operation for recurrent thrombosis. Surgical treatment consisted of resection of the bony tumor and venous bypass to reestablish arterial continuity. Femoropopliteal vascular complications of exostosis are rare, with most cases involving arterial aneurysms or false aneurysms. Differential diagnosis in our young patient took into account the other causes of popliteal thrombosis: entrapped popliteal artery, adventitious cyst, fibrodysplasia, and juvenile arteriopathy. In patients with major functional disability, operative treatment is recommended to remove the bony abnormality and repair the arterial lesion.

[Disclosure of Behcet disease by concomitant dental infection]. / Poussée inaugurale de maladie de Behçet concomitante d'un foyer infectieux dentaire.

BACKGROUND: Behçet's disease can be aggravated by chronic infection. Our case illustrates how the disease can be disclosed during an acute activation of a chronic infectious focus. CASE REPORT: A 40-year-old man had Behçet's syndrome associating fever, bipolar aphtosis, cutaneous pseudo folliculitis, anterior uveitis, and meningitis following an acute episode of a dental infection, with periapical granuloma. Antibiotic therapy was ineffective in calming this first flare-up of Behçet's disease. Corticosteroid and colchicine therapy were effective. DISCUSSION: Clinical and experimental work would implicat buccal streptococcal flora in the pathogenesis of Behçet's disease. Antibiotics offer interesting perspectives but further studies are needed to define their role in the treatment of Behçet's disease.

[Venous thrombosis of the legs and cancer. Evaluation of risk factors of venous thrombosis in the medical environment]. / Thromboses veineuses des membres inférieurs et cancer. Evaluation des facteurs de risque de thrombose veineuse en milieu médical.

In medical patients, risk factors of leg venous thrombosis are not well evaluated. Cancer is considered as an important one. The aim of this study was to evaluate the role of intrinsic thrombotic risk (tumor hypercoagulable state) and external thrombotic risk (associate factors). We have made a prospective analysis of thrombotic venous risk factors in two medical populations with leg venous thrombosis: patients with cancer and patients without cancer. Risk of thrombosis depends on the thrombogenic importance of the risk factor and its chronicity or not. We assessed cancer and thromboembolic disease at the time of diagnosis and during a median follow up of 125.2 days. We included 31 consecutive cases of cancer (21 men, 10 women, mean age 63.8 years), and 50 consecutive cases of non cancer patients (32 men, 18 women, mean age 65.5 years), these two populations were not different. The classic risk factors of venous thrombosis were not frequent in cancer patient. Analysis of thrombotic risk showed that 61% of cancer patient group had venous thrombosis without classic thrombotic risk, as compared to 32% in non cancer patient group, showing the direct role of cancer in thrombosis (p < 0.01). The cancer was often aggressive and metastatic adenocarcinoma of various origins. The effect of chemotherapy is not clear, only hormonotherapy seemed to be responsible in two cases. Cancer hypercoagulability, defined by clinical characteristics, is a real risk factor of venous thrombosis but of low frequency. Indeed, the incidence of venous thrombosis in oncologic unit is rare (0.4%). Finally, thromboembolic disease in cancer patients is not different than in no cancer patients, Trousseau's syndrome is unfrequent. Prognosis is poor (40% death with 44.5 days of median survival), and antithrombotic therapy complications are frequent (bleeding 16%, oral anticoagulants resistance 20%).

Spontaneous dissection of the celiac artery.

We describe a case of intestinal angina caused by spontaneous dissection of the celiac artery and thrombosis of the superior mesenteric artery. Spontaneous dissection of a visceral artery is an uncommon occurrence that is usually diagnosed after fatal hemorrhage or ischemia. The underlying mechanism is unclear but the frequent association with multiple arterial lesions suggests general arterial disease. In symptomatic forms, surgical reconstruction is mandatory, to treat the lesion and allow definitive histological diagnosis.

[Limitations of the protein profile for diagnostic orientation in initial internal medicine consultation. Prospective study on 76 patients]. / Limites du profil protéique d'orientation diagnostique en consultation initiale de médecine interne. Etude prospective chez 76 malades.

Determination of the protein profile of orientation (PPO) is now considered by some authors as a means of improving the diagnosis in internal medicine. The feasibility of systematizing this practice was investigated in 76 outpatients (79 included, three excluded secondarily) seen for pathology of undetermined diagnosis. The 79 patients (mean age: 52 years) underwent the classical biological explorations plus PPO. The physicians were divided into two groups (seniors and assistants). Two complete clinical files were established for each patient, with one difference concerning inflammatory and immunologic data: one file included the minimum number of tests considered necessary by the physician and the other the complete PPO (nine proteins). Each file (with or without PPO) was randomly distributed to one of two physicians in the same group. Each physician filled in a diagnostic evaluation sheet indicating whether there was organic pathology or not, the main diagnosis (inflammatory, neoplastic, infectious or other), the secondary diagnosis and the hypothesis of probability. The relevance of the clinical opinion was analyzed by an internal medicine specialist from outside the department with 40 years of clinical experience. The duration of symptoms before the medical visit was from 3 weeks to 5 years (mean 6 months). A diagnosis of organic pathology was reached for three out of four patients. Sixty-seven patients were seen again after a minimum of 6 months, and nine were lost to follow-up. Diagnostic efficiency was no greater for cases with PPO, which appears to be a biological examination of second intention. We suggest that the term "protein profile of orientation" be replaced by "broad protein profile."

[Inguinal mastocytosis with histiocytic infiltrate]. / Mastocytose inguinale avec infiltrat histiocytaire.

INTRODUCTION: The diagnosis of mastocytosis is usually not difficult in cases with typical skin lesions. Intertrigo is exceptional. CASE REPORT: An 83-year-old patient developed inguinal intertrigo bilaterally. The lesions contained maculopapulous elements without Darier's sign. The histology examination revealed a mixed mast cell infiltration predominated by histiocytes. The ultrastructure confirmed the diagnosis of cutaneous mastocytosis. No extension was observed. DISCUSSION: Skin folds are rarely involved in infiltrating diffuse mastocytosis. Two cases have been reported in the literature including one doubtful case. Mastocytosis should thus be included in the possible diagnoses in patients with macupopapulous intertrigo. In addition, the mastocytic and histiocytic polymorphism observed here is rare.

Primary Raynaud's phenomenon. Age of onset and pathogenesis in a prospective study of 424 patients.

Many authors consider that late onset is a suspect criterion for differentiating primary Raynaud's phenomenon (Raynaud's disease, RD) from Raynaud's syndrome (RS). However, many cases of late-onset Raynaud's phenomenon in patients over forty years of age remain without etiologic diagnosis and therefore deserve the designation "late-onset RD." One hundred and ninety-four patients with RD (143 women, 51 men) were selected among 424 patients with Raynaud's phenomenon, according to Allen and Brown's criteria with negative serologic investigations and normal capillaroscopy. The purpose of the study was to consider the possible discriminant value of age of onset in distinguishing between RD and RS. The following epidemiologic features were studied: age of onset, sex, family history of Raynaud's phenomenon and migraine, and smoking and working habits. Microcirculation was assessed by capillaroscopy and strain-gauge plethysmography. Maximal digital flow at 45 degrees C and reactivity to cold were determined for each patient. Results were related to age of onset. The existence of true cases of late-onset RD in patients over forty years of age was confirmed (prevalence 27%), showing a correlation with a family history of Raynaud's phenomenon inferior to that found in early-onset cases (p < 0.0001). Microcirculation studies generally indicated a strong correlation between reactivity to cold, familial RD, and early onset, whereas no correlation was found with migraine or smoking. Nor was there any clinical or plethysmographic evidence for arteritis as a possible pathogenetic factor in late-onset RD. These results indicate that late-onset RD is a valid designation and that its pathogenesis seems less dependent on genetic sensitivity to cold than that of early-onset cases. In the absence of underlying arteritis, neurovascular dysfunction or a hemorheologic mechanism may be suggested as plausible causes of late-onset RD.

[Deep venous thrombosis and cancer. A one year evaluation of etiologic screening of patients hospitalized in Internal Medicine]. / Thromboses veineuses profondes et cancer. Evaluation a un an du bilan étiologique des patients hospitalisés en médecine interne.

The aim of this study was to evaluate over one year the minimal etiologic screening carried out on each patient hospitalized in Internal Medicine for a deep venous thrombosis (DVT) and to search for clinical features possibly relating thrombosis to neoplasia. 53 patients (27 male and 26 female; mean age 63.6 years, range: 18-19) with DVT of the lower limbs were included in the study. The initial etiologic screening consisted of a complete clinical examination, a biological assessment (blood cell and platelet count, Activated Partial Thromboplastin Time, inflammation and liver investigations), a radiography of the thorax and abdomino-pelvic ultrasonography. This etiologic screening was evaluated one year after the thrombotic event. The initial assessment was positive in 44 cases (83% of the patients), 9 thrombosis remained unexplained. Neoplasia was identified in 8 cases (15% of the patients), the diagnosis being often evoked at the time of interrogation or during the clinical examination; the systematic biological assessment only revealed an etiology in one case: this was a primary thrombocythemia discovered by platelet count. On evaluation one year later, amount the 9 DVT unexplained by the initial screening, 8 remained totally unexplained. On the other hand, a cancer of the colon revealed by proctorrhagia during oral anticoagulant treatment, had been undiscovered. No significant difference was found between the two groups (cancers and noncancerous etiologies) as concerns age, sex, thrombus topography, the side affected or the embolic events. However, the occurrence of subsequent thrombosis under well controlled anticoagulant treatment clearly characterises thrombosis associated with neoplasia.(ABSTRACT TRUNCATED AT 250 WORDS)

[Cerebral atrophy of vascular origin in the course of neurofibromatosis]. / Atrophie cérébrale d'origine vasculaire au cours d'une neurofibromatose.

The authors report the case of a 39-year-old woman with type I neurofibromatosis who presented a right incomplete proportional hemiplegia which progressively worsened over a 6-month period. Left hemispheric atrophy with heterogeneous features, predominant in the temporoparietal region, was revealed by computerized tomography. Atrophy was associated with diffuse vascular lesions in the distal part of the left sylvian and anterior cerebral arteries, leading to major cortical hypoperfusion. Vascular examination showed no hypertension nor any sign of arterial involvement in another region. This case illustrates the nature of vasculopathy associated with neurofibromatosis. Its expression is polymorphous, with lesions inducing stenosis (the most common ones), aneurysmal lesions or veritable angiodysplasias (either hypo- or hyperplastic). The vascular expression of neurofibromatosis is often overlooked. However, in the presence of an inexplicable occlusive or aneurysmal vasculopathy, it is advisable to search for signs of neurofibromatosis since ill-defined forms exist.

[Comparative study of digital vascular reactivity as a function of sex]. / Etude comparée de la réactivité vasculaire digitale en fonction du sexe.

The authors have studied the vasomotor status and digital vascular reactivity to cold in a series of 37 healthy volunteers (18 women and 19 men; mean age 30.6 years, range: 24-34) using digital mercury strain-gauge plethysmography. Correlations between digital blood flow and sex or other individual parameters (height, weight, smoking habits ...) were particularly looked for. Women's digital vascular tone evaluated under standardized conditions was found superior to men's, thus corroborating clinical and experimental data reported in literature. On the other hand, vasculotropic digital reactivity to local cooling was found similar in women and men. Moreover, no visible effect of local cooling was evidenced on the non-tested contralateral hand, suggesting the absence of significant general sympathetic influence during local cooling test.

[Ehlers-Danlos syndrome type IV, revealed by a primary dissecting aneurysm of the left subclavian artery]. / Syndrome d'Ehlers-Danlos de type IV, révélé par un anévrysme disséquant primitif de l'artère sous-clavière gauche.

The authors report an unusual dissecting aneurysm of the left subclavian artery occurring in a patient suffering from the Ehlers-Danlos syndrome. Clinical history and skin biopsy permitted this diagnosis. Surgical treatment associated resection of the aneurysm, ligation of the proximal part of the subclavian artery and a prosthetic bypass between carotid and subclavian artery. Spontaneous dissecting aneurysm is a rare disease and one must think before surgery to arterial dysplasia. Technical features of the surgical treatment are discussed.